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Left anteromedial strangulated congenital diaphragmatic hernia in an 11-year-old child: a case report
Congenital Diaphragmatic Hernia (CDH) is commonly diagnosed in antenatal scans or presents in the neonatal age group. Presentation of CDH in infants or older children with respiratory symptoms is less common. Similarly, the presentation of CDH with primarily gastrointestinal symptoms is also quite rare and poses a diagnostic and therapeutic challenge. An atypical case of CDH with later presentation with gastrointestinal symptoms is described. A failure to diagnose and correct it in a timely fashion could have a deleterious impact on the outcome.
A case of a five-month-old child presenting with features of bowel obstruction is described. On evaluation, the child had rapidly progressive respiratory distress and gross abdominal distension, which was found to be due to left-sided CDH. A closed-loop obstruction with impending strangulation was managed with prompt surgery. The patient had a favorable outcome.
Late presenting CDH may present with primarily gastrointestinal symptoms with impending respiratory compromise. A high index of suspicion is necessary to ensure early diagnosis and management.
With increasing use of ultrasound screening, the prenatal diagnosis of congenital diaphragmatic hernia (CDH) in better resourced areas has become the norm. However, early diagnosis is still not universal in resource-poor settings and late presentations of CDH continue. We retrospectively analysed the medical records of children operated for late-presenting CDH from 2001 to 2016 at our tertiary care centre in North India. A total of 32 patients were operated during the period with a male-to-female ratio of 3:1. Of these, 78% presented with respiratory symptoms, 37% with recurrent vomiting and 18% with an acute abdomen. Nine (28%) had been treated erroneously for gastroenteritis and another six (18%) had received anti-tubercular therapy for variable periods. A plain chest radiograph with a Ryle’s tube in situ was confirmatory in 75% (24/32). In conclusion, initial misdiagnosis and subsequent unnecessary therapeutic interventions were the leading cause of morbidity.
Cutaneous leishmaniasis (CL) is a parasitic disease which has a biphasic life cycle; infection by promastigotes from the sandfly reaches a wound where it is phagocytosed by macrophages, producing the amastigote (the Leishmania donovani body) in the host. A protozoan parasite transmitted by the phlebotomous sandfly causes human leishmaniasis. Cutaneous forms include classical cutaneous, mucocutaneous and post-kala-azar dermal leishmaniasis. It affects c. 300 million individuals in more than 90 nations around the globe.
The cutaneous form in the Old World is caused at low altitudes mainly by L. major (which has an animal reservoir, rodents such as mouse) and in swampy regions and high altitudes by L. tropica (which has no animal reservoir). L. aethiopica and L. major lead to disseminated ulcers in Saudi Arabia, Yemen, Iraq, Iran, Pakistan, India, Tunisia, Sudan and Ethiopia, whose main electrophoretic isozyme pattern Zymodeme in Saudi Arabia is LON-4.
Delayed herniation of the abdominal contents through a congenital diaphragmatic hernia may occur beyond the neonatal period. This report describes a 9-week-old female baby who presented with excessive crying, irritability and respiratory distress secondary to late presentation of left-sided congenital diaphragmatic hernia. The chest radiograph showed tension gastrothorax. She underwent surgical reduction of the hernia. She made an excellent recovery and was discharged a few days after the operation. It is assumed that sudden increase of the intra-abdominal pressure caused herniation of abdominal content through a pre-existing diaphragmatic defect. This report aims to increase the awareness of this condition among physicians and pediatric surgeons to allow early diagnosis and management.
Purpose
Late-presenting congenital diaphragmatic hernia (CDH) beyond the neonatal period is rare and often misdiagnosed, with delayed treatment.
Materials and Methods
We retrospectively reviewed our experience with late-presenting CDH over 30 years at a single institution to determine the characteristics of late-presenting CDH for early diagnosis.
Results
Seven patients had operations due to late-presenting CHD in our institution over 30 years. The patients' ages ranged from 2.5 months to 16 years. There were six boys and one girl. Five hernias were left-sided, one was right-sided and one was a retrosternal hernia. All patients had normal intestinal rotation. Non-specific gastrointestinal or respiratory symptoms and signs were usually presented. Intestinal malrotations were absent; therefore, only organs adjacent to the defect or relatively movable organs such as the small bowel and transverse colon were herniated. Two cases were accompanied by stomach herniation with the volvulus and liver, respectively. The duration from presentation to diagnosis varied from 5 days to 1 year. Diagnoses were made by chest X-ray, upper gastrointestinal series and chest computed tomography. All patients underwent primary repair with interrupted non-absorbable sutures by a transabdominal approach. None had postoperative complications. The follow-up period in six patients ranged from 4 months to 20 years (median 3.8 years). There was no recurrence in any of the patients on follow-up.
Conclusion
A high index of suspicion is important for the diagnosis of late-presenting CDH because it can be a life-threatening condition such as CDH with a gastric volvulus. Early diagnosis and appropriate treatment can lead to a good prognosis.
Congenital Bochdalek diaphragmatic hernia (CDH) is easily recognized if it is present shortly after birth. However, cases of delayed CDH presentation are more subtle and therefore less easily diagnosed.
We retrospectively analyzed 85 patients who were under 18 and diagnosed with CDH between June 1987 and May 2007. Those diagnosed before 1 month of age were categorized as having early-presenting CDH and those diagnosed after 1 month were categorized as having late-presenting CDH.
Of the 85 cases, 68 (80%) were early-presenting and 17 (20%) were late-presenting CDH. Respiratory symptoms were more common in early-presenting CDH, while gastrointestinal complaints were more dominant in the late-presenting group, particularly in patients with left diaphragmatic defects. Late-presenting CDH, which was more likely to be associated with chronic gastrointestinal symptoms, was associated with a lower mortality than acute early-presenting CDH with respiratory symptoms, despite the more prompt diagnosis of the latter. The small bowel was the organ most commonly herniated into the thorax. Congenital heart disease was the most commonly associated malformation, but the presence of associated anomalies did not increase mortality.
Early-presenting CDH shows acute symptoms, is readily diagnosed, and requires prompt intervention to prevent death. In contrast, late-presenting CDH shows more subtle symptoms and is more difficult to diagnose. Although the late-presenting CHD has better prognosis, it still requires surgery intervention to alleviate the symptoms. Clinicians must maintain a high index of suspicion for CDH, regardless of its presentation.
We report 3 cases of congenital diaphragmatic hernia (CDH) in the second decade of life which were misdiagnosed on initial presentation. The first case had an iatrogenic gastric injury because of intercostal tube drainage for suspected pleural effusion. The second case was treated for pulmonary tuberculosis for 6 months before being diagnosed as a case of CDH. The third case presented as acute chest pain on the left side. It was treated accordingly for 1 month and was diagnosed as a CDH on a CT scan of the chest when seen by a surgeon.
The incidence of congenital diaphragmatic hernia (CDH) may be as high as 1 in 2000. Over the past two decades, antenatal diagnosis rates have increased, the pathophysiology of CDH has become better understood, and advances in clinical care, including foetal surgery, have occurred. However, there remains a paucity of randomised controlled trials to provide evidence-based management guidelines. Reports of improved survival rates appear to be confined to a select subset of CDH infants, surviving to surgical repair, while the overall mortality, at over 60%, appears to be unchanged, largely due to the often forgotten 'hidden mortality' of CDH. The significant long-term morbidity in surviving infants has become apparent, and the need for long-term multidisciplinary follow up established. A total of 10% of cases may present later in life, and misdiagnosis on initial chest X-ray may lead to significant morbidity.
Congenital right diaphragmatic hernia of Bochdalek rarely occurs in adults. Most of them are asymptomatic. In this article, we report a case of a 21-year-old male with rightsided Bochdalek diaphragmatic hernia who presented with abdominal pain and dyspnea. The chest radiography showed features suggestive of right-sided diaphragmatic hernia. This was confirmed on a computed tomography. The patient underwent right posterolateral thoracotomy whereby a 10 cm posterolateral diaphragmatic defect with herniation of the colon and kidney through the opening was found. During surgery the colon and right kidney were reduced into peritoneal cavity, and the diaphragmatic defect was repaired with non-absorbable sutures. The patient's recovery was uneventful. He remained well at six-month follow-up.
The prevalence of congenital diaphragmatic hernia (CDH) ranges between 1:2000 and 1:4000 live births; it accounts for 8% of
all major congenital anomalies. Recurrence risk for a subsequent pregnancy is estimated at 2%.1 Approximately 90% of diaphragmatic defects occur posterolaterally and 80% are left-sided. The severity of the condition varies
widely, the degree of pulmonary hypoplasia and pulmonary hypertension largely determining outcome. After recent advances in
the care of these patients, several centres are now reporting survival rates >80%. This improved survival is ascribed to increased
knowledge of the pathophysiology of the condition and, consequently, better perioperative management.
The management of congenital diaphragmatic hernia (CDH) is undergoing continual change and refinement, fuelled by recent advances in this field. Although many studies have documented the benefits of these recent advances but definite recommendations are lacking. Also, injudicious use of some of these strategies may be counterproductive, underscoring the importance of evidence based treatment strategy. This article discusses the utility of the recent advances in the management of CDH.
Five adult patients with the Bochdalek type of congenital diaphragmatic hernia are presented. Four of the hernias were on the left side and one on the right. One of the patients was asymptomatic, while 3 of them had gastrointestinal symptoms. In 2 of these 3 cases, the symptoms became worse when the patients were lying down. One patient complained mostly of dyspnoea, and also had a patent ductus arteriosus. The other anomaly, in addition to hernia, was diaphragmatic pulmonary sequestration. All the patients were operated on transthoracally. In one case, laparotomy was also performed. There was no strangulation in any of the cases. No operative or postoperative complications occurred. A complete roentgenologic examination including chest X-ray, barium meal, barium enema, and sometimes pneumoperitoneum may be needed in order to make an accurate diagnosis preoperatively. Because of the strangulation danger operation is recommended whenever the diagnosis is made.
This is the third reported instance of identical twins, each with left-sided posterolateral congenital diaphragmatic hernia (CDH) (Bochdalek type), who have been operated upon successfully. The associated anomalies were mirror image undescended testicles. Comparative review of familial and sporadic cases of CDH revealed that males were more commonly affected in the former, while females were more commonly affected in the latter. There was no known etiology in either type. Both familial and sporadic cases shared a high incidence of associated anomalies (40% to 50%). Three anomalies were equally found in both types, mainly pulmonary hypoplasia, intestinal malrotation, and patent ductus arteriosus. Central nervous system anomalies were highly prevalent in the sporadic cases (55% to 75%), while cardiovascular and genitourinary anomalies (30% each) were the more common anomalies encountered in the familial cases.
Delayed presentation of congenital diaphragmatic hernia (CDH) has been considered rare, and clinical manifestations differ from the more common newborn entity. Associated malformations in late-appearing CDH have been reported in a few patients. The authors reviewed their clinical experience to catalogue the frequency and clinical importance of associated malformations in patients with late-presenting CDH.
The records of patients greater than 1 month of age with Bochdalek type CDH treated in the authors' clinic, from 1991 to 2001, were retrospectively reviewed. Twenty patients (age range, 1 month to 10 years) were included in the study.
Associated malformations were documented in 16 of the patients (80%) and included malrotation in 12 patients, umbilical hernia in 2, pulmonary hypoplasia in 4, pulmonary sequestration in 1, gastroesophageal duplication cyst coexisting with polysplenia in 1, atrial septal defect and ventricular septal defect in 1, hydronephrosis in 1, wandering spleen in 1, talipes equinovarus in 1, and type I diabetes mellitus in 1.
The results of this study show a significant incidence and a wide spectrum of associated malformations. These findings suggest that patients with late CDH should be evaluated carefully for additional anomalies that may help to establish correct diagnosis and treatment.
Evaluation of abnormal adult chest computed tomography (CT) scans.
Retrospective series of 3 cases.
Two University-based hospitals.
Three adult patients (age range 56 to 61) underwent chest CT scans. Two were trauma patients and one complained of chest pain with a negative cardiac workup. One CT scan revealed a lung mass that was highly suspicious for malignancy and the other two CT scans were suspicious for diaphragmatic hernia. Two patients underwent elective surgery and the third underwent emergent surgery.
The CT scan of patient 1 was suspicious for malignancy; however, the pathology of the lung mass revealed only inflammation consistent with an intralobular bronchopulmonary sequestration. Patient 2 had a congenital Morgagni hernia with omentum and colon in the mediastinum, and patient 3 had a diaphragmatic defect combined with a bifid sternum and defective pericardium, fulfilling three criteria of pentalogy of Cantrell.
Thoracic congenital malformations may go unnoticed and unsuspected until adulthood. They should be kept in mind when reviewing unusual CT scans of the chest in adults. Their surgical management, however, is similar to their counterparts in infancy.
Late-presenting congenital diaphragmatic hernia in children: the experience of single institution in Korea
- Kim Dj
- J H Chung
Kim DJ and Chung JH. Late-presenting congenital diaphragmatic hernia in children: the experience of single
institution in Korea. Yonsei Med J 2013; 54: 1143-1148.