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Evolving experience with video-assisted thoracic surgery in congenital cystic lung lesions in a British pediatric center
Abstract
Video-assisted thoracic surgery (VATS) is increasingly used for the resection of congenital cystic lung lesions (CLLs). This study aimed to evaluate the efficacy of VATS and its outcome in both antenatally and postnatally detected CLLs.
Forty-six patients managed during 2000-2005 were studied. Demographics, investigations, operative details, and outcome data were collected and evaluated. Patients were divided into 3 groups for analysis.
Antenatally diagnosed (groups I and II, n = 35): group I (20) had VATS at 20 months median (range, 16-35 months). Video-assisted thoracic surgery was successful in 14 of 20 (70%), notably in all cases of extralobar sequestrations and foregut duplication cysts. Inadequate vision/lung collapse and technical difficulties were the main reasons for conversion to open thoracotomy. Group II (n = 15) was considered unsuitable for VATS because of neonatal symptoms (6 congenital cystic adenomatoid malformations of the lung [CCAMs]) and/or large size/inexperience (5 CCAMs, 4 sequestrations) and had elective thoracotomy at 8 months median (range, 6 days-20 months). Postnatally diagnosed (group III, n = 11): 3 CCAMs, 6 duplications, and 2 sequestrations were diagnosed because of recurrent chest infection (8) or stridor (2), or incidentally (1) at 8 years median (range, 1.2-14 years). Video-assisted thoracic surgery was successful in 3 foregut duplications. A duplication and an intralobar sequestration were converted; open thoracotomy was performed in others because of previous recurrent pneumonic episodes. Postoperative pain and hospital stay were significantly less (P < .001) in successful VATS resection: median of 2 days (range, 1-7 days) compared with thoracotomy median of 6 days (range, 4-20 days).
Video-assisted thoracic surgery is a safe and effective option for asymptomatic congenital CLLs. It is anticipated that more successful CCAM resections using VATS will occur in the future as our technical ability improves.
... Thoracoscopic approaches to both congenital and acquired lung disease are increasing in utilization [7,26,42]. The feasibility and safety of thoracoscopic approaches to CLM have been previously described [11,12,[14][15][16]22,[42][43][44][45][46]. ...
... Thoracoscopic approaches to both congenital and acquired lung disease are increasing in utilization [7,26,42]. The feasibility and safety of thoracoscopic approaches to CLM have been previously described [11,12,[14][15][16]22,[42][43][44][45][46]. In the adult literature, the approach has been more readily embraced and accepted [47][48][49]. ...
... In contradistinction, this practice remains controversial in the management of children with CLM, as there are significant considerations in the adoption of thoracoscopic techniques in this population. In particular, challenges such as the limited working space, more delicate pulmonary anatomy, complex nature of the disease process and surgical dissection, and unique anesthetic issues including reduced pulmonary reserve on thoracic insufflation and use of single lung ventilation, have limited the adoption of this technique in infants [7,26,42]. ...
... Thoracoscopic approaches to both congenital and acquired lung disease are increasing in utilization [7,26,42]. The feasibility and safety of thoracoscopic approaches to CLM have been previously described [11,12,[14][15][16]22,[42][43][44][45][46]. ...
... Thoracoscopic approaches to both congenital and acquired lung disease are increasing in utilization [7,26,42]. The feasibility and safety of thoracoscopic approaches to CLM have been previously described [11,12,[14][15][16]22,[42][43][44][45][46]. In the adult literature, the approach has been more readily embraced and accepted [47][48][49]. ...
... In contradistinction, this practice remains controversial in the management of children with CLM, as there are significant considerations in the adoption of thoracoscopic techniques in this population. In particular, challenges such as the limited working space, more delicate pulmonary anatomy, complex nature of the disease process and surgical dissection, and unique anesthetic issues including reduced pulmonary reserve on thoracic insufflation and use of single lung ventilation, have limited the adoption of this technique in infants [7,26,42]. ...
... All reports that potentially included postnatal management were studied in detail. Reports from centers that published 2 or more series within this period were analyzed, and the most relevant or recent article were selected if cumulative numbers were reported [1,16], or alternatively 2 articles were included if the series were clearly sequential chronologically [10,11,17,18]. This analysis was intended to focus on the management of CCAM and BPS and their hybrids. ...
... The nature of surgical intervention most often discussed is of course open surgery involving thoracotomy. The introduction of thoracoscopic and minimally invasive techniques to excise CCAM and lung sequestration have added an unexpected dimension to the debate as hospital stay and certainly cosmesis may be superior to the open approach, although it seems doubtful whether complications would be any less [18,38,44,46,47]. The type of resection undertaken is either lobectomy or nonanatomical segmentectomy. ...
Antenatally detected asymptomatic congenital cystic lung lesions may be managed conservatively or by surgical resection. We undertook a systematic review and meta-analysis to quantify the risks of elective surgery, emergency surgery, and observation.
All series published between 1996 and 2008, where the postnatal management of congenital cystic lung lesions was described, were reviewed. A meta-analysis was performed to determine whether elective or emergency surgery was associated with a higher risk of adverse outcomes.
There were 41 reports describing 1070 patients (of whom 79% were antenatally detected). Five hundred five neonates survived without surgery into infancy, of whom only 16 (3.2%) became symptomatic. For all ages, elective surgery was associated with significantly less complications than emergency surgery. The risk ratio was 2.8 (95% confidence interval, 1.4-5.5; P < .005) when comparing complications after elective surgery with emergency surgery.
The risk of asymptomatic cases developing symptoms is small. However, elective surgery is associated with a better outcome than emergency surgery. If elective surgery is undertaken, it should be performed before 10 months. Although no prognostic indicators have so far been identified in the literature, a conservative approach may be appropriate for small lesions.
... The CLM resection performed in early childhood, unlike neonatal lobectomy, has also allowed us to perform parenchyma-preserving minimally invasive surgery in most of these cases [27]. Lobectomy is justified if more than 40% of the lobe is occupied by the CLM or the distribution is such that it does not allow safe and complete resection without damaging the vascular supply to remaining normal lobe. ...
Purpose
We report our experience with the clinical management of complex lung malformations: multilobar, multiple unilobar, and bilateral congenital lung malformations (CLM) associated with duplication cysts (bronchogenic or foregut) or congenital diaphragmatic defects including congenital diaphragmatic hernia (CDH) or diaphragmatic duplication (DD).
Materials and methods
This is a retrospective case notes review of all patients with complex lung malformations presenting to a tertiary center between 1997 and 2023. Patient demographics, pre-operative symptoms, antenatal and postnatal investigations, surgical intervention, and overall outcome were audited and reported.
Results
Twenty-four patients had complex lung malformations. Antenatally, 11 were diagnosed appropriately, while 13 were thought to have only a single malformation. Postnatal management identified a total of 53 abnormalities. Twenty-three patients underwent resection of 44 lesions (and 3 CDH repairs), demonstrating 10 histologically distinct lung malformations. Twenty patients underwent single-stage surgical management. Thoracoscopic lung parenchyma-sparing segmental CLM resection was achieved in 17 patients. There were no major complications. The average length of stay was 5 days (range 3–14). No patients required readmission by day 30.
Conclusion
We recommend awareness of the coexistence of complex CLM and peri-diaphragmatic malformations. Minimally invasive, single-stage resection with lung parenchyma sparing is safe and produces very good outcomes.
... Many authors agree that elective operations before the development of respiratory symptoms are associated with lower incidences of postoperative complications 1,10,13-16 . Nonetheless, surgery immediately after birth in asymptomatic prenatally diagnosed patients is not ideal due to technical problems such as small intercostal spaces, poor tolerance of one-lung ventilation, and limited visualization 17 . Not only is surgical timing crucial, but adequate experience is also key to performing safe and technically demanding thoracoscopic surgeries in children 18 . ...
Congenital lung malformations (CLM) are most commonly treated with a pulmonary lobectomy. However, due to technological advancement, video-assisted thoracoscopic surgery (VATS) segmentectomy is becoming an attractive alternative to VATS lobectomy. This study aimed to evaluate the safety, feasibility, and efficacy of VATS segmentectomy as a lung parenchyma-saving strategy in children with CLM. A retrospective analysis was performed on 85 children, for whom VATS segmentectomy was tried for CLM between January 2010 and July 2020. We compared the surgical outcomes of VATS segmentectomy with the outcomes of 465 patients who underwent VATS lobectomy. Eighty-four patients received VATS segmentectomy and thoracotomy conversion was necessary for one patient for CLM. The mean age was 3.2 ± 2.5 (range 1.2–11.6) years. The mean operative time was 91.4 ± 35.6 (range 40–200) minutes. The median duration of chest tube drainage was 1 (range 1–21) day, and the median length of postoperative hospital stay was 4 (range 3–23) days. There were no postoperative mortality and postoperative complications developed in 7 patients (8.2%), including persistent air leakage in 6 patients (7.1%) and postoperative pneumonia in 1 patient (1.2%). The median follow-up period was 33.5 (interquartile range 31–57) months and there were no patients requiring re-intervention or reoperation during the follow-up period. In the VATS segmentectomy group, the persistent air leakage rate was higher than in the VATS lobectomy group (7.1 vs. 1.1%, p = 0.003). Otherwise, postoperative outcomes were comparable between the two groups. VATS segmentectomy in children with CLM is a technically feasible alternative to VATS lobectomy with acceptable early and mid-term outcomes. However, the persistent air-leakage rate was higher in VATS segmentectomy.
... Four studies compared the use of epidural anesthesia after surgery. 11,13,14,16 This meta-analysis indicated that VATS was associated with less use of epidural anesthesia after surgery than with thoracotomy (OR, 0.08; 95% CI, 0.03-0.23; p < 0.00001; I 2 = 0%, Figure 2B). ...
Background
A meta-analysis was performed for a comparison of outcomes between video-assisted thoracoscopic surgery (VATS) and thoracotomy for congenital lung malformations (CLM).
Methods
Electronic databases, including PubMed, Scopus, Embase, and the Cochrane Library were searched systematically for literature aimed mainly at reporting the therapeutic effects for CLM administrated by VATS and thoracotomy.
Results
A total of 40 studies meeting the inclusion criteria were included, involving 2896 subjects. VATS was associated with fewer complications [odds ratio (OR) 0.54; 95% confidence interval (CI), 0.42–0.69], less use of epidural anesthesia (OR, 0.08; 95% CI, 0.03–0.23), shorter length of hospital stay [standard mean difference (SMD) −0.98; 95% CI, −1.4 to −0.55] and chest drainage (SMD, −0.43; 95% CI, −0.7 to −0.17), as compared with thoracotomy. However, thoracotomy showed superiority in reduced operative time (SMD, 0.44; 95% CI, 0.04–0.84). Pearson analysis (Pearson r = 0.85, 95% CI, 0.28 to 0.98, p = 0.01) and linear regression ( R square 0.73) confirmed a positive correlation between percentage of symptomatic cases and conversion in patients using VATS.
Conclusion
VATS is associated with fewer complications, less use of epidural anesthesia, shorter length of stay and length of chest drainage, but longer operative time, as compared with thoracotomy. Symptomatic patients with CLM using VATS may be prone to conversion to thoracotomy. The reviews of this paper are available via the supplemental material section.
... Ответственный автор -Бочкова Лариса Геннадьевна Тел.: 89276251889 E-mail: lu_lg@mail.ru По данным ряда авторов [3,4], кистозная гипоплазия (поликистоз лёгких) составляет 60 % всех врождённых пороков развития. Эта патология наблюдается на ранних стадиях эмбрионального развития. ...
The aim of the article is to describe the case of congenital cystic adenomatoid malformation. The definition of this pathology, its morphology, classification, frequency and clinical forms are considered in the work. The data of the observation of a newborn with a complicated form of the defect have been determined: the peculiarities of the neonatal adaptation, the results of examination and treatment.
... Surgical resection is the standard treatment for CPAM and the treatment of prenatally diagnosed asymptomatic patients is increasing and is controversial [2,3]. Recently, thoracoscopic treatment of CPAM has been reported in the literature [4][5][6][7]. ...
Aim:
Thoracoscopic lobectomy (TL) and open lobectomy (OL) were compared for treating congenital pulmonary airway malformation (CPAM) with preoperative complications, specifically pneumonia/abscess formation (PA).
Methods:
The medical records of 46 CPAM patients treated by lobectomy at our institution from 1990 to 2014 were reviewed retrospectively. Four groups, TL for patients without PA (n = 17; TL-), TL for patients with PA (n = 8; TL+), OL for patients without PA (n = 16; OL-), and OL for patients with PA (n = 5; OL+) were compared for operative time, intra/postoperative complications, blood loss, duration of chest tube insertion, postoperative analgesia, pre: postoperative white blood cell (WBC) ratio, and duration of hospitalization.
Results:
Operative time for TL+ was longest, but not statistically significant. Incidences of intra/postoperative complications were similar in all groups. Blood loss was significantly less for TL+ versus OL+ (p < .05). WBC ratio was significantly lower in TL+ versus OL+ (p < .05), similar for TL+ and TL-, and significantly higher in OL+ versus OL- (p < .01). Chest tube insertion was significantly longer in OL- versus TL- (p < .01).
Conclusion:
PA would not appear to be a contraindication to perform TL in CPAM. TL is associated with less surgical stress than OL despite longer operative time.
... This suggests that lobectomy in neonates or infants is desirable, even in asymptomatic cases. Although there are still few reports of thoracoscopic lobectomy in children, thoracoscopic lobectomy for CPAM has been indicated from the neonatal period to adolescence [4][5][6]. However, adhesions and incomplete or absent interlobar fissures make thoracoscopic surgery difficult, even in adults [7]. ...
Thoracoscopic lobectomy for congenital pulmonary airway malformation has been indicated from the neonatal period to adolescence. However, it is difficult to approach the pulmonary artery for lobectomy in congenital lung malformations with incomplete or absent interlobar fissures. Multidetector computed tomographic images and computed tomography pulmonary angiography gave us helpful information before the operation. We performed thoracoscopic lobectomy for congenital pulmonary airway malformations with absent interlobar fissures and adhesions in accordance with information from multidetector computed tomographic images.
A 14-year-old Japanese girl received a diagnosis of congenital pulmonary airway malformation when she presented with pneumonia. Using multidetector computed tomography and three-dimensional reconstruction provides meticulous characterization of the anatomy in pediatric patients. We confirmed that her left A4+5 artery arose from her left pulmonary artery medial to A6. Her left pulmonary artery was divided just proximal to the A6 origin before the lobes were separated safely. We took advantage of using a stapler to divide the fissureless thick parenchyma. Perioperative diagnosis was congenital cystic adenomatoid malformation.
We used preoperative multidetector computed tomography to outline the bronchovascular anatomy and guide hybrid video-assisted thoracoscopic surgery for a congenital cystic adenomatoid malformation in a fissureless left lung.
... Most cystic lesions are accessible by thoracoscopy. [10,17] This technique is characterized by low morbidity and minor scars. [18] In particular, thoracoscopy was found to significantly decrease chest tube time, hospital stay, and postoperative complications. ...
The need to systematically remove congenital cystic lung lesions is based on three main arguments. First, cystic malformations are often considered as congenital cystic adenomatoid malformations (CCAM), while other less favorable diagnoses are possible, such as pleuropulmonary blastoma. Only postsurgical pathological analysis allows diagnosis. Second, there are clinical and biological arguments for considering macrocystic lesions as likely to degenerate. The only prevention is surgical removal. Finally, there is no recommendation on how to follow these children, in the absence of removal, causing unnecessary family stress. This seems unjustified, compared to a feasibility of thoracoscopic removal in most cases.
Background:
Despite evidence of the long-term implications of unrelieved pain during infancy, it is evident that infant pain is still under-managed and unmanaged. Inadequately managed pain in infancy, a period of exponential development, can have implications across the lifespan. Therefore, a comprehensive and systematic review of pain management strategies is integral to appropriate infant pain management. This is an update of a previously published review update in the Cochrane Database of Systematic Reviews (2015, Issue 12) of the same title.
Objectives:
To assess the efficacy and adverse events of non-pharmacological interventions for infant and child (aged up to three years) acute pain, excluding kangaroo care, sucrose, breastfeeding/breast milk, and music.
Search methods:
For this update, we searched CENTRAL, MEDLINE-Ovid platform, EMBASE-OVID platform, PsycINFO-OVID platform, CINAHL-EBSCO platform and trial registration websites (ClinicalTrials.gov; International Clinical Trials Registry Platform) (March 2015 to October 2020). An update search was completed in July 2022, but studies identified at this point were added to 'Awaiting classification' for a future update. We also searched reference lists and contacted researchers via electronic list-serves. We incorporated 76 new studies into the review. SELECTION CRITERIA: Participants included infants from birth to three years in randomised controlled trials (RCTs) or cross-over RCTs that had a no-treatment control comparison. Studies were eligible for inclusion in the analysis if they compared a non-pharmacological pain management strategy to a no-treatment control group (15 different strategies). In addition, we also analysed studies when the unique effect of adding a non-pharmacological pain management strategy onto another pain management strategy could be assessed (i.e. additive effects on a sweet solution, non-nutritive sucking, or swaddling) (three strategies). The eligible control groups for these additive studies were sweet solution only, non-nutritive sucking only, or swaddling only, respectively. Finally, we qualitatively described six interventions that met the eligibility criteria for inclusion in the review, but not in the analysis. DATA COLLECTION AND ANALYSIS: The outcomes assessed in the review were pain response (reactivity and regulation) and adverse events. The level of certainty in the evidence and risk of bias were based on the Cochrane risk of bias tool and the GRADE approach. We analysed the standardised mean difference (SMD) using the generic inverse variance method to determine effect sizes. MAIN RESULTS: We included total of 138 studies (11,058 participants), which includes an additional 76 new studies for this update. Of these 138 studies, we analysed 115 (9048 participants) and described 23 (2010 participants) qualitatively. We described qualitatively studies that could not be meta-analysed due to being the only studies in their category or statistical reporting issues. We report the results of the 138 included studies here. An SMD effect size of 0.2 represents a small effect, 0.5 a moderate effect, and 0.8 a large effect. The thresholds for the I2 interpretation were established as follows: not important (0% to 40%); moderate heterogeneity (30% to 60%); substantial heterogeneity (50% to 90%); considerable heterogeneity (75% to 100%). The most commonly studied acute procedures were heel sticks (63 studies) and needlestick procedures for the purposes of vaccines/vitamins (35 studies). We judged most studies to have high risk of bias (103 out of 138), with the most common methodological concerns relating to blinding of personnel and outcome assessors. Pain responses were examined during two separate pain phases: pain reactivity (within the first 30 seconds after the acutely painful stimulus) and immediate pain regulation (after the first 30 seconds following the acutely painful stimulus). We report below the strategies with the strongest evidence base for each age group. In preterm born neonates, non-nutritive sucking may reduce pain reactivity (SMD -0.57, 95% confidence interval (CI) -1.03 to -0.11, moderate effect; I2 = 93%, considerable heterogeneity) and improve immediate pain regulation (SMD -0.61, 95% CI -0.95 to -0.27, moderate effect; I2 = 81%, considerable heterogeneity), based on very low-certainty evidence. Facilitated tucking may also reduce pain reactivity (SMD -1.01, 95% CI -1.44 to -0.58, large effect; I2 = 93%, considerable heterogeneity) and improve immediate pain regulation (SMD -0.59, 95% CI -0.92 to -0.26, moderate effect; I2 = 87%, considerable heterogeneity); however, this is also based on very low-certainty evidence. While swaddling likely does not reduce pain reactivity in preterm neonates (SMD -0.60, 95% CI -1.23 to 0.04, no effect; I2 = 91%, considerable heterogeneity), it has been shown to possibly improve immediate pain regulation (SMD -1.21, 95% CI -2.05 to -0.38, large effect; I2 = 89%, considerable heterogeneity), based on very low-certainty evidence. In full-term born neonates, non-nutritive sucking may reduce pain reactivity (SMD -1.13, 95% CI -1.57 to -0.68, large effect; I2 = 82%, considerable heterogeneity) and improve immediate pain regulation (SMD -1.49, 95% CI -2.20 to -0.78, large effect; I2 = 92%, considerable heterogeneity), based on very low-certainty evidence. In full-term born older infants, structured parent involvement was the intervention most studied. Results showed that this intervention has little to no effect in reducing pain reactivity (SMD -0.18, 95% CI -0.40 to 0.03, no effect; I2 = 46%, moderate heterogeneity) or improving immediate pain regulation (SMD -0.09, 95% CI -0.40 to 0.21, no effect; I2 = 74%, substantial heterogeneity), based on low- to moderate-certainty evidence. Of these five interventions most studied, only two studies observed adverse events, specifically vomiting (one preterm neonate) and desaturation (one full-term neonate hospitalised in the NICU) following the non-nutritive sucking intervention. The presence of considerable heterogeneity limited our confidence in the findings for certain analyses, as did the preponderance of evidence of very low to low certainty based on GRADE judgements.
Authors' conclusions:
Overall, non-nutritive sucking, facilitated tucking, and swaddling may reduce pain behaviours in preterm born neonates. Non-nutritive sucking may also reduce pain behaviours in full-term neonates. No interventions based on a substantial body of evidence showed promise in reducing pain behaviours in older infants. Most analyses were based on very low- or low-certainty grades of evidence and none were based on high-certainty evidence. Therefore, the lack of confidence in the evidence would require further research before we could draw a definitive conclusion.
Esophageal duplications and bronchogenic cysts are aberrations of primitive foregut development. Duplications can be found along the entire length of the esophagus with varying presentation depending on their location and pressure effects on neighboring structures. Although duplications can remain asymptomatic, they may get discovered incidentally during unrelated investigations. The complicated duplications can present with life-threatening symptoms. Differential diagnosis of posterior mediastinal rounded opacity on radiological investigation should include esophageal duplication cyst. Investigations such as an upper gastrointestinal contrast study and computed tomography of the chest are not diagnostic but can suggest duplication cyst. Once identified and investigated, the duplications should be resected. Thoracoscopic surgery is recommended for resection of all uncomplicated and some complicated cysts. Thoracoscopic surgery reduces the hospital stay, postoperative analgesia requirement, and morbidity compared to open surgery. Although thoracotomy can achieve resection, it should be reserved for the complicated cysts. The outcome after complete resection is excellent. Surgical complications can be avoided by optical magnification and careful dissection close to the cyst. Incomplete resection results in recurrence. Surgical complications are related to inadvertent accidental injury to the surrounding vessels, nerves, thoracic duct, esophagus, and neighboring trachea. This chapter attempts to give an overview of the esophageal duplication and its current management.
Pediatric patients present in varying stages of development, from the premature neonate to full-grown teenager. Appreciation of the unique physiologic states associated with the different stages of development will direct anesthetic management. Preoperative evaluation of the small child should include the neonatal history as this may indicate comorbid pulmonary and cardiac disease and linked syndromes which must be investigated. Lung isolation is not always necessary in pediatric thoracic surgery. Appropriate lung isolation techniques will depend on the age and size of the patient as there is no single technique that is suitable for all pediatric patients. Physiologic manifestation of one-lung ventilation may be more pronounced in children than in adults. The compliant rib cage, compressible lung parenchyma, reduced FRC under anesthesia, and higher oxygen consumption in the child contribute to aggravate hypoxemia during lung isolation. Adult thoracic surgery is often related to tumor excision whereas pediatric thoracic disease encompasses a greater variety of pathology. Each specific disease state has its own particular anesthetic considerations and management strategy. Pain management in the pediatric population has evolved to include a greater use of regional and neuraxial techniques, even in the smallest of infants. Postoperative disposition will depend on the type and length of surgery, extent of resection or manipulation, and nature of the underlying condition. Many pediatric patients will require postoperative ventilation or close cardiorespiratory monitoring following the procedure.
Esophageal duplications and bronchogenic cysts are aberrations of primitive foregut development. Duplications can be found along the entire length of the esophagus with varying presentation depending on its location and pressure effects on neighboring structures. Although duplications can remain asymptomatic, they may get discovered incidentally during unrelated investigations. The complicated duplications can present with life-threatening symptoms.
Differential diagnosis of posterior mediastinal rounded opacity on radiological investigation should include esophageal duplication cyst. Investigations such as an upper gastrointestinal contrast study and computed tomography of the chest are not diagnostic but can suggest duplication cyst. Once identified and investigated, the duplications should be resected.
Thoracoscopic surgery is recommended for resection of all uncomplicated and some complicated cysts. Thoracoscopic surgery reduces the hospital stay, postoperative analgesia requirement, and morbidity compared to open surgery. Although thoracotomy can achieve resection, it should be reserved for the complicated cysts. The outcome after complete resection is excellent. Surgical complications can be avoided by optical magnification and careful dissection close to the cyst. Incomplete resection results in recurrence. Surgical complications are related to inadvertent accidental injury to the surrounding vessels, nerves, thoracic duct, esophagus, and neighboring trachea. This chapter attempts to give an overview of the esophageal duplication and its current management.
Anatomical segmentectomy is an advantageous procedure because it spares healthy lung that has potential to show compensatory growth after lung resection and decreases the risk of air leak and residual resection, which becomes a problem in wedge resection. However, anatomical segmentectomy has not become a common procedure in pediatrics because it requires more complicated procedure than lobectomy or wedge resection, especially in patients with a history of pulmonary infection. In this case report, anatomical basal segmentectomy was safely performed with magnified vision by a hybrid video-assisted thoracic surgery (VATS) approach in a 6-year-old girl with intralobar pulmonary sequestration after severe pneumonia. The result suggests that the indications for hybrid VATS segmentectomy can expand further to include segmental lesions in children.
Aim:
The apparent incidence of antenatally diagnosed congenital lung malformations (CLM) is rising (1 in 3000), and the majority undergo elective resection even if asymptomatic. Thoracoscopy has been popularized, but early series report high conversion rates and significant complications. We aimed to perform systematic review/meta-analysis of outcomes of thoracoscopic vs open excision of asymptomatic CLMs.
Methods:
A systematic review according to PRISMA guidelines was performed. Data were extracted for all relevant studies (2004-2015) and Rangel quality scores calculated. Analysis was on 'intention to treat' basis for thoracoscopy and asymptomatic lung lesions. Meta-analysis was performed using the addon package METAN of the statistical package STATA14™; p<0.05 was considered significant.
Results:
36 studies were eligible, describing 1626 CLM resections (904 thoracoscopic, 722 open). There were no randomized controlled trials. Median quality score was 14/45 (IQR 6.5) 'poor'. 92/904 (10%) thoracoscopic procedures were converted to open. No deaths were reported. Meta-analysis showed that regarding thoracoscopic procedures, the total number of complications was significantly less (OR 0.63, 95% CI 0.43, 0.92; p<0.02, 12 eligible series, 912 patients, 404 thoracoscopic). Length of stay was 1.4days shorter (95%CI 2.40, 0.37;p<0.01). Length of operation was 37 min longer (95% CI 18.96, 54.99; p<0.01). Age, weight, and number of chest tube days were similar. There was heterogeneity (I(2) 30%, p=0.15) and no publication bias seen.
Conclusions:
A reduced total complication rate favors thoracoscopic excision over thoracotomy for asymptomatic antenatally diagnosed CLMs. Although operative time was longer, and open conversion may be anticipated in 1/10, the overall length of hospital stay was reduced by more than 1day.
Level of evidence:
4 (based on lowest level of article analyzed in meta-analysis/systematic review).
This work was a retrospective audit of CCAM ? 1994 to 2008. A total of 26 cases were identified. Mean gestational age at diagnosis was 20 weeks. All were unilateral and had serial scans. In 31% the lesion resolved, 8% decreased, 42% were unchanged and 4% increased in size. Only one foetus developed hydrops. All were born alive. Of 8 foetuses where the CCAM was thought to have resolved, 6 had persistent lesions (overall sensitivity and PPV of US 64% and 69%, respectively). Computerised tomography performed better than chest X-ray in detecting lesions postnatally (sensitivity 100% vs 88%, PPV 95% vs 78%). Seventeen cases required surgery, 4 conservative management and 2 were discharged following negative CXR; 12% had associated anomalies. Four babies had recurrent chest infections. CCAMs have a good overall prognosis. CT scan rather than CXR should be performed to confirm the resolution of antenatally diagnosed lesions. Surgical management is the preferred option over conservative management.
Purpose:
We sought to determine factors influencing survival and resource utilization in patients undergoing surgical resection of congenital lung malformations (CLM). Additionally, we used propensity score-matched analysis (PSMA) to compare these outcomes for thoracoscopic versus open surgical approaches.
Methods:
Kids' Inpatient Database (1997-2009) was used to identify congenital pulmonary airway malformation (CPAM) and pulmonary sequestration (PS) patients undergoing resection. Open and thoracoscopic CPAM resections were compared using PSMA.
Results:
1547 cases comprised the cohort. In-hospital survival was 97%. Mortality was higher in small vs. large hospitals, p<0.005. Survival, pneumothorax (PTX), and thoracoscopic procedure rates were higher, while transfusion rates and length of stay (LOS) were lower, in children ≥3 vs. <3months (p<0.001). Multivariate analysis demonstrated longer LOS for older patients and Medicaid patients (all p<0.005). Total charges (TC) were higher for Western U.S., older children, and Medicaid patients (p<0.02). PSMA for thoracoscopy vs. thoracotomy in CPAM patients showed no difference in outcomes.
Conclusion:
CLM resections have high associated survival. Children <3months of age had higher rates of thoracotomy, transfusion, and mortality. Socioeconomic status, age, and region were independent indicators for resource utilization. Extent of resection was an independent prognostic indicator for in-hospital survival. On PSMA, thoracoscopic resection does not affect outcomes.
Pediatric patients present in varying stages of development, from the premature neonate to full-grown teenager. Appreciation of the unique physiologic states associated with the different stages of development will direct anesthetic management. Preoperative evaluation of the small child should include the neonatal history as this may indicate comorbid pulmonary and cardiac disease and linked syndromes which must be investigated. Lung isolation is not always necessary in pediatric thoracic surgery. Appropriate lung isolation techniques will depend on the age and size of the patient as there is no single technique that is suitable for all pediatric patients. Physiologic manifestation of one-lung ventilation may be more pronounced in children than in adults. The compliant rib cage, compressible lung parenchyma, reduced FRC under anesthesia and higher oxygen consumption in the child contribute to aggravate hypoxemia during lung isolation. Adult thoracic surgery is often related to tumor excision whereas pediatric thoracic disease encompasses a greater variety of pathology. Each specific disease state has its own particular anesthetic considerations and management strategy. Pain management in the pediatric population has evolved to include a greater use of regional and neuraxial techniques, even in the smallest of infants. Postoperative disposition will depend on the type and length of surgery, extent of resection or manipulation, and nature of the underlying condition. Many pediatric patients will require postoperative ventilation or close cardiorespiratory monitoring following the procedure.
Background: Infant pain has been historically under-managed. Review question: This review assessed 24 different ways of reducing young children's pain during medical procedures without using drugs, such as using a pacifier, distracting the child, and rocking a child. We analysed studies separately for babies who were born preterm, full-term newborns, and older infants from one month to three years. We also looked at if there was a difference on the impact of the interventions depending on whether the infant had just had the painful procedure (pain reactivity), as opposed to calming down from their peak distress (immediate pain regulation). Study characteristics: This updated review examined 63 randomised controlled trials of 4905 participants. Key results and Quality of evidence: While there was evidence for non-nutritive sucking, swaddling and tucking, massage, environment modification, rocking, video distraction, structured non-parent involvement at different ages, and pain types, none of the analyses were based on sufficient evidence to allow us to draw firm conclusions (i.e. high quality studies from at least two independent laboratories).
Bronchopulmonary sequestration (BPS) is a rare developmental disorder in which part of the lung is disconnected from the main airway and receives aberrant arterial inflow from the aorta instead of from the pulmonary arteries. BPS belongs to the group of foregut malformations and is often accompanied by other defects. The incidence of BPS has increased considerably in the last few years due to the widespread routine use of fetal ultrasound screening. Several patterns of clinical expression are possible and therapeutic attitudes ranging from expectant observation to early surgical removal or embolization remain controversial.
Infections of the lung and pleura cause substantial morbidity in children. Children generally present early in the course of lower respiratory tract infections with respiratory distress and prompt treatment in the acute stage will often prevent late suppurative complications.[1,2] Tuberculosis and fungal infections typically produce chronic diseases. Children may present late in the course of these infections either with an acute exacerbation or as the result of progressive lung damage. The principle factors causing chronic sequelae of acute infections include inadequate primary treatment, immune deficiency, malnutrition (kwashiorkor and marasmus) and poor hygiene.[3] In the last decade acquired immunodeficiency syndrome (AIDS) has become an important cause of recurrent pulmonary infection in children with opportunistic bacteria, fungal infections and tuberculosis. The incidence of bronchiectasis in children has declined over the last 50 years. In part this has occurred due to better understanding and early, effective treatment of acute pneumonia. Improvements in sanitation, nutrition, education, widespread use of antibiotics and childhood immunization programs have also contributed. However, for reasons that not yet clear the incidence of parapneumonic empyema in the western world has increased substantially over the last decade and there is limited consensus regarding treatment.[4] In the tropics parasitic infestations, either alone or in association with secondary bacterial infection, cause substantial morbidity and mortality. As medical therapy for parasitic infestations has improved the need for surgical resection has reduced considerably. In western countries parasitic pulmonary disease is rare although it must be considered in children who have traveled from tropical countries.[5] Incorrect treatment as a result of mistaken diagnosis (e.g., for congenital lung malformation) is common.[6].
Pediatric lung tumors are rare compared with adults.1 In contrast, metastatic tumors are commonly reported in children. The majority of primary lung tumors in children are malignant. 2,3 Most benign and malignant lung tumors in children are discovered accidentally on a routine chest X-ray taken for nonspecific symptoms. Although radiological investigations define these lesions accurately, it is difficult to differentiate between benign and malignant lesions without microscopic examination. Therefore, all lung lesions in children should be resected unless it is possible to confirm an infective etiology.
Antenatal diagnosis of lung lesion has become more accurate resulting in dilemma and controversies of its antenatal and postnatal management. Majority of antenatally diagnosed congenital lung lesions are asymptomatic in the neonatal age group. Large lung lesions cause respiratory compromise and inevitably require urgent investigations and surgery. The congenital lung lesion presenting with hydrops requires careful postnatal management of lung hypoplasia and persistent pulmonary hypertension. Preoperative stabilization with gentle ventilation with permissive hypercapnia and delayed surgery similar to congenital diaphragmatic hernia management has been shown to result in good outcome. The diagnostic investigations and surgical management of the asymptomatic lung lesions remain controversial. Postnatal management and outcome of congenital cystic lung lesions are discussed.
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Purpose:
Congenital lung malformations (CLM) are traditionally treated by pulmonary lobectomy. The foremost indication for surgery is that these lesions predispose to respiratory tract infections and to malignancy. Owing to the improvement of prenatal diagnosis, most patients are operated in a timely manner and prophylactically. In this context, lung-sparing surgery (LS) has gained interest as a lung preservation strategy, especially for asymptomatic patients. In the present study, we evaluated both thoracoscopic and open lung-preserving resections as an alternative to lobectomy for CLM resection.
Methods:
We retrospectively reviewed all patients who underwent lung-sparing resection (segmentectomy and atypical resection) for CLM from 2001 to 2010. Data were collected regarding preoperative diagnostic workup, type of intervention, and follow up.
Results:
Fifty-four patients received LS for CLM during the study period. Twenty-six were approached thoracoscopically, with 18 cases requiring open conversion for a complete resection. There were six postoperative complications: three asymptomatic pneumothoraces that resolved without intervention, one tension pneumothorax that required replacement of a drainage catheter, and three instances of intra-operative bleeding requiring blood transfusion. Mean duration of follow-up was 65.2 months. Two patients experienced pneumonia during the follow-up period. A third patient had a cystic lung lesion on postoperative computed tomography (CT) which required a second-look surgery.
Conclusions:
LS for CLM is a safe and effective means of lung parenchymal preservation in pediatric patients. Complication rates are comparable to that of traditional lobectomy. In our experience, this type of lung surgery does not carry a higher risk of residual disease and recurrence if accurately planned in selected patients, i.e., those with small asymptomatic lesions. The complication rate is acceptable and apparently not affected by preoperative symptoms. The thoracoscopic approach is recommended, although open conversion should be advocated to avoid too long operative times.
Congenital lung malformations (CLM) predispose patients to recurrent respiratory tract infections and pose a rare risk of malignant transformation. Although pulmonary lobectomy is the most common treatment of a CLM, some advocate segmental resection as a lung preservation strategy. Our study evaluated lung-preserving thoracoscopic segmentectomy as an alternative to lobectomy for CLM resection.
We conducted a retrospective review of patients who underwent thoracoscopic segmentectomy for CLM from 2007 to 2010.
Fifteen patients underwent thoracoscopic segmentectomy for CLM. There were five postoperative complications: three asymptomatic pneumothoraces and a small air leak that resolved without intervention. One patient developed a bronchopulmonary fistula requiring thoracoscopic repair. At follow-up, all patients are asymptomatic. One patient has a small amount of residual disease on postoperative computed tomography (CT), and re-resection has been recommended.
Thoracoscopic segmentectomy for CLM is a safe and effective means of lung parenchymal preservation. The approach spares larger airway anatomy and has a complication rate that is comparable with that of thoracoscopic lobectomy. Residual disease can often only be appreciated on postoperative CT scan and may require long-term follow-up or reoperation in rare cases. This lung preservation technique is best suited to smaller lesions.
Background:
Infant acute pain and distress is commonplace. Infancy is a period of exponential development. Unrelieved pain and distress can have implications across the lifespan. This is an update of a previously published review in the Cochrane Database of Systematic Reviews, Issue 10 2011 entitled 'Non-pharmacological management of infant and young child procedural pain'.
Objectives:
To assess the efficacy of non-pharmacological interventions for infant and child (up to three years) acute pain, excluding kangaroo care, and music. Analyses were run separately for infant age (preterm, neonate, older) and pain response (pain reactivity, immediate pain regulation).
Search methods:
For this update, we searched the Cochrane Central Register of Controlled Trials (CENTRAL) in The Cochrane Library (Issue 2 of 12, 2015), MEDLINE-Ovid platform (March 2015), EMBASE-OVID platform (April 2011 to March 2015), PsycINFO-OVID platform (April 2011 to February 2015), and CINAHL-EBSCO platform (April 2011 to March 2015). We also searched reference lists and contacted researchers via electronic list-serves. New studies were incorporated into the review. We refined search strategies with a Cochrane-affiliated librarian. For this update, nine articles from the original 2011 review pertaining to Kangaroo Care were excluded, but 21 additional studies were added.
Selection criteria:
Participants included infants from birth to three years. Only randomised controlled trials (RCTs) or RCT cross-overs that had a no-treatment control comparison were eligible for inclusion in the analyses. However, when the additive effects of a non-pharmacological intervention could be assessed, these studies were also included. We examined studies that met all inclusion criteria except for study design (e.g. had an active control) to qualitatively contextualize results. There were 63 included articles in the current update.
Data collection and analysis:
Study quality ratings and risk of bias were based on the Cochrane Risk of Bias Tool and GRADE approach. We analysed the standardized mean difference (SMD) using the generic inverse variance method.
Main results:
Sixty-three studies, with 4905 participants, were analysed. The most commonly studied acute procedures were heel-sticks (32 studies) and needles (17 studies). The largest SMD for treatment improvement over control conditions on pain reactivity were: non-nutritive sucking-related interventions (neonate: SMD -1.20, 95% CI -2.01 to -0.38) and swaddling/facilitated tucking (preterm: SMD -0.89; 95% CI -1.37 to -0.40). For immediate pain regulation, the largest SMDs were: non-nutritive sucking-related interventions (preterm: SMD -0.43; 95% CI -0.63 to -0.23; neonate: SMD -0.90; 95% CI -1.54 to -0.25; older infant: SMD -1.34; 95% CI -2.14 to -0.54), swaddling/facilitated tucking (preterm: SMD -0.71; 95% CI -1.00 to -0.43), and rocking/holding (neonate: SMD -0.75; 95% CI -1.20 to -0.30). Fifty two of our 63 trials did not report adverse events. The presence of significant heterogeneity limited our confidence in the findings for certain analyses, as did the preponderance of very low quality evidence.
Authors' conclusions:
There is evidence that different non-pharmacological interventions can be used with preterms, neonates, and older infants to significantly manage pain behaviors associated with acutely painful procedures. The most established evidence was for non-nutritive sucking, swaddling/facilitated tucking, and rocking/holding. All analyses reflected that more research is needed to bolster our confidence in the direction of the findings. There are significant gaps in the existing literature on non-pharmacological management of acute pain in infancy.
The size of congenital pulmonary malformation (CPM) in infants might interfere with the feasibility of thoracoscopic resection. This study was undertaken to evaluate the impact of the size of CPM on the applicability of video-assisted thoracic surgery (VATS) in infants.
Twenty-two infants were operated on for CPM from November 2000 to June 2009. The intra- and postoperative course was analyzed retrospectively from patient charts. Preoperative scans were evaluated blindly by a radiologist to calculate the relation between the maximum size of the lesion and the thoracic diameter in VATS and open procedures.
VATS was performed in 14 (64%) of the 22 patients and thoracotomy in 8. VATS was successfully performed in 11 (79%) of the 14 patients, whereas VATS was converted to thoracotomy due to lack of overview in 3 (21%). The mean relative size of CPM at preoperative imaging was 0.34 ± 0.05 (range: 0.3-0.4) in patients who received successful VATS, 0.57 ± 0.06 (range: 0.5-0.6) in converted cases, and 0.68 ± 0.10 (range: 0.5-0.8) in infants who underwent thoracotomy. The relative CPM size was significantly lower in successful VATS than in cases of conversion (P<0.01) and thoracotomy (P<0.01).
The relative size of CPM at preoperative imaging might be useful information for a decision-making on the use of VATS in infants. A relative CPM size below 0.5, which is less than half of the thoracic diameter, indicates a good feasibility for thoracoscopic resection of CPM. A larger size may indicate that VATS might be technically difficult.
There are still few reports on thoracoscopic lobectomy in neonates. The rate of prenatally diagnosed congenital cystic lung diseases is increasing, and such diseases appear to be a good indication for thoracoscopic lobectomy.
We performed a prospective trial of thoracoscopic lobectomy for all congenital cystic lung diseases in children. Complete thoracoscopic lobectomy was performed by employing carbon dioxide insufflation without single lung ventilation. The results were analyzed, comparing patients with pre- and postnatal diagnoses.
Seven patients were prenatally diagnosed with congenital pulmonary airway malformations (CPAM) between January 2008 and August 2009, and all but one underwent surgery during the neonatal period (prenatal group). Nine patients underwent surgery after infection subsided due to lesions (7 CPAM, 2 intrapulmonary sequestration) postnatally identified by infection presence (median 2 years; range 15 days to 14 years) (postnatal group). The prenatal group showed a significantly lower rate of adhesions, shorter operation time, and smaller volume of blood loss. There were two conversions in the postnatal group. There were two postoperative complications (persistent air leak and phrenic nerve paralysis) in the prenatal group. The esthetic results were good.
Thoracoscopic lobectomy for congenital cystic lung diseases in neonates was practicable, with good esthetic results. Dissection was easier with significantly less blood loss in patients without adhesions, even in neonates. However, this procedure necessitated highly skilled endoscopic maneuvers within a small working space.
This study compares the outcome between thoracoscopic and thoracotomy resection of congenital lung lesions.
From November 2005 to August 2007, 14 consecutive cases of video-assisted thoracoscopic (VATS) lung resections have been performed in our institution. A retrospective review comparing these cases to the previous open thoracotomies for lung resection was performed. Intraoperative and early postoperative results were compared.
The mean age for VATS resection was 10 months compared with 7 months for thoracotomy. There were no major intraoperative complications. One case was converted from thoracoscopy to thoracotomy, and there was one anesthetic failed attempt of VATS resection, which was then performed open. Seven VATS resections and 6 thoracotomies were for congenital cystic adenomatous malformations. Intraoperative chest drains were used for all VATS resections but only 10 of the 14 thoracotomies, one of which developed a tension pneumothorax within hours of discharge. Perioperative outcomes including time to removal of chest drain, length of postoperative intravenous opioid requirement, and hospital stay were similar for both groups. Three had postoperative complications. Operative time was significantly lower in the thoracotomy group (124 minutes compared with 170 minutes, P < .05). The subgroup of congenital lobar emphysema had a particularly prolonged VATS resection time of 220 vs 155 minutes (P < .05). The thoracotomy group was more likely to receive adjuvant regional anesthesia (12 of 14 compared with 5 of 14).
Thoracoscopic resection of lung lesions results in longer operative time but is a safe and feasible alternative to open thoracotomy. Congenital lobar emphysema is a subgroup more challenging thoracoscopically, and it is recommended that these should be preselected for open surgery.
Congenital cystic adenomatous malformation of the lung (CCAM) is a rare lung lesion easily diagnosed on prenatal scan. The pathology of fetal lesions differs from postnatal lesion, hence the need for separate classifications during the different stages of development. Fetuses with CCAMs and hydrops have a poor prognosis and may be candidates for prenatal intervention where available. Most prenatally diagnosed CCAMs have a favourable outcome. Early surgery is required for symptomatic babies. Management of prenatally diagnosed asymptomatic lesions remains controversial, with the options of conservative management with CT scan surveillance or surgical excision. Surgical excision is favoured by many centres because of the risk of infection and malignant transformation if the CCAM remains in situ. Surgical outcomes are excellent.
A type I congenital cystic adenomatoid malformation (CCAM) in the left lower lobe was removed from a 11‐year‐old boy with a 3‐month history of recurrent pneumonia. As incidental finding, a bronchioloalveolar carcinoma (BAC) was found in the lung parenchyma adjacent to the cyst. A left lower lobectomy was performed. At 18 months after surgery the patient is well and free of neoplastic disease. To the best of our knowledge, this association has not been reported previously in a pediatric patient. Malignancies complicating CCAM are rarely seen, but have been reported in adults. Including our case, eight cases of BAC and five cases of rhabdomyosarcoma (RMS) in association with CCAM have been reported so far. As CCAM can host metaplastic mucous cells, primitive mesenchymal cells and differentiated but poorly organized striated muscle fibers, it has been proposed that CCAM may act as a predisposing condition for oncogenesis. Our experience adds further support that CCAM can act as a premalignant lesion. Previous reports of both BAC and RMS in asymptomatic CCAM suggest prompt resection shortly after diagnosis. Pediatr. Pulmonol. 1998; 25:62–66. © 1998 Wiley‐Liss, Inc.
Dissatisfied with standard techniques for pulmonary diagnosis in children, we have evaluated the usefulness of thoracoscopy for diagnosis of intrathoracic pathology. Between July 1, 1975, and May 1, 1978, 65 thoracoscopy procedures have been performed in 57 children at the University of Florida. Thirty-four procedures were performed in immunosuppressed patients to rule out Pneumocystis carinii pneumonia. Twenty of these patients were proven to have Pneumocystis pneumonia, a diagnostic accuracy of 100%. Twelve nonimmunosuppressed patients underwent thoracoscopy for the diagnosis of persistent pulmonary infiltrates with a 100% diagnostic accuracy. Fifteen procedures were performed for the diagnosis of intrathoracic tumors. In two patients, previously unsuspected areas of involvement were encountered while in two patients false-negative biopsies were obtained. Four patients underwent therapeutic thoracoscopy. In three small infants, unsuccessful attempts were made to unroof pulmonary cysts through the thoracoscope and one patient underwent a talc poudrage. Thoracoscopy has proven to be a safe and rapid procedure which may be performed under local anesthesia without need for endotracheal intubation. In patients with pulmonary infiltrates, the accuracy has been 100%. The capability of viewing the entire hemithorax has proven valuable in evaluating children with intrathoracic tumors. Complications have included pneumothorax in six patients and bleeding in two.
We have adopted a total muscle-sparing technique for thoracotomies in infants and children. The technique preserves the latissimus dorsi and serratus anterior muscles and provides excellent exposure for most thoracic and mediastinal operations. Thirty-two such procedures have been performed with only one complication, a small wound seroma. Use of this technique may not only decrease postoperative pain and splinting, but may decrease the incidence of scoliosis and muscle dysfunction found in children having undergone thoracotomies as infants.
Congenital malformations of the lung are rare and vary widely in their presentation and severity. The authors reviewed 25 years of experience with specific reference to diagnosis, treatment, and outcome. From July 1970 to June 1995, 70 patients were diagnosed with congenital malformations of the lung, which included sequestration (n = 20), foregut anomalies (n = 20), congenital lobar emphysema (CLE; n = 10), congenital cystic adenomatoid malformation (CCAM; n = 5), benign lung cysts (n = 6), lung aplasia/ hypoplasia (n = 4), and other miscellaneous disorders (n = 5). All patients with pulmonary hypoplasia presented at birth. With the exception of one patient, infants with CCAM and CLE presented before 5 months of age. All other patients presented from birth to 16 years of age. A prenatal diagnosis was accurate in two patients. Although prompt surgical management is the rule, the exceptions were children with bilateral lung involvement. Corrective surgery was delayed in some patients in whom extended respiratory support was required or in whom the delay led to an increase in pulmonary reserve. Extracorporeal membrane oxygenation (ECMO) was used in two patients pre- and postoperatively to manage persistent pulmonary hypertension. This review, representing the largest series of congenital lung lesions, showed that (1) prenatal diagnosis is useful but generally does not change the outcome; (2) computerized tomography (CT) is the optimum postnatal diagnostic imaging modality if chest radiography is not definitive; (3) ECMO can be an adjunct in treating associated pulmonary hypertension; (4) pulmonary resection (lobectomy) is the surgical procedure of choice for most lesions; (5) expected survival is good; and (6) pulmonary hypertension is the most common cause of mortality.
Prenatal ultrasonographic (US) detection of congenital adenomatoid malformation (CAM) was made in 18 fetuses at 17 - 36 weeks' gestation and managed in our institution during a 10-year period (1985-1994). The lesion was left-sided in 13 cases, right-sided in 4, and bilateral in 1. According to Stocker's classification, 12 cases were type I, 4 type II, and 2 type III. The prenatal course was followed with serial US examinations in 13 cases; the size of the lesion was stable in 8 and decreased in 5. Mediastinal shift was usually observed, and amniotic fluid volume was increased in 4 cases. One fetus was aborted. Six infants presented with respiratory distress syndrome and required neonatal surgery; delayed surgery was performed in 9 cases. Spontaneous regression of the lesion was observed on follow-up in 2 cases. Surgery consisted in lobectomy in 8 cases and segmentectomy in 6. The presence of fetal hydrops, type III lesions, and bilateral lung involvement are prenatal factors known to be associated with a poor prognosis. However, this series and a review of the literature suggest that caution should be observed with regard to the initial impression when counseling the parents regarding prognosis.
A type I congenital cystic adenomatoid malformation (CCAM) in the left lower lobe was removed from a 11-year-old boy with a 3-month history of recurrent pneumonia. As incidental finding, a bronchioloalveolar carcinoma (BAC) was found in the lung parenchyma adjacent to the cyst. A left lower lobectomy was performed. At 18 months after surgery the patient is well and free of neoplastic disease. To the best of our knowledge, this association has not been reported previously in a pediatric patient. Malignancies complicating CCAM are rarely seen, but have been reported in adults. Including our case, eight cases of BAC and five cases of rhabdomyosarcoma (RMS) in association with CCAM have been reported so far. As CCAM can host metaplastic mucous cells, primitive mesenchymal cells and differentiated but poorly organized striated muscle fibers, it has been proposed that CCAM may act as a predisposing condition for oncogenesis. Our experience adds further support that CCAM can act as a premalignant lesion. Previous reports of both BAC and RMS in asymptomatic CCAM suggest prompt resection shortly after diagnosis.
The development of thoracoscopic surgery has made many procedures possible, including the treatment of mediastinal cysts in children. The authors report their experience with this procedure between 1992 and 1997.
Surgery was performed on 22 children aged from 1 month to 9 years (median, 27 months), weighing 5 to 49 kg (median, 12.5 kg). Diagnosis was made by antenatal ultrasound scan in six cases (27%), with a chest x-ray performed for respiratory symptoms in 14 cases, and with a chest x-ray performed for positive tuberculin intradermoreaction in two cases. Decision to resect the cyst was determined by thoracoscopy in 21 of the 22 cases, and by open surgery in one case only (subcarinal compressive cyst with left lung distension and a mediastinal shift).
Eighteen of the 21 (86%) cases were treated successfully by thoracoscopy. In three cases of bronchogenic cysts, we performed an associated thoracotomy because the dissection was too difficult and dangerous. In three cases, a small part of a common wall between the cyst and the bronchus was not removed. The pathological diagnosis was bronchogenic cysts in 15 cases (71%), pleuropericardiat cysts in three cases (14%), esophageal duplication in two cases (10%), and cystic hygroma in one case (5%). Two postoperative complications were observed: one esophageal wound and a case of recurrent pneumothorax after chest tube removal. Patients were discharged after 2 to 11 days (median, 3 days). Follow-up was uneventful.
Treatment of mediastinal cyst by thoracoscopy is feasible in most cases. Compressive cysts with lung distension and mediastinal shift remain a contraindication. If the cysts have a common wall with the bronchus or esophagus, or if they are subcarinal, the dissection may be difficult and dangerous, and thoracotomy may be preferable.
Eight children underwent minimal-access surgery (MAS) for duplications of foregut derivatives. The efficacy and safety of this approach are reviewed. The seven patients with mediastinal lesions had video-assisted thoracoscopic resection. One lesion presented as a subdiaphragmatic esophageal diverticulum, which was excised laparoscopically. Between March 1991 and October 1997, eight children were treated. Mean age was 27 months and mean weight was 11. 4 kg. Mean operating time was 106 min, and mean postoperative hospital stay was 4.5 days (median = 2 days). Persistent air leaks occurred in two patients who had centrally-located bronchogenic cysts. One of these, who had undergone subtotal excision with laser photoablation of the remaining cyst mucosa, developed a recurrence that was excised at thoracotomy. We conclude that esophageal and bronchogenic cysts and duplications may be safely excised by MAS in children, with excellent cosmetic and functional outcome. Two technical points are noted: (1) a thoracostomy tube is required for central mediastinal lesions; and (2) complete excision is required to prevent recurrence.
Sequestration with associated cystic adenomatoid malformation is rare. A study was undertaken to determine whether pulmonary sequestration associated with congenital cystic adenomatoid malformation has a more favourable natural history than that of sequestration without associated cystic adenomatoid malformation.
An outline of the postnatal work up leading to the management of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation diagnosed antenatally as pulmonary malformation is presented and the indications for surgical intervention are discussed.
In five infants in whom an antenatal ultrasound scan had detected a congenital lung malformation at 18-19 weeks gestation a final diagnosis of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation was made postnatally. Postnatal ultrasound and computerised axial tomographic scans confirmed the diagnosis of sequestration by delineating anomalous vascular supply. Cystic changes were also observed in the basal area of the sequestration in all patients. Four children remained asymptomatic and one infant presented at 10 months of age with pneumonia. The mean age at surgical resection was 6.8 months (range 2-10). Histopathological examination confirmed intralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in two patients and extralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in three patients. The mean period of follow up was four years (range 1-8). The children remain well and are developing normally.
The importance of seeking an anomalous blood supply in children with congenital lung lesions is emphasised. Pulmonary sequestration and congenital cystic adenomatoid malformation probably share a common embryogenesis despite diverse morphology. The natural history of antenatally diagnosed lung masses is variable. Early postnatal surgical resection of pulmonary sequestration with cystic adenomatoid malformation is recommended. Surgical excision should be conservative, sparing the normal lung parenchyma.
Currently, congenital cystic adenomatoid malformation of the lung (CCAM) is often diagnosed antenatally by ultrasound, allowing prompt and appropriate medical and surgical management after birth. The authors report 21 cases of CCAM admitted from 1988 to 1997 to a neonatal intensive care unit and treated by high-frequency oscillation (HFO) and early surgery. Six infants developed respiratory distress, of whom 4 required ventilation by HFO. HFO was also the mode of ventilation used in all cases except 1 during the perioperative period. There was no death from respiratory failure. The authors emphasize the usefulness of antenatal diagnosis, the efficiency of HFO in cases with severe respiratory failure, and well-tolerated early surgery.
The aim of this study was to evaluate the technique of video-assisted thoracic surgery (VATS) in lung resections in infants and children.
From December 1992 to December 1998 113 consecutive patients, ages 3 weeks to 19 years, underwent VATS for biopsy or resection of various lung pathology. This included 88 wedge biopsies, 12 resections of bullous or cystic disease, 9 lobectomies or segmental resections, and 4 bronchogenic cysts.
All procedures were completed successfully. Two patients with metastatic disease had surgery converted to a standard thoracotomy for extensive resections. The average operating time for a wedge biopsy of 2 sites was 26 minutes and 210 minutes for a lobectomy. The average hospital stay after wedge resection was 1.1 days. There were no complications related to the VATS approach.
VATS is a safe and effective technique in the diagnosis and treatment of pediatric pulmonary disease.
The spectrum of congenital cystic disease of the lung ranges from hydrops and neonatal respiratory distress to asymptomatic lesions. Surgical management is dictated by the presence of symptoms, recurrent infection, and the potential risk of malignant transformation.
Since 1995, all consecutive patients with congenital cystic lung lesions underwent follow-up for symptoms, treatment, and correlation of presumptive with pathological diagnosis.
Twelve cystic lung lesions were identified. Seven were diagnosed with mediastinal shift in utero; in 6 of 7, the shift subsequently resolved. Overall, 6 of 7 lesions that were followed up serially decreased in size. Two patients were symptomatic in utero; 1 underwent thoracoamniotic shunting, 1 pleurocentesis for impending hydrops. Postnatally, these 2, and 2 other newborns required urgent surgery. Five of 8 asymptomatic patients had elective resection by 16 months, and 4 await operation. In 6 of the 9 surgical cases (67%), there was a discrepancy between preoperative and pathological diagnosis. There were 4 hybrid congenital cystic adenomatoid malformation (CCAM)/sequestrations.
At least 6 of 7 congenital cystic lung lesions decreased in size regardless of gestational age or presence of mediastinal shift. Antenatal intervention is therefore rarely indicated. Hybrid morphology may necessitate resection of stable, asymptomatic lesions to prevent the theoretical concern for associated malignancies as well as other complications of CCAM.
This study was designed to assess the effect of prenatal sonographic diagnosis on the treatment of congenital cystic adenomatoid malformation of the lung.
The medical records of 27 patients with pathologically proven congenital cystic adenomatoid malformations were retrospectively reviewed. Patients were divided into four groups based on mode of presentation: with or without abnormal findings on prenatal sonography and with or without symptoms at birth. Age at diagnosis, age at surgical intervention, complications, and length of hospital stay were recorded for each group.
Twenty-seven patients with 31 proven congenital cystic adenomatoid malformations were included. Eleven patients underwent prenatal sonography establishing the diagnosis (6 asymptomatic at birth, 5 symptomatic), and 16 did not have a prenatal diagnosis (10 asymptomatic at birth, 6 symptomatic). In the symptomatic populations, prenatal diagnosis had no impact on age at surgery, length of stay, or surgical complication rate (p = 0.78-0.83). In the asymptomatic population, prenatal diagnosis allowed early diagnosis (p < 0.001) and resection in the asymptomatic period. It was also associated with a shorter length of stay at the time of surgical resection (mean time, 4.2 days for patients with prenatal diagnosis versus 12.9 days for those without it;p < 0.001) and with a trend toward lower serious complication rate (3 patients without prenatal diagnosis versus 1 patient with it).
Prenatal sonography provides the radiologist a means to identify congenital cystic adenomatoid malformations in a population of infants who are asymptomatic at birth. Surgical intervention in the asymptomatic infant is associated with a shorter length of stay, a trend toward fewer complications, and decreased medical cost compared with intervening after symptoms develop.
The goal of this study was to identify the proportion of sequestrations that were atypical or associated with other entities, such as congenital cystic adenomatoid malformations, communicating bronchopulmonary foregut malformations, bronchogenic cyst, and scimitar syndrome.
All charts of patients with pulmonary sequestration admitted at 2 children's hospitals from 1982 to July 1999 were reviewed retrospectively. The authors included all anomalies with a systemic arterial supply or without bronchial connection.
Only 22 of the 39 patients (56%) had a classic isolated extralobar or intralobar sequestration, whereas the others presented with a spectrum of anomalies. Of the 13 cases diagnosed prenatally, 85% were asymptomatic at birth. In contrast, 26 cases diagnosed postnatally were all symptomatic, with those patients less than 2 weeks old presenting with various degrees of respiratory distress, and those older than 2 weeks old presenting with respiratory infections. The correct diagnosis was made preoperatively in 59% of cases. Only 4 patients did not undergo resection of their lesion, of which, 1 underwent interventional radiology with embolization of the anomalous arterial supply. Follow-up issues of importance included pneumonia, asthma, gastroesophageal reflux, and pectus excavatum.
Sequestrations represent a spectrum of anomalies that overlap with other lung lesions. To facilitate management, they should be described according to their (1) connection to the tracheobronchial tree, (2) visceral pleura, (3) arterial supply, (4) venous drainage, (5) foregut communication, (6) histology, (7) mixed/multiple lesions, and (8) whether there are associated anomalies. Surgeons should be aware that approximately 50% of sequestrations could be atypical or associated with other anomalies. This should be kept in mind when weighing the benefits of resection versus conservative management of pulmonary sequestrations.
Thoracoscopy has been accepted as a technique in pediatric surgery for diagnosis of thoracic pathology, but there has been little experience using it as a therapeutic modality as well. The purpose of this report is to describe and critically evaluate a 7-year experience with thoracoscopic diagnosis and resection of mediastinal masses in infants and children.
From February 1993 to June 2000, 39 patients presented with mediastinal masses and no tissue diagnosis. Age ranged from 5 months to 18 years old and weight from 3.6 to 110 kg. Twelve children had anterior mediastinal masses, 27 posterior. The patients were positioned in a modified prone or supine position, and single lung ventilation was performed on the contralateral side. Three or 4 valved trocars were utilized with 3 and 5 mm instrumentation.
A total of 38 of 39 procedures were completed successfully endoscopically. The procedure in 1 patient with a sarcoma was converted to thoracotomy because of extensive disease. Operating times ranged from 20 to 185 minutes. Diagnosis was obtained in all cases, and complete excision was performed in 33. All children were extubated in the operating room; 8 chest tubes were placed but removed within 24 hours. Hospital stay ranged from 12 hours to 4 days. Pathology included 12 foregut duplications, 7 ganglioneuromas, 6 neuroblastomas, 6 lymphomas, 3 teratomas, 2 sarcomas, and 3 other lesions.
Thoracoscopy is a safe and effective method to evaluate, biopsy, and in most cases resect lesions of the anterior and posterior mediastinum in infants and children.
The aim of this report is to assess the technique and outcome of thoracoscopic lobectomy for asymptomatic prenatally diagnosed lung lesions.
From June 1999 to March 2002, 14 consecutive asymptomatic patients with a prenatal diagnosis of congenital cystic adenomatoid malformation or pulmonary sequestration, ages 3 to 15 months, underwent postnatal thoracoscopic lobectomy. Single-lung ventilation and controlled pneumothorax with low pressure (4 torr) and low flow (1.0 L/min) were used in all. Follow-up ranged from 4 to 35 months.
All procedures were completed successfully using 3 ports. Rather than using stapling devices or clips, pulmonary vessels were sealed and the fissure completed (when necessary) with the Ligasure thermal energy device. Eleven lesions were on the left (10 lower lobe), and 3 were in the right lower lobe. The mean operating time was 110 minutes. The average hospital stay was 38 hours. There were no intraoperative or postoperative complications.
This is the first report of a completely thoracoscopic technique for pulmonary lobectomy in small children. Thoracoscopic lobectomy is a relatively quick and safe procedure, and the cosmetic result is excellent. Early resection obviates the risk of infection in these lesions.
Although much is known about the prenatal course of cystic adenomatoid malformations (CCAM), the postnatal course of asymptomatic lesion is less well documented. The authors studied the pre- and postnatal course and treatment of asymptomatic CCAM.
The authors reviewed the files of all patients referred to Necker-Enfants Malades hospital with an antenatal diagnosis of CCAM and asymptomatic at birth.
Files of 29 patients were studied. The first x-ray film was considered normal in 12 cases (41.3%). Computed tomography was normal in 4 cases and showed cystic lung malformations in the other patients. Postnatally, clinical manifestations occurred in 3 patients (10.3%). CCAM vanished in 6 cases. Surgical resection of CCAM was performed in 17 cases (58.6%). All the patients currently are asymptomatic.
CCAM can shrink or vanish during pregnancy and antenatal ultrasound findings are not predictive of the postnatal course. Thus, all infants with prenatal diagnosis of CCAM require postnatal evaluation. Normal radiographic findings at birth do not rule out CCAM persistence on CT. The treatment of asymptomatic CCAM is controversial. Surgery may be advocated because of the low morbidity and the prevention of late complications, above all, cancer. The surgical indications of small (<3 cm) and asymptomatic lesions should be discussed on a case-by-case basis with the parents.
Background/purpose:
The natural history of parenchymal lung lesions such as congenital cystic adenomatoid malformation (CCAM) and pulmonary sequestration (PS) has been altered by the advent of antenatal ultrasonography. Initial reports were characterized by a high (about 30%) incidence of adverse features (eg, hydrops) and a poor outcome and did not accord with our recent experience. The authors have reviewed the outcome of fetuses that had been diagnosed in a large tertiary referral fetal medicine unit with the aim of delineating current experience. The term cystic lung disease was used throughout to avoid unjustifiable histologic precision.
Methods:
The scans of all fetuses that had been diagnosed with cystic lung disease between January 1995 and July 2001 were reviewed. The outcome of each pregnancy was established, and, where possible, all infants underwent appropriate investigations, including thoracic computed tomography (CT) scans.
Results:
Sixty-seven fetuses had a cystic lung abnormality diagnosed from January 1995 to July 2001. The median (range) age at diagnosis was 21 (19 to 28) weeks. The lesion was right sided in 29 (43%), left in 36 (54%), and bilateral in 2 (3%); it was characterized as dominantly macrocystic in 27 (40%), microcystic in 35 (52%), and mixed in 5 (8%). Mediastinal shift was present in 30 fetuses (45%). Severe signs of fetal distress (eg, hydrops) were present in 5 fetuses (7%). Antenatal intervention was performed in 4 fetuses (thoraco-amniotic shunts [n = 3] and percutaneous intrauterine laser therapy [n = 1]). Sixty-four (96%) of the fetuses were born alive. There was 1 termination of pregnancy and 2 intrauterine deaths (all severe microcystic lesions). Forty-two infants (63%) underwent thoracotomy and appropriate excisional surgery at a median of 7.5 months (range, 1 day to 34 months). Two infants (which included the fetus having intrauterine laser therapy) died early in the postnatal period. Both were large microcystic lesions and had antenatal features of severe fetal distress. Twelve infants were investigated in the postnatal period but did not undergo surgery. Ten infants were not appropriately investigated or were lost to follow-up. Histologic examination showed definitive diagnostic features of CCAM (n = 25) or PS (n = 6). Other lesions with hybrid features of both were also seen (n = 11). There was a degree of correlation between antenatal ultrasound features (size of cyst [P =.03], in-utero behavior [P =.06], mediastinal shift [P = 0.05]) and the need for surgery but not with the final histologic diagnosis. Surgical excision was required in 45% of lesions showing late-gestation "resolution."
Conclusions:
Antenatally diagnosed "cystic lung disease" has an excellent prognosis in the absence of signs of severe fetal distress. The need for surgery should be based on appropriate postnatal investigations (eg, CT scans), rather than on antenatal behavior.
Thoracoscopy has an expanding role in the treatment of FD cysts (bronchogenic cysts and esophageal duplications). We examined this trend in our patients and reviewed our overall experience.
All charts of children undergoing surgery for foregut duplications (FDs) in 2 pediatric hospitals between 1992 and 2003 were retrospectively reviewed. Data gathered included age, weight, symptoms, diagnostic tests, operative technique, postoperative course, complications, and outcome.
There were 39 children, with FD resected by thoracotomy in 21 patients, thoracoscopy in 11 patients (no conversions to open), cervical incision in 6 patients, and laparotomy in 1 patient for an FD near the gastroesophageal junction. Diagnosis was made by antenatal ultrasound in 7 cases. Four of these neonates had tachypnea or cough, and the rest were asymptomatic. Seventy-five percent of patients diagnosed postnatally presented with respiratory symptoms. Excision of isolated FD (without lung resection) was compared between those who had a thoracotomy (n = 16) vs thoracoscopy (n = 11). The age, weight, operating time, and anesthesia time were not different between the 2 groups. However, the thoracoscopy group had significantly fewer chest tube days (1.6 vs 3.3 days) and a shorter hospital stay (2.6 vs 6.6 days). Intraoperative complications consisted of tracheal injury in 3 patients (2 thoracotomy, 1 thoracoscopy) and esophageal mucosal injury in 2 patients (both thoracotomy), which were all recognized and repaired.
Foregut duplications may present in a variety of ways and locations. Thoracoscopy is advantageous for isolated intrathoracic FDs.
Can all congenital cystic lung lesions be treated conservatively, without the need for surgery? Our purpose here is to present the morbidity associated with symptomatic cystic lung lesions which have failed to respond to medical treatment. In the past 8 years, 22 consecutive cystic thoracic lesions were retrospectively assessed for clinical presentation, diagnostic modalities, operative findings, technical tribulations, and outcome. The endpoint was complete cessation of recurrent pneumonia and dysphagia. Age at presentation was 7.7 +/- 2.2 years, with 4 +/- 2 episodes per year of lower respiratory tract infection, which had been treated for the past 2.6 +/- 0.3 years. Cough and dyspnea (100%) were the common symptoms, with episodes of cyanosis occurring in 58%. Other significant clinical presentations were dysphagia (55%), failure to thrive (55%), chest pain (46%), haemoptysis (18%), and pleuritic pain (18%). Definitive growth was seen in 91% of the excised specimens. Preoperative morbidity resulted from intractable pneumonia, dysphagia, and failure to thrive. Surgical excision was curative. All 22 children after resection are thriving, with an absence of pneumonia and dysphagia, with normal ventilation/perfusion scans, at 48 +/- 6 months of follow-up. In conclusion, surgical excision of a symptomatic cystic lung lesion that has not responded to medical treatment is recommended.
Management of asymptomatic congenital cystic adenomatoid malformation (CCAM) is controversial. The natural history of untreated asymptomatic CCAM is unknown, although most surgeons recommend resection of these lesions to prevent future infection. The aim of this study was to determine the relative surgical risk of resection compared with the risk of observation for these patients.
A retrospective review of hospital records between 1996 and 2002 in a tertiary care pediatric referral center was conducted. All perinatally (prenatal or neonatal) diagnosed CCAMs were included. In addition, patients presenting with late diagnosis of CCAM were also reviewed.
Forty-eight children had CCAM diagnosed perinatally. Thirteen of these were symptomatic and required surgery within 6 months; these were excluded from the analysis. Of the 35 asymptomatic infants, 6 were operated on electively before 6 months of age (median age, 4.5 months). The other 29 asymptomatic infants were followed up for more than 6 months. Of these, 9 remained asymptomatic and were eventually operated on electively (median age, 13 months). Three (10%) had CCAM infections at 7, 8, and 11 months of age and required resection. The remaining 17 children have not undergone resection and are still asymptomatic (median follow-up, 3 years). An additional 12 patients presented with a late diagnosis of CCAM. All of these presented with complications (infection or pneumothorax) and underwent resection (median age, 6 years). Overall, the complication rate after resection of an asymptomatic CCAM was not significantly different from those of resected CCAM that had already developed infection or pneumothorax (P =.64).
Ten percent of perinatally diagnosed asymptomatic patients had 5 complications requiring surgery during follow-up. The true incidence is probably higher given the relatively short follow-up in our series. Morbidity after resection of a complicated CCAM was not statistically significantly higher than after elective resection for an asymptomatic CCAM. Although conservative management of asymptomatic CCAM may be warranted, a more extended period of follow-up is necessary before this approach can be recommended.
Foregut duplication cyst; BrC, Bronchogenic cyst; EDC, Esophageal duplication cyst; CT, Computed tomography; ICD, Intercostal drain
- Ccam
CCAM, Congenital cystic adenomatoid malformation of the lung; FDC, Foregut duplication cyst; BrC, Bronchogenic cyst; EDC, Esophageal duplication cyst; CT, Computed tomography; ICD, Intercostal drain. * Corresponding author. Tel.: +44 121 333 8083; fax: +44 121 333 8081. E-mail address: dakshesh.parikh@bch.nhs.uk (D.H. Parikh).
Congenital cystic adenomatoid malformation. Impact of prenatal diagnosis and chang-ing strategies in the treatment of the asymptomatic patient
- Marshall Kw Ce Blane
- Teitelbaum
- Dh
Marshall KW, Blane CE, Teitelbaum DH, et al. Congenital cystic adenomatoid malformation. Impact of prenatal diagnosis and chang-ing strategies in the treatment of the asymptomatic patient. AJR 2004;175:1551 -4.
Perinatally diagnosed asymptomatic congenital cystic adenomatoid malformation: to resect or not?
- Aziz D Jc Langer
- Tuuha
- Se
Aziz D, Langer JC, Tuuha SE, et al. Perinatally diagnosed asymptomatic congenital cystic adenomatoid malformation: to resect or not? J Pediatr Surg 2004;39(3):329 -34 [discussion 329-34].
Bronchiolo-alveolar carcinoma arising in congenital cystic adenomatoid malformation in a child: a case report and review on malignancies originating in congenital cystic adenomatoid malformation
- Granata