Late-presenting Congenital Diaphragmatic Hernia Research Articles

Review of Late-Presenting Congenital Diaphragmatic Hernia in Children

Review of Late-Presenting Congenital Diaphragmatic Hernia in Children

Appendicite aiguë intrathoracique. À propos d’un cas

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.

Late-presenting congenital diaphragmatic hernia: diagnostic pitfalls and outcome

We sought to review the presentation, diagnosis, and outcome of a series of children with late-presenting, congenital diaphragmatic hernias (CDH). Bochdalek and Morgagni hernias that were diagnosed after 30 days of age, between January 1989 and December 2009, were reviewed retrospectively. A medical record review and telephone survey were conducted in 2010. Thirty-one subjects, diagnosed with CDH between 45 days and 13 years of age (mean, 16 months), were reviewed. Bochdalek hernias were present in 18 (58%) and Morgagni hernias in 13 (42%). There were twenty (64%) left-sided, eight (26%) right-sided, and three (10%) bilateral CDH. Five (16%) had other congenital anomalies. Eight (25.8%), including a subject with strangulated intestine that required resection, were initially misdiagnosed, due mostly to failure to obtain or correctly interpret a chest radiograph. Thirty (97%) were repaired by an abdominal approach, including seven laparoscopic closures. Follow-up ranged from 1 to 20 years (median, 7 years). All subjects survived without recurrence. Unlike neonatally diagnosed CDH, neither right-sided hernia, patch repair, nor associated esophageal atresia predicted morbidity. Although diagnostic delays may lead to morbidity, if late-presenting CDH are expeditiously identified and repaired, their outcome is very good, in contrast to those that present in neonates.

Delayed Presentation Of Congenital Diaphragmatic Hernia With Splenic And Caecal Herniation

The vast majority of posterolateral congenital diaphragmatic hernia (CDH) diagnosed on prenatal ultrasound screening after 24 weeks of gestation present with respiratory distress at birth. It has been observed that late presenting CDH has better outcome and is not associated with pulmonary hypoplasia. We report a case of posterolateral CDH with herniated spleen presented first in a four year old male child with chest infection. This case report highlights the fact that posterolateral CDH which presents late is not associated with pulmonary hypoplasia, but may cause respiratory symptoms.

Radiographic findings in late-presenting congenital diaphragmatic hernia: helpful imaging findings

Imaging findings in delayed presentation of congenital diaphragmatic hernia can be confusing and misleading, resulting in a delay in diagnosis. To evaluate the often puzzling plain film findings of late-presenting CDH in an effort to determine whether any of the findings could be helpful in arriving at an early diagnosis. We reviewed and documented the plain film findings and clinical data in eight patients seen during the last 20years with late-presenting CDH. IRB exempt status was obtained in this study. There were five boys and three girls. The age range was 4months to 12years with a mean of 2.4years. Five children presented with acute respiratory problems while three presented with acute abdominal pain. Two children presented with both respiratory and abdominal findings and one also presented with hematemesis. Two children had radiographic findings that were not difficult to analyze while the remaining six had findings that posed initial diagnostic problems. Although not common, late-presenting CDH can result in confusing plain film radiographic findings and a delay in diagnosis. We found that the most important finding in analyzing these radiographs is in evaluating the location and position of the gastric bubble with the more common left-side hernias.

Challenges in the management of early versus late presenting congenital diaphragmatic hernia in a poor resource setting

Despite the advances in management, congenital diaphragmatic hernia (CDH) has continued to pose a significant challenge to paediatric surgeons. This is amplified in a setting like ours where there is a dearth of facilities to cope with the problem of CDH. This study was undertaken to highlight the peculiarities of the management of CDH in a poor resource setting. All confirmed cases of CDH were prospectively documented from 2003 till date. Seven children were treated from 2003 till date. The diaphragmatic defect was on the left side in six (83.8%) and on the right side in one (17.7%). All the patients had primary closure of the defect without patch via an abdominal approach. The three patients presenting at birth died while the remaining four patients survived. With inadequate neonatal intensive care facilities, the severe early presenting CDH has a dismal prognosis. In contrast, the late presenting CDH poses more diagnostic challenges; but once identified and appropriate treatment instituted, it has an excellent prognosis. We recommend that physicians should include CDH in the differential diagnosis of patients with birth asphyxia and in patients with chronic respiratory symptoms with failure to thrive.

P-289 Late-presenting congenital diaphragmatic hernia requiring emergency surgery : report of 3 cases

P-289 Late-presenting congenital diaphragmatic hernia requiring emergency surgery : report of 3 cases

CONGENITAL DIAPHRAGMATIC HERNIA

We report a case of late-presenting congenital diaphragmatic hernia (CDH) in a three-month-old male infant presenting to the pediatric hospital with attacks of cough and cyanosis .Although CDH is usually manifested on the first day of life, there are a number of case reports of late-presenting CDH usually presenting with respiratory or gastrointestinal symptoms. In this case report, we have focused on the anesthetic management of late-presenting CDH. Text: Congenital diaphragmatic hernia is usually manifested on the first days of life as respiratory distress and a scaphoid abdomen. The abnormality is herniation of the abdominal viscera through a defect in the diaphragm, most commonly the foramen of Bochdalek on the left side1. These infants often have a dramatic presentation3, in contrast, late onset or late- presenting CDHs present outside the neonatal period with variable signs and symptoms4. We describe a case of late-presenting CDH that referred to the pediatric hospital with attacks of cough and cyanosis.

Late presentation of congenital diaphragmatic hernia in patients with cystic fibrosis

Late presentation of congenital diaphragmatic hernia (CDH) is unusual, especially in patients with cystic fibrosis (CF). To our knowledge, cases of CDH in CF patients and the combined effects on lung function have not been previously described. Here we report two cases of late presenting CDH in CF patients and describe effects on lung function. Late presentation of CDH in CF patients can cause gastrointestinal or respiratory symptoms and requires a high index of suspicion as well as proper interpretation of imaging. In patients with CF and CDH, lung function abnormalities could include obstructive, restrictive defects, or combined defects.

A late presenting congenital diaphragmatic hernia misdiagnosed as spontaneous pneumothorax

Congenital diaphragmatic hernia (CDH) is described as (1) failure of diaphragmatic closure at development, (2) presence of herniated abdominal contents into chest and (3) pulmonary hypoplasia. Usually, pleural space is drained urgently when there is respiratory distress and radiological appearance of mediastinal shift. We present a case of a 5-month-old baby, diagnosed as tension pneumothorax and treated with chest drain insertion. CDH was the intraoperative diagnosis.

A 6-week-old Boy with Apnea and Abdominal Distention

A 6-week-old Boy with Apnea and Abdominal Distention

Neonatal minimally invasive surgery for congenital diaphragmatic hernias: a multicenter study using thoracoscopy or laparoscopy

Minimally invasive surgery (MIS) for late-presenting congenital diaphragmatic hernia (CDH) has been described previously, but few neonatal cases of CDH have been reported. This study aimed to report the multicenter experience of these rare cases and to compare the laparoscopic and thoracoscopic approaches. Using MIS procedures, 30 patients (16 boys and 14 girls) from nine centers underwent surgery for CDH within the first month of life, 26 before day 5. Only one patient had associated malformations. There were 10 preterm patients (32-36 weeks of gestational age). Their weight at birth ranged from 1,800 to 3,800 g, with three patients weighing less than 2,600 g. Of the 30 patients, 18 were intubated at birth. The MIS procedures were performed in 18 cases by a thoracoscopic approach and in 12 cases by a laparoscopic approach. No severe complication was observed. For 20 patients, reduction of the intrathoracic contents was achieved easily with 15 thoracoscopies and 5 laparoscopies. In six cases, the reduction was difficult, proving to be impossible for the four remaining patients: one treated with thoracoscopy and three with laparoscopy. The reasons for the inability to reduce the thoracic contents were difficulty of liver mobilization (1 left CDH and 2 right CDH) and the presence of a dilated stomach in the thorax. Reductions were easier for cases of wide diaphragmatic defects using thoracoscopy. There were 10 conversions (5 laparoscopies and 5 thoracoscopies). The reported reasons for conversion were inability to reduce (n = 4), need for a patch (n = 5), lack of adequate vision (n = 4), narrow working space (n = 1), associated bowel malrotation (n = 1), and an anesthetic problem (n = 1). Five defects were too large for direct closure and had to be closed with a patch. Four required conversion, with one performed through video-assisted thoracic surgery. The recurrences were detected after two primer thoracoscopic closures, one of which was managed by successful reoperation using thoracoscopy. In the neonatal period, CDH can be safely closed using MIS procedures. The overall success rate in this study was 67%. The indication for MIS is not related to weeks of gestational age, to weight at birth (if >2,600 g), or to the extent of the immediate neonatal care. Patients with no associated anomaly who are hemodynamically stabilized can benefit from MIS procedures. Reduction of the herniated organs is easier using thoracoscopy. Right CDH, liver lobe herniation, and the need for a patch closure are the most frequent reasons for conversion.

Late-presenting congenital diaphragmatic hernia in pediatric emergency room: two case reports

A congenital diaphragmatic hernia is a serious, complex condition with high mortality. It is actually mostly diagnosed prenatally. In most cases, it presents in the neonatal period, but can rarely present later in life when it becomes symptomatic. The clinical presentation and prognosis depend on the time of visceral herniation. We report two cases of left Bochdalek hernia with delayed presentation. The two cases shared some important characteristics. In both patients, the asymptomatic period ended with an acute attack of disease. To initiate appropriate therapy, emergency doctors need to be aware of the various presentations of a delayed left-sided diaphragmatic hernia.

Jarcho-Levin Syndrome Presenting with Diaphragmatic Hernia

Jarcho-Levin syndrome (spondylothoracic or spondylocostal dysostosis) is an eponym that is used to define individuals with a short neck, short trunk, and short stature and multiple vertebral anomalies. The prognosis is directly related to respiratory complications. Reported findings associated with Jarcho-Levin syndrome include congenital heart defects, abdominal wall malformations, genitourinary malformations, upper limb anomalies, and neural tube defects. We report on a 6-day-old girl who presented with an incomplete form of Jarcho-Levin syndrome with late-presenting congenital diaphragmatic hernia and congenital heart disease.

Death Due to Late-Presenting Congenital Diaphragmatic Hernia in a 2-Year-Old Child

Congenital diaphragmatic hernia (CDH) is classically regarded as a neonatal defect presenting with respiratory distress; however, not all CDH will present in this manner. Unlike newborn deaths related to CDH, where the mechanism of death is respiratory in nature, the mechanism of death in late-presenting CDH is not always due to respiratory compromise. In this case report, we present a death occurring in a 2 1/2-year-old child who presented to the emergency department with complaints of abdominal pain and emesis, and then rapidly decompensated and died. Autopsy revealed a CDH, with herniation of abdominal contents into the left thoracic cavity, with associated gastric volvulus, necrosis, and rupture.

Late-Presenting Congenital Diaphragmatic Hernia Mimicking Bronchiolitis

We report a case of a late-presenting congenital diaphragmatic hernia (CDH) in an otherwise healthy infant initially presenting to the emergency department with wheezing and respiratory distress. Late-presenting CDH can manifest a vast array of clinical symptoms and therefore may frequently masquerade as other more common pediatric entities. Prompt and accurate diagnosis is essential in the management of late-presenting CDH; patients may be critically ill at presentation, and selection of appropriate therapeutic interventions may avoid potentially life-threatening complications. In this case report, we seek to inform emergency physicians about this rare, but serious, disorder.

Late Presentation of Bochdalek-Type Congenital Diaphragmatic Hernia in Children: A 23-Year Experience at a Single Center

The purpose of this article is to report on our 23-year experience of treating children with late-presenting congenital diaphragmatic hernia (CDH), focusing on diagnostic difficulties, associated anomalies, and morbidity. We reviewed 19 children in whom Bochdalek-type CDH was diagnosed after the neonatal period, between 1983 and 2005. There were 14 boys and 5 girls, with a mean age of 18.3 months (range, 5 weeks-14 years). Ten (52.6%) of the patients presented with respiratory symptoms and five (26.3%) with gastrointestinal symptoms. The diagnosis of CDH was based on the findings of chest X-rays, gastrointestinal tract contrast radiographs, and computerized tomography findings. The CDH was on the left side in 16 patients and on the right side in three patients. A hernia sac was present in seven (36.8%) patients. The only postoperative complications were intestinal obstruction caused by adhesions, incisional hernia, and eventration of the diaphragm in one patient each. None of the patients died within this study period. The possibility of a delayed presentation of CDH should be considered in the differential diagnosis of recurrent non-specific respiratory or gastrointestinal tract symptoms in a child. A plain roentgenogram with a swallowed nasogastric tube might assist in the diagnosis. Early surgical correction of the diaphragmatic defect is crucial for preventing life-threatening complications.

Late-Presenting Congenital Diaphragmatic Hernia in Children

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Laparoscopic repair of late presenting congenital diaphragmatic hernia

Congenital diaphragmatic hernia usually presents in the neonatal period, with delayed presentation being uncommon. Traditionally repair was performed by laparotomy or thoracotomy. We have performed laparoscopic repair of a previously undiagnosed congenital diaphragmatic hernia that presented acutely in a 10-year-old male. Laparoscopic repair of late-presenting congenital diaphragmatic hernia is a safe and effective approach even in an emergency. The laparoscopic approach has advantages including reduced hospital stay, excellent visualisation of the defect even for obese patients, and improved cosmesis.

Late-presenting congenital diaphragmatic hernia associated with ectopic thoracic kidney

Although congenital defects of diaphragma often occur in the period immediately following birth, 10-20% of these cases are diagnosed later. We report on a 7-month-old male infant with late-presenting congenital diaphragmatic hernia associated with a thoracic ectopic kidney. We conclude that congenital diaphragmatic defects should be considered in young children with respiratory distress and that computerized tomography is a noninvasive and accurate diagnostic method in the evaluation of additional abnormalities in these patients.