221/781. Congenital left-sided Bochdalek diaphragmatic hernia. Thoracoscopic repair--case report. The authors report the case of a 12-year-old girl who presented with a left-sided diaphragmatic hernia that was diagnosed after a pulmonary infection. CT scan confirmed a posterolateral diaphragmatic Bochdalek defect. The child underwent a thoracoscopic repair of the defect in 1997. The herniated contents included spleen, stomach, and intestine. The child was discharged from hospital the day after surgery. She has been followed up and is clinical and radiologically well. ( info) |
222/781. Morgagni's hernia. Congenital diaphragmatic hernias, including Morgagni s hernia, usually present in early childhood and are treated by surgical repair. This case report is about an unusual Morgagni s hernia, presenting with dyspepsia and chest pain, at the age of 45 years. For many years the diagnosis remained a dilemma because patient s chest x-ray was not done and she was treated for "angina " and "dyspepsia". diagnosis was obvious once a chest x-ray was done, however, barium studies were performed for further confirmation. ( info) |
223/781. Congenital diaphragmatic hernia associated with ipsilateral upper limb reduction defects: report of a case with thumb hypoplasia. Four unrelated cases of congenital diaphragmatic hernia associated with ipsilateral upper limb reduction defects were reported by McCredie and Reid in 1978 (J Pediatr 92: 762-765). As contiguous segments of the cervical neural crest are involved in the development of diaphragm and arms, the authors suggested that an early injury to the cervical neural crest might be the common underlying pathogenesis. We describe here a further example of this malformation complex: a newborn with a left posterolateral diaphragmatic hernia associated with ipsilateral thumb hypoplasia. ( info) |
224/781. Triply discordant triplets: probability, management options, and risks. The spontaneous occurrence of triplets is rare. With increased utilization of "assisted reproductive technologies," multifetal gestations have become more common. The empiric fetal risk for major malformation is approximately 3%. In a triplet pregnancy each fetus independently carries this risk so that the probability of having at least one malformed fetus is approximately 9%. It is much less likely to have 2 or 3 simultaneously but discordantly malformed fetuses in a multizygotic triplet gestation (.09% and .0027% risk, respectively). We report on the first case, to our knowledge, of an ovulation-stimulated triplet pregnancy complicated by 3-way discordance for major malformations diagnosed in the late second trimester by ultrasound. fetus A was affected by congenital diaphragmatic hernia and trisomy 21; fetus B had encephalocele, a midline facial defect, and a cleft palate; and fetus C had evidence of unilateral claw hand but an otherwise normal fetal survey. At 19 weeks of gestation, fetus A was found to have spontaneously died, and a selective termination of triplet B was performed. We conclude: (1) the finding of a single major malformation in one fetus should lead to extensive search for malformations in all members of the pregnancy, and (2) the simultaneous occurrence of major malformations in more than one member of a multifetal gestation is a circumstance under which multiple selective termination deserves consideration. In this article we discuss important issues and caveats in the performance of selective termination for abnormal members of multifetal gestations. ( info) |
225/781. Cystic hygroma and congenital diaphragmatic hernia: early prenatal sonographic evaluation of Fryns' syndrome. We report a case of cystic hygroma and diffuse lymphangiectasia detected by sonogram at 12 weeks' gestation. Fetal karyotype was normal. At 20 weeks' gestation, herniation of the bowel into the chest was noted. At delivery, the infant was diagnosed as having Fryns' syndrome. This is the first reported case of Fryns' syndrome presenting with cystic hygroma. ( info) |
226/781. Diaphragmatic hernia resulting from injury during microwave-assisted laparoscopic hepatectomy. 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. ( info) |
227/781. Treatment of rebound and chronic pulmonary hypertension with oral sildenafil in an infant with congenital diaphragmatic hernia. OBJECTIVE: We describe a case of chronic pulmonary hypertension in a 7-wk-old infant with congenital diaphragmatic hernia and an oral teratoma. Our patient was dependent on low-dose inhaled nitric oxide and was still very unstable with systemic right ventricular pressures leading to frequent oxygen desaturations. We administered sildenafil therapy to stabilize the infant with discontinuation of inhaled nitric oxide. We describe successful discontinuation of the inhaled therapy as well as a period of stabilization and improvement with continued sildenafil administration. DESIGN: Case report. SETTING: intensive care nursery in tertiary academic center. PATIENT: A 7-wk-old infant with congenital diaphragmatic hernia who was mechanically ventilated from birth. INTERVENTION: Oral sildenafil 0.3 mg/kg/dose every 12 hrs. Measurements and RESULTS: Right ventricular pressure (from tricuspid valve regurgitant flow) to systemic systolic arterial pressure was measured by echocardiogram. Right ventricular to systemic pressure ratio was marginally improved with the initiation of sildenafil therapy. Inhaled nitric oxide was successfully discontinued, and the patient clinically stabilized temporarily, but he ultimately succumbed to his pulmonary hypertension. CONCLUSION: Sildenafil may be a useful therapy for chronic pulmonary hypertension in congenital diaphragmatic hernia, but further studies of safety and efficacy need to be performed. ( info) |
228/781. Late presenting congenital diaphragmatic hernia. Delayed herniation of the abdominal contents through a congenital diaphragmatic hernia may occur beyond the neonatal period. The case is reported of a 9 month old child presenting with acute respiratory distress secondary to tension gastrothorax. The chest radiograph showed a tension gastrothorax. Nasogastric tube placement confirmed herniation of the stomach into the left chest and is the initial treatment of choice when a tension gastrothorax is identified. The late presenting congenital diaphragmatic hernia poses considerable diagnostic challenges often leading to misdiagnosis and risk of thoracocentesis. The possibility of late presenting congenital diaphragmatic hernia should be considered in unusual cases of pneumothorax, especially in the absence of trauma so that unnecessary procedures like chest tube drainage can be avoided. ( info) |
229/781. Giant Meckel's diverticulum associated with a congenital diaphragmatic hernia. Giant Meckel's diverticulum is a very rare lesion and its association with a congenital diaphragmatic hernia has not been reported previously. We report a case of newborn with a giant Meckel's diverticulum and congenital diaphragmatic hernia. A large round atypical air-filled bowel segment was found by chest radiography preoperatively, and a giant Meckel's diverticulum was located within the left hemithorax during surgery. ( info) |
230/781. Right-sided diaphragmatic hernia following group B streptococcal pneumonia and sepsis. A case of right-sided diaphragmatic hernia following group B streptococcal pneumonia and sepsis is reported herein. The clinical course was characteristic. The position of the right hemidiaphragm was initially normal. After an antecedent group B streptococcal infection, an abnormal shadow indicating either pneumonia or a pleural effusion on the chest x-ray was recognized and an elevation of the bowel and liver into the right hemithorax gradually appeared. Repair of the hernia was indicated and the postoperative result was excellent. The relationship between a delayed-onset diaphragmatic hernia and a group B streptococcal infection is still unknown. Increased intrathoracic pressure caused by mechanical ventilation coupled with an abnormal lung compliance due to inflammation may have resulted in the delayed herniation. Among various methods for diagnosis applied, chest x-ray and ultrasonography were noninvasive and useful. ( info) |