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Acute Appendicitis After Diaphragmatic Hernia After Pediatric Liver Transplant
Abstract
Multiple complications in liver transplant have been described in the literature. However, appendicitis and diaphragmatic hernia have rarely been reported after solid-organ transplant. The clinical presentation of appendicitis is similar to that of nontransplant patients, but complications are more frequent, because the majority of the patients do not have leukocytosis. Diaphragmatic hernia can present with a variety of atypical clinical symptoms. In this report, 1 patient who developed a diaphragmatic hernia and appendicitis after liver transplant is presented. A 2-year-old boy with end-stage liver cirrhosis owing to progressive familial intrahepatic cholestasis type-2 received a living-donor liver transplant. The posttransplant course was complicated. The diagnosis of diaphragmatic hernia was confirmed by thoracoabdominal computed tomography, and we decided to proceed with surgical repair. The patient had evidence of perforation, and the appendix was removed. After repositioning the intestine in the abdomen, a chest tube was placed, and the defect repaired with interrupted polypropylene sutures. The patient recovered after surgery without untoward sequelae.
... [1] The lifetime risk of an AAp episode is 8.6% in male and 6.7% in female patients. [1][2][3][4][5] Epidemiologic studies state that the risk of undergoing an appendectomy at any point in their lives in male and female patients is 12% and 23%, Cite this article as: Sarıcı KB, Akbulut S, Koç C, Tuncer A, Yılmaz S. Liver transplant versus non-liver transplant patients underwent appendectomy with presumed diagnosis of acute appendicitis: Case-control study. Ulus Travma Acil Cerrahi Derg 2020;26: 705-712. ...
... [5,8,9,11] The first publication regarding AAp in patients with LT was published in 2005 by Abt et al., [11] and since then, 14 articles have been published with one being a review article. [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] Our literature review with 33 LT patients who received appendectomy for AAp has been summarized in Table 3. The studies in the field show that AAp incidence in patients with LT ranges between 0.09%-0.67%. ...
... [2,6,7,13,14] In the remaining patient, a perforation was noticed during laparoscopic exploration and the operation was converted to open surgery. [3] Although laparoscopic surgery is recommended in the early postoperative period, laparoscopic appendectomy may also be performed many years after the transplant surgery. [2,6,7,13,14] The first trocar should always be placed under direct vision during laparoscopic appendectomy. ...
Background:
This study aims to compare liver transplant and non-liver transplant patients who underwent appendectomy with a presumed diagnosis of acute appendicitis.
Methods:
Demographic and clinicopathological features of 13 liver transplant recipients (transplant group) who underwent posttransplant appendectomy with a presumed diagnosis of acute appendicitis were compared with the features of 52 non-liver transplant patients (non-transplant group). They underwent appendectomy with a presumed diagnosis of acute appendicitis during the same time period. The transplant group was matched at random in a 1: 4 ratio with the non- transplant group. While the continuous variables were compared using the Mann Whitney-U test, categorical variables were compared with Fisher's exact test. A p-value of less than 0.05 was considered statistically significant.
Results:
A total of 65 patients aged between one year and 84 years were included in this study. While the age of the 52 patients (32 male and 20 female) in the non- transplant group ranged from 17 years to 84 years, the age of the 13 patients (nine male and four female) in the transplant group ranged from one year to 64 years. Statistically significant differences were noted between both groups concerning WBC (p=0.002), neutrophil (p=0.002), lymphocyte (p=0.032), platelets (p=0.032), RDW (p=0.001), CRP (p=0.009), PNR (p=0.042), WNR (p=0.03), and appendiceal length (p<0.001). The negative appendectomy rate was relatively higher in transplant than the non-transplant group but this difference was not statistically significant (30.8% vs. 21.2%; p=0.477). Perforated acute appendicitis occurred more frequently in the transplant group; however, this difference was not statistically significant (30.8% vs. 9.6%; p=0.070).
Conclusion:
WBC and neutrophil were lower in the LT group; however, the CRP and RDW were higher in the LT group. Further, perforation and negative appendectomy rates were higher in the LT group, although this difference was not statistically significant.
... Previous studies have concluded that acute appendicitis after liver transplantation may present differently than in non-immunosuppressed patients and thus may result in more complications. In agreement with this, open appendectomy after exploratory laparotomy has been more prevalent than laparoscopic appendecto-my in liver transplant recipients [3][4][5][6][7]. Herein, we present the fourth reported case of laparoscopic appendectomy after liver transplantation and review the previously published case reports to examine the incidence and clinical presentation of appendicitis after orthotopic liver transplantation. ...
... Therefore, the delay in seeking medical care was likely the cause of perforation, and perhaps unrelated to transplantation status. Evidence of perforation was reported in two single other case reports [7]. However, out of the studies evaluated for this paper, two did not account for perforation [3,4]. ...
... In one case study of eight patients with appendicitis after liver transplantation, none of the patients had lymphoid hyperplasia on pathological examination, likely because immunosuppression may lead to decreased lymphoid hyperplasia [5]. In liver transplant recipients, the mean age at the presentation of acute appendicitis is 2 (Table 1) [1,3,4,6,7], and lymphoid tissue atrophies with age [12], thus providing additional support for an alternative cause. Therefore, acute appendicitis after liver transplantation is likely caused by particulate matter including a fecalith obstructing the lumen. ...
Acute appendicitis is one of the most common etiologies for acute abdomen. However, fewer than 30 cases of acute appendicitis after liver transplantation have so far been reported in the literature. Previous case studies have concluded that acute appendicitis after liver transplantation may present differently than in non-immunosuppressed patients and thus may lead to more complications. Herein, we describe the fourth case of laparoscopic appendectomy in a 40-year-old female presenting with an acute abdomen, 10 years after orthotopic liver transplantation for autoimmune hepatitis. Additionally, we review the literature, and emphasize the importance for laparoscopic, rather than open appendectomy after liver transplantation. Overall, despite the small number of reported cases of appendicitis after orthotopic liver transplantation, we found the incidence and clinical presentation are similar to patients without liver transplantation. The etiologies for appendicitis in patients after liver transplantation may be different than in those not chronically immunosuppressed, with significantly less lymphoid hyperplasia and increased fecalith and cytomegaloviral infections. Preliminary results showed that laparoscopic appendectomy after liver transplantation results in decreased hospital stays and fewer complications.
... Complications from diagnostic delays are frequent, as well as the difficulty of making the differential diagnosis with other causes of acute abdomen. Only a few case reports and studies are present in literature 1,2,3,4,7,8,10,11 . ...
... In liver transplant patients the cause of appendicitis is not different from that found in patients not immunosuppressed, being the main causes mechanical obstruction and bacterial overgrowth. In addition to these causes, there were already described in literature lymphoid hyperplasia and infections by cytomegalovirus 2,3,7 . ...
Background:
Appendicitis is a common cause of emergency surgery that in the population undergoing organ transplantation presents a rare incidence due to late diagnosis and treatment.
Aim:
To report the occurrence of acute appendicitis in a cohort of liver transplant recipients.
Methods:
Retrospective analysis in a period of 12 years among 925 liver transplants, in witch five cases of acute appendicitis were encountered.
Results:
Appendicitis occurred between three and 46 months after liver transplantation. The age ranged between 15 and 58 years. There were three men and two women. The clinical presentations varied, but not discordant from those found in non-transplanted patients. Pain was a symptom found in all patients, in two cases well located in the right iliac fossa (40%). Two patients had symptoms characteristic of peritoneal irritation (40%) and one patient had abdominal distention (20%). All patients were submitted to laparotomies. In 20% there were no complications. In 80% was performed appendectomy complicated by suppuration (40%) or perforation (40%). Superficial infection of the surgical site occurred in two patients, requiring clinical management. The hospital stay ranged from 48 h to 45 days.
Conclusion:
Acute appendicitis after liver transplantation is a rare event being associated with a high rate of drilling, due to delays in diagnosis and therapy, and an increase in hospital stay.
... Acute appendicitis is the most common surgical emergency around the world and in a study conducted in 2012 in Pennsylvania, USA, there was an increase in the annual incidence from 7.62 to 9.38 per 10,000 between 1993 and 2008 [1]. However, in the postoperative period of solid organ transplantation appendicitis is rare, and only 22 cases (one being a child) [2] occurring in the post-liver transplantation have been reported in the literature so far. ...
... The clinical findings do not differ from common acute appendicitis, and may occur: diffuse abdominal pain, fixed tenderness in the right iliac fosse with or without rebound tenderness, fever, nausea, vomiting and diarrhea [2]- [7]. The laboratory may have leukocytosis, but six of these patients (26%) did not [2] [4]. Imaging tests are extremely useful in such cases. ...
Acute appendicitis is rare in the postoperative period of liver transplantation; only 23 cases were described in the literature to date, including late and immediate postoperative. Our case reports a patient who was presented with acute appendicitis in the immediate post-transplant and died in the subsequent postoperative period. The article reviews the available literature and all cases known until now, commenting on incidence, casual factors, symptoms, diagnostic and management.
... Our patient presented a right-sided abcess appendicular due to a large paraoesophagal hernia. Herniation through an diaphragmatic hernia occurs for 3 reasons: pressure differential between abdominal and thoracic cavities; delayed healing caused by constant motion of the diaphragm; and thin musculature of the diaphragm [8]. In our case, the intrathoracic passage of the abdominal viscera is explained by the surgical history of minor blunt truncal trauma, congenital defect of fixation of the right colon and a large paraoesophagal hernia unnoticed during the first intervention of cholecystectomy. ...
The authors describe the case of intrathoracic appendiceal abscess associated with right diaphragmatic hernia (hiatal hernia) discovered in a 52 years old woman. The surgical treatment consisted of conventional laparotomy appendectomy after reduction of paraoesophagal hernia into the abdominal cavity in the fist time. In second time, we realised a pleural decortication using esophageal hiatus like an uniportal video-assisted thoracic surgery and at the end, reparation of paraoesophagal hernia. The clinical course was satisfactory. A review of the literature allowed us to understand and discuss the diagnostic and surgical approches of this association of two pathologicals entities, benign and anodyne in its isolated and uncomplicated clinical forms. The available literature on intrathoracic appendicitis is reviewed.
... T he incidence rate of acute appendicitis following liver transplantation (LT) has been reported as 0.09% to 0.49%, and thus far, the largest case series cited in the English-language medical literature are 6 reports, which include a total of 32 cases worldwide [1][2][3][4][5][6][7][8][9][10]. The present study found an incidence of appendicitis developing after LT of 0.35% (7/1990), and is the third largest case series in the literature. ...
OBJECTIVE
The incidence of acute appendicitis after liver transplantation (LT) is extremely low, reported to be 0.09% to 0.49%, but the efficacy of the Alvarado score in this patient group has not been studied. This study was an investigation of the clinical management of patients who developed acute appendicitis after LT and the usefulness of the Alvarado score in the diagnosis.
METHODS
The study was performed using the data of 7 patients treated for acute appendicitis who were among 1990 patients who underwent LT between March 2002 and July 2017. The Alvarado score of the patients was calculated and reliability was analyzed.
RESULTS
In this study, the incidence of acute appendicitis in LT patients was 0.35%. All of the patients were in the adult age group; 86% were male. The mean age was 46.4±10.7 years and the timeframe for the development of appendicitis after transplantation was a median of 12 months (range: 4-101 months). The median Alvarado score was 7 (range: 5-9). All of the patients had an Alvarado score above 5 and 71% had a score of 7 or more.
CONCLUSION
Acute appendicitis is very rare in LT patients. As with non-transplant patients, Alvarado scoring can be safely performed in LT patients.
Background
Acquired diaphragmatic hernia (DH) following liver transplantation (LT) is usually considered a surgical emergency. Interplay of contributing elements determines its occurrence but, in children, LT with partial liver grafts seems to be the most important causative factor.
Methods
This retrospective study describes the clinical scenario and outcomes of 11 patients with acquired DH following LDLT.
Results
During the study period, 1109 primary pediatric LDLT were performed (0.8% DH). The median age and BW of the recipients with DH at transplantation were 17 months and 11.1 kg, respectively; 63.7% of the cases had a weight/age Z-score of less than −2 at transplantation. The median interval between transplantation and diagnosis of DH was 114 days (32–538 days). A total of 6 (54.5%) of the patients had bowel obstruction due to bowel migration into the hemithorax. Ten defects were right-sided. Three patients required enterectomy and enterorrhaphy. Two patients required a new bilioenteric anastomosis, and one of them had complete necrosis of the Roux-in-Y limb. The patient with left-side DH presented gastroesophageal perforation.
Conclusion
Most defects necessitate primary closure as the first treatment, and recurrence is rare. The associated problems encountered, especially related to intestinal complications, can determine increased morbidity following DH repair. Early diagnosis and intervention are required for achieving better outcomes.
Diaphragmatic hernias (DHs) are rare complications after pediatric liver transplantation (PLT). It is now widely accepted that DHs after liver transplantation (LT) is a pediatric related condition. PLTs (under of age 18) performed between January 2013 and June 2019 at Malatya Inonu University Institute of Liver Transplantation were retrospectively scanned. Study group consisting DHs and a control group were compared. Among 280 PLTs, 8 of them were complicated with DHs (%2.9). Median age of the patients with DH was 3.0 (0.8‐9.5) years. Median graft recipient weight ratio was 2.5 (0.9‐4.4). Five patients were below 5th percentiles in terms of pediatric weight growth chart at the time of LT. Also, 6 patients were below 5th percentiles in terms of pediatric height growth chart. There was no statistical difference between study and control groups. There are many risk factors mentioned in literature that may be primarily responsible for DHs after PLT. These factors are left lobe and large‐for‐size grafts, malnutrition, trauma or diathermy of diaphragmatic nerve and vessels and immunosuppressants. In our study, we could not specify any reason that differs in DHs. In our aspect, narrow diaphragma and thorax are exposed to high intra‐abdominal pressure from abdomen. Large‐for‐size grafts, which are specific to children, also may contribute to this affect. Excessive diathermy and trauma to diaphragmatic collaterals may aggravate the risk of DH. More patients are needed to make an exact conclusion, in order to evaluate with comparable study on this aspect.
1 Background
ADH is a rare and potentially fatal complication following LT. In this study, a systematic review was completed to identify risk factors which may contribute to ADH.
2 Methods
Transplant databases at three LT programs were reviewed. Four pediatric and zero adult cases were identified. Next, a systematic review was completed. Fourteen studies describing 41 patients with ADH were identified. Patient demographics, transplant characteristics, and features of ADH diagnosis were examined.
3 Results
The majority (90.2%) of ADH were in children. In pediatric LT, 95.1% received a segmental allograft. ADH occurred in the right P diaphragm 92.7% of the time, and 87.8% were repaired primarily. Patient demographics, post‐transplant complications, and immunosuppression regimens were broad and failed to predict ADH. Most patients presented with either respiratory or gastrointestinal symptoms. There were two pediatric deaths related to undiagnosed ADH. The combined worldwide incidence of ADH in pediatric LT is 1.5% (34/2319 patients).
4 Conclusion
ADH is a rare complication post‐LT that primarily occurs in pediatric recipients. When diagnosed early, ADH can be repaired primarily with good outcomes.
Diaphragmatic hernia is an unusual complication after pediatric liver transplant. Nearly half of bowel obstruction cases, which require surgical intervention in liver transplant patients, are caused by diaphragmatic hernia. The smaller patients are at risk for higher rates of diaphragmatic complication after pediatric liver transplant, but diaphragmatic hernia has not been reported as a unique occurrence. Here, we report 3 cases of diaphragmatic hernia after liver transplant and discuss the possible contributing factors. Diaphragmatic hernia should nevertheless be added to the list of potential complications after liver transplant in the pediatric population. Pediatric transplant physicians and surgeons should be aware of this complication so that it is recognized promptly in both acute and nonacute settings and appropriate action is taken.
Intrathoracic appendicitis is an uncommon diagnosis. We report the case of a 6-year-old boy with elevated CRP and no fever, who complained of nonspecific abdominal pain. A diaphragmatic hernia was suspected on a chest X-ray and confirmed by an ultrasound examination. A multidetector CT scan revealed intrathoracic acute appendicitis associated with a right posterolateral Bochdalek hernia. Abdominal diseases associated with late-presenting congenital diaphragmatic hernia are often manifested by an atypical clinical presentation, which can be a source of delay or error in diagnosis. We recommend radiological exploration in the case of diaphragmatic hernia, even with subtle clinical findings in the search of associated gastrointestinal complications.
Intrathoracic appendicitis is an uncommon diagnosis. We report the case of a 6-year-old boy with elevated CRP and no fever, who complained of nonspecific abdominal pain. A diaphragmatic hernia was suspected on a chest X-ray and confirmed by an ultrasound examination. A multidetector CT scan revealed intrathoracic acute appendicitis associated with a right posterolateral Bochdalek hernia. Abdominal diseases associated with late-presenting congenital diaphragmatic hernia are often manifested by an atypical clinical presentation, which can be a source of delay or error in diagnosis. We recommend radiological exploration in the case of diaphragmatic hernia, even with subtle clinical findings in the search of associated gastrointestinal complications.
Shigeta T, Sakamoto S, Kanazawa H, Fukuda A, Kakiuchi T, Karaki C, Uchida H, Matsuno N, Tanaka H, Kasahara M. Diaphragmatic hernia in infants following living donor liver transplantation: Report of three cases and a review of the literature.
Abstract: DH is a rare complication following LT. This report presents three cases of right‐sided DH after LT using a left‐sided graft. All of the patients were younger than one yr of age, and they were critically ill owing to their original disease, characterized by biliary atresia, progressive familiar intrahepatic cholestasis, and acute liver failure. DH occurred with sudden onset within three months after LT. All of the cases were promptly diagnosed and treated. A literature review of 24 cases of DH identified four factors associated with DH: left‐sided graft, right‐sided DH, relatively delayed onset of DH, and age‐specific chief complaint. DH following LT should be considered as a potential surgical complication when a left‐sided graft is used, especially in small infants with coagulopathy and malnutrition.
Fifty-nine renal transplant recipients with overt CMV disease were treated at the University of Minnesota Health Sciences Center between October 1, 1977 and November 15, 1978. In a group of 141 consecutive transplant patients, the incidence of overt CMV disease was 31%. Fifty-three patients (90%) developed clinical manifestations of CMV disease within 4 months of transplantation, and it was during this time period that overt CMV disease was associated with a significantly increased incidence of transplant nephrectomy and death. Fever was the most common presenting symptom (95% of patients), and overt CMV disease was found to be the single most common cause of fever in all hospitalized transplant recipients. Prolonged fever, diffuse pulmonary infiltrates, gastrointestinal bleeding, pancreatitis, transplant nephrectomy and development of other systemic infections were clinical features used to categorize patients according to disease severity. A number of these features were found to be significantly associated with the diagnosis of overt CMV disease. Twelve patients (20%) developed lethal CMV disease characterized by the presence of most of these features, 6 (10%) had severe disease, 9 (15%) had disease of moderate severity and 32 patients (54%) had mild CMV disease with fever being essentially their only clinical finding. Development of secondary systemic infection was most ominous, and occurred before death in 10 of the 12 patients with lethal CMV disease. The only patients to die with serious bacterial, fungal or protozoan infection during the period of this study had concomitant overt CMV disease. Abnormal liver function tests and leukopenia were common, and the degree of abnormality correlated with the severity of CMV disease. Of the multiple factors analyzed for their influence on the risk of developing overt CMV disease, several factors related to the kidney donor (the relationship of the donor to the recipient, HLA matching and CMV serology) appeared to be most important.
Twelve patients who had undergone diaphragmatic plication with the diagnosis of congenital diaphragmatic eventration between 1975 to 1989 were evaluated for the long-term results of plication. Assessment of the long-term functions of the plicated diaphragms 1.5 to 11 years postoperatively was achieved by fluoroscopic, ultrasonographic, and spirometric studies. The absence of paradoxical motion with normal localization of the diaphragms in all patients, and satisfactory motions of diaphragms in 9 patients were documented by fluoroscopy. Measurements of diaphragmatic thicknesses showed that plicated diaphragms of all patients maintained their growths in proportion to the contralateral sides. Additionally, normal values of pulmonary function tests in five of six patients of suitable age for spirometry were obtained. All the clinical studies demonstrated that diaphragmatic plication did not interfere with further development of diaphragms, and late functional results of the plication were acceptable. This supports the choice of surgery in the treatment of diaphragmatic eventration.
Blunt or penetrating truncal traumas can result in diaphragmatic rupture or injury. Because diaphragmatic defects are difficult to diagnose, those that are missed may present with latent symptoms of obstruction of herniated viscera.
A chart review of all patients admitted with late presentations of posttraumatic diaphragmatic hernias from 1980 to 1996 was undertaken.
Ten patients with posttraumatic diaphragmatic hernias were treated in this specified period. There were six males and four females with a mean age of 65 years. Eight patients sustained blunt truncal traumas and two patients sustained penetrating truncal traumas. The hernias occurred in two patients on the right and in eight patients on the left side and contained the liver (n = 2), bowel (n = 10), stomach (n = 4), omentum (n = 5), or spleen (n = 1). The time until the hernias became clinically symptomatic ranged from 20 days to 28 years. In all but one patient, either routine chest roentgenograms or upper gastrointestinal contrast studies were diagnostic. All 10 patients underwent laparotomy (n = 9) or thoracotomy (n = 2) with direct repair of the diaphragmatic defect. One patient died 3 days after the operation, representing a mortality of 10%; the morbidity was 30%.
Initial recognition and treatment of diaphragmatic rupture or injury is important in avoiding long-term sequelae.
The diagnostic workup in stable patients with penetrating thoracoabdominal injuries can be extremely difficult. Conventional diagnostic tests such as plain chest radiography, computed tomography scan, digital exploration, and diagnostic peritoneal lavage can be misleading. Classically, most of these patients have undergone exploratory laparotomy to determine whether there is a diaphragmatic injury.
In this study, 52 patients with penetrating thoracoabdominal trauma, and without any indication for immediate surgery, underwent video-assisted thoracoscopy to determine the presence of diaphragmatic injuries.
Of the 52 patients, 48 were men. The left hemithorax was involved in 38 patients (73%). Chest x-ray was normal in 40 patients (77%) who were clinically asymptomatic. Stab wounds were responsible for 80% of the injuries. At the time of the thoracoscopy, 35 patients (67%) were found to have a diaphragmatic injury. All 35 diaphragmatic injuries were successfully repaired thoracoscopically. The procedure was completed in 50 patients (96%). There were no deaths or complications.
The incidence of diaphragmatic injuries is higher than anticipated in asymptomatic patients with penetrating thoracoabdominal wounds. Video thoracoscopy can be used as a safe, expeditious, minimally invasive, and extremely useful technique to facilitate the diagnosis of these injuries in asymptomatic patients. Furthermore, diaphragmatic injuries can be repaired easily through a thoracoscopic approach with no complications.
(1) To determine the actual incidence rate of blunt and penetrating diaphragmatic injuries (DI); (2) to evaluate the effectiveness of urgent surgical intervention for treatment of DI; and (3) to reveal main causes of postoperative complications.
We reviewed: (1) forensic medical examination charts of 3353 subjects, who died due to polytrauma (including injuries to the chest and/or abdomen) at accident sites; and (2) medical case reports of 4857 patients, treated for thoracoabdominal trauma (TAT) from 1962 to 1998. A detailed analysis was completed with 12 years (1987--1998) of clinical experience, involving 65 (43 penetrating, and 22 blunt) cases of DI.
According to forensic medical data, blunt and penetrating DI occurred in 3.7% and 2.6% of individual cases, respectively. Among patients suffering from TAT, it was revealed that blunt DI had occurred in 1.1%, and penetrating in 3.9% of the cases. This data indicates if all the victims, who had sustained TAT, had survived, the incidence rate of DI would have been 2.6% (blunt -- 2.1%, and penetrating -- 3.4%). All the patients, provided surgical operations due to DI, survived. Morbidity in patients, suffering from blunt and penetrating DI, was 50%, and 35%, respectively. In the group of patients, suffering from penetrating DI, shock, intrapleural and/or intraabdominal haemorrhage, and liver injuries constituted a significant (P<0.05) influence, relevant to development of postoperative complications. The risk of complications was significantly (P<0.05) greater in cases of gunshot injuries. Fractures of chest bones, injuries of abdominal organs, and intraabdominal haemorrhage constituted a significant influence (P<0.05), relevant to development of complications after blunt DI.
(1) The danger to the health or even life of patients is not directly caused by DI, but by consequential complications and associated injuries; (2) the effectiveness of treatment is determined by purposeful surgical diagnostics with particular regard to DI and urgent surgical intervention.
Liver surgery has evolved in one “surgical lifetime” from being almost nonexistent to a repertoire of operations that can safely remove nearly any amount of liver tissue. These operations are now performed at numerous hospitals and medical centers throughout the world. Such phenomenal achievement did not come easily, but with the “blood, sweat, and tears” of patients and their families and of courageous surgeons who were enabled by extraordinary developments in anesthesiology, infectious disease, and radiologic imaging. At the beginning of this extraordinary era, the major concerns were how to remove a tumor in the liver without major, often fatal, bleeding and the uncertainty about the amount of liver that could be removed safely. Mortality and morbidity rates were unacceptably high initially and cure rates were unknown. Total hepatectomy and replacement by liver transplantation were dreams. Step by step, these problems were addressed. Initial limited excisions were followed by major ablative operations, including total hepatectomy, by less invasive procedures with laparoscopy, and then by increasing popularity for more limited segmental excisions of tumors, representing a cycle of achievements. Today, a new revolution looms: the possibility of computer-directed operations using the techniques of robotics and virtual reality. We hope that these technologic advances will be followed by the discovery of effective adjuvant therapy. The evolution of treatment in this century might be expected to result in surgery’s becoming irrelevant to liver neoplasms. It is well to remember that, in the 19th century, surgery was thought to have reached its apogee—but the best was yet to come. Key features of the liver that allow major resection were known in ancient times. Its structural arrangement into lobes was apparent to those preparing animals for food, ceremony, or human mummification. Clay models of the liver were made around 2000 BC by the Babylonians, who gave names to various areas and made prognostications from their appearances. Similar concepts were followed in later civilizations. Two other properties of the liver, namely functional reserve and rapid regeneration, were recorded in the early Greek myth of Prometheus, in which Prometheus’s liver regrew nightly after the eagle’s daily and apparently bloodless “surgical resections.” This oft-cited fantasy does not necessarily mean that the ancient Greeks knew about liver regeneration. But war wounds and animal sacrifice provided dramatic evidence of the liver’s power for massive hemorrhage, causing the Babylonians to consider it the seat of the soul. Knowledge of the liver’s lobar and segmental structures, functional reserve, capacity to regenerate, and prevention of hemorrhage is essential for successful major surgical resection. It is also of relevance to transplantation, particularly of split-liver homografts and the use of living related donors. The historic development from ancient times of knowledge about these key characteristics is a vast body of work and beyond the scope of this review. The purpose of this article is to present our admittedly imperfect views of the contributions that were seminal to the development of liver surgery for tumors. We have placed particular emphasis on the last 50 years of the second millennium, when progress was most rapid and we were participants.
Pediatric orthotopic liver transplantation (OLT) has a low mortality. Some children, however, have an adverse outcome defined as a prolonged ventilatory support requirement and protracted pediatric intensive care unit (PICU) stay. The aim of this study was to determine if that adverse outcome related to the child's condition pre-OLT and/or the development of a pleural effusion or diaphragmatic dysfunction.
The study included 210 children with a median age at transplantation of 45.5 months (range 0.2-252 months). Fourteen had undergone retransplantation. The duration of ventilatory support (intermittent positive pressure ventilation [IPPV]) and PICU admission and development of a pleural effusion and/or diaphragmatic dysfunction were documented for each child. The patients were divided into three groups according to whether they had acute liver failure (ALF), chronic liver disease at home (CHOM), or chronic liver failure sufficiently ill to be in the hospital awaiting transplantation (CHOSP).
The 36 children with ALF were of similar age to the 138 CHOM and 36 CHOSP children but required longer IPPV (P<0.0001) and PICU stay (P<0.0001). Overall, 17 children developed diaphragmatic dysfunction and 138 pleural effusions; affected children required longer IPPV and PICU stay (P<0.01). Regression analysis demonstrated that diaphragmatic dysfunction, but not pleural effusion development, was associated with prolonged ventilation (P<0.01) and protracted PICU stay (P<0.05). Other risk factors were ALF (P<0.01), retransplantation (P<0.01), and young age (P<0.05).
Diaphragmatic dysfunction adversely influences PICU morbidity after OLT. Early assessment of diaphragmatic function, and if necessary aggressive management, might improve outcome.
Delayed presentation of a traumatic diaphragmatic hernia is an infrequent condition with a high mortality and morbidity rate. This case describes a 26-year-old man presenting with a 2-day history of cramping abdominal pain, dyspnea, nausea, and vomiting. The patient reported a penetrating thoracic injury one year prior to the development of clinical signs. Computed tomography revealed the presence of empyema or parapneumonic effusion of the left hemithorax. Based on the clinical history and physical findings, a diaphragmatic hernia was considered and an emergency laparotomy with segmental resec-tion of strangulated jejunum and reduction of remaining bowel was performed. A 1.5 cm tendinous defect was identified and repaired. The patient recovered and was discharged uneventfully. Conclusion: the early recognition of a delayed diaphragmatic hernia contributed to the uneventful recovery of this critically ill patient.
A 31-year-old woman underwent microwave-assisted laparoscopic hepatectomy of the left lateral segment for focal nodular hyperplasia on January 14, 1998. On September 9, 1998, she felt continuous left abdominal pain and was admitted to our hospital for further examination. An upper gastrointestinal series showed converging folds of the greater curvature of the upper third of the stomach and craniad displacement of this portion. Thoracic magnetic resonance imaging showed herniation of the stomach into the pleural cavity. The patient was referred to our department, where she underwent surgery for a diaphragmatic hernia. The fundus of the stomach had escaped into the left pleural cavity through a defect in the diaphragm near where laparoscopic hepatectomy had been performed. The stomach was returned to the peritoneal cavity and the defect sutured. The patients postoperative course was uneventful. Although diaphragmatic hernia after laparoscopic surgery is a rare complication, with the performance of more advanced laparoscopic procedures and the use of higher-technology tissue-destruction/hemostatic devices such as the microwave coagulator, more caution should be observed to prevent injury to adjacent organs such as the diaphragm.
Patients who undergo living related left lateral segment liver transplants have been reported to have a high incidence of biliary complications and some studies suggest that most patients will ultimately need operative revision. We reviewed our experience with living related transplantation in pediatric recipients to examine the occurrence of biliary complications and the utility of percutaneous biliary procedures in their management. Over a 10-yr period, 48 living donor transplants were performed in 47 patients. Sixteen patients (33%) had biliary complications. Complications included 10 leaks (20%) and eight strictures (17%). Although leaks were treated predominantly with operation, other biliary complications were treated almost exclusively non-operatively. Self limited leaks that lead to biloma accumulation were most often treated via percutaneous catheter drainage and all strictures were treated using percutaneous transhepatic biliary cholangioplasty and stenting. Sixty-seven percent of biliary complications underwent non-operative biliary intervention. Most strictures were focal anastomotic strictures and were successfully treated with cholangioplasty although multiple interventions were necessary and patients required stenting for an average of 13 months. Three of eight strictures were diffuse in nature and these included the only patient who required retransplantation. Graft survival with respect to biliary complications was 94%; 1 yr, 5 yr and overall patient survival for those with biliary complications was 88, 88 and 81%, and for the entire living related group was 84, 81 and 77%, respectively. Although biliary complications are frequent in pediatric living related transplantation, they are not associated with decreased patient survival. Excepting significant bile leaks, the majority can be treated non-operatively via biliary cholangioplasty and stenting. Strictures are especially amenable to this technique which, in our experience, has been successful at decreasing or postponing the need for retransplantation.
Liver transplantation is just as successful in infants as in older children, but more challenging. This relates to the low weight of the recipients and to their rapidly deteriorating clinical condition (malnutrition and end-stage liver disease) ( J Pediatr 1990;117:205-210; BMJ 1993;307:825-828; Ann Surg 1996;223:658-664; Transplantation 1997;64:242-248; J Pediatr Surg 1998;33:20-23). In addition, higher rates of diaphragmatic complications have been shown to significantly correlate with a younger age ( Transplantation 2002;73:228-232; Transpl Int 1998;11:281-283; Pediatr Transplant 2000;4:39-44), but diaphragmatic hernia has never been reported as a complication of liver transplantation. In this report, 2 patients who developed diaphragmatic hernia after liver transplantation are presented. The possible role of several contributing factors resulting in diaphragmatic hernia is discussed. These factors include (1) diaphragm thinness related to low weight and malnutrition, (2) direct trauma at operation (dissection and diathermy), (3) increased abdominal pressure after transplantation caused by the use of a slightly oversized liver graft, and (4) the medial positioning of the partial liver graft in the abdomen.