Congenital diaphragmatic hernia: associated anomalies and antenatal diagnosis

Congenital diaphragmatic hernia: associated anomalies and antenatal diagnosis

602 experience and strategy in the management of pulmonary hydatid disease. Sixty-six patients with 83 pulmonary hydatid cysts underwent surgical trea...

46KB Sizes 3 Downloads 104 Views

602 experience and strategy in the management of pulmonary hydatid disease. Sixty-six patients with 83 pulmonary hydatid cysts underwent surgical treatment. Double-lumen endotracheal tubes were used in children over 12 years of age. There were 38 boys and 28 girls with a mean age of 9.6 F 7 years (range 5-15 yrs). Of the 83 cysts, 61 were intact and 22 were ruptured. Isolated pulmonary hydatid cysts were seen in 61 patients (92.4%), while 5 patients (7.6%) had combined pulmonary and hepatic cysts. Lateral thoracotomy was performed in 54 patients (82%), thoracophrenotomy in 5, bilateral thoracotomy 4, and median sternotomy in 3 patients. Cystectomy and capitonnage were performed in 58 cysts, cystotomy alone in 21, and resection techniques in 4 patients. There were 8 postoperative complications in 7 patients. Atelectasis was the most common complication. It is concluded that surgery is the treatment of choice for most patients with pulmonary hydatid disease. The use of double-lumen tubes may decrease intra- and postoperative complications. Thoracophrenotomy can be chosen as the surgical procedure in the management of simultaneous pulmonary and hepatic cysts. — Thomas A. Angerpointner The vagus and recurrent laryngeal nerves in experimental congenital diaphragmatic hernia Martinez L, Gonza´les-Reyes S, Burgos E, et al. Pediatr Surg Int 2004 (April);20:253-257. Nitrofen induced congenital diaphragmatic hernia (CDH) in rats shows associated malformations similar to those seen in human CDH. This includes a disturbed regulatory action of the neural crest (NC) on the organogenesis of the pharyngeal pouch and some derivatives. Because even the vagus and the recurrent laryngeal nerves derive from the NC, the authors investigated whether these nerves are anatomically abnormal in CDH fetal rats. Because CDH is often associated with gastroesophageal reflux (GER), disturbance of NC and abnormality of vagus and recurrent laryngeal nerves may explain esophageal dysfunction. One group of rats received nitrofen, another group served as controls. The fetuses were recovered one day before term, killed and evaluated for the presence of CDH. A thoracic block was sectioned, stained with HE and assessed for the vagus and recurrent laryngeal nerves. A computer-assisted three-dimensional reconstruction was done. Nine control fetuses without CDH were compared with 9 fetuses with CDH. The anatomy of the vagus nerves and the recurrent laryngeal nerves was normal in the control group, but were grossly abnormal in seven of nine fetuses with CDH. In 4 fetuses, one of the vagus trunks was absent. Two of them presented an additional absence of the recurrent laryngeal nerve on the side of the normal vagus. In two animals a marked hypoplasia of the vagus trunk was found. One of these two and one further rat showed anomalies in the course of one of the nerves. In three animals, even an aberrant right subclavian artery was mentioned. GER after repair of CDH is explained by malposition of the gastroesophageal junction or increased abdominal pressure. But sometimes a dilated, fluid- or air-filled esophagus is found, suggesting functional disturbance of the esophagus himself. The presented work showed gross malformation of the NC-derived vagal and recurrent laryngeal nerves. Anomalies of this nature may even be present in newborn babies with CDH. If these anomalies have counterparts in human, GER in this patients may derive more from esophageal innervational failure than from extraesophageal reasons. —Peter Schmittenbecher

International Abstracts counsel the specific population, to direct available therapy and to identify patients that may benefit from special therapies. Within 14 years, 77 patients with CDH were presented to two children hospitals. Data on maternal and infant demographics, prenatal fetal ultrasound parameters, outcome of pregnancy, associated anomalies, and infant hospital course were collected. 6.5 F 1.9 cases per year were seen. Mean age of mothers was 27.4 years, there was no history of stillborn. 89% of pregnancies resulted in live births, 1.4% in stillbirth and 9.6% in voluntary interruption. Survival of N30 days was 53.8%, neonatal death 30.8% and late death 1.5%. The fact of prenatal diagnosis did not influence survival. The fetal ultrasound parameters CC/AC (chest circumference/abdominal circumference) and CC/HC (.../head circumference) were useful to predict prognosis, because all cases with a CC/AC ratio b0.95 and a CC/HC ratio b0.8 died, but 2/3 of children with ratios above these levels survived. Birthweight and gestational age of survivors and non-survivors did not differ. Nearly 2/3 of the children went on ECMO. 52% of ECMO children and 77% of non-ECMO patients survived. Forty-three percent had associated anomalies. Cardiac anomalies were most frequent (52%), followed by muscoloskeletal (20%), CNS (12.5%), chromosomal (6.3%), genitourinary (5%) or gastrointestinal anomalies (3%). Any major associated anomaly resulted in a poorer outcome (43.5% survival), whereas survival in isolated CDH was 70%, without a difference between left- and rightsided defects. The worst outcome indicators were pulmonary hypoplasia and the presence of liver, stomach, or spleen in the thorax. Comparing the data with a recent metaanalysis of mortality factors, survival rates were found uniformly higher. Fetal ultrasound parameters indicating pulmonary hypoplasia were helpful to calculate the prognosis as it is known from the literature for similar parameters like lung area to head circumference ratio (LHR). Once again, liver herniation especially in left sided hernia was a poor prognostic parameter. Fetus with leftsided CDH, liver herniation and low LHR benefit from newer therapeutic strategies like ECMO. Contrary to other studies, prenatal diagnosis did not worse the prognosis. This may be caused by the growing number of prenatal examinations and diagnosis. —Peter Schmittenbecher

Alimentary Tract Tracheoesophageal fistula due to disc-battery ingestion Alkan M, Bu¨yu¨kyavuz I, Dogru D, et al. Eur J Pediatr Surg 2004 (August); 14:274-278. Ingestion of a disc-battery can lead to hazardous complications including tracheo-esophageal fistula (TEF), particularly if the battery is impacted in the esophagus. Urgent esophagoscopic removal of the battery is essential in these cases. Persisting respiratory symptoms and/or feeding problems after removal should alert the surgeon to evaluate for TEF. Esophagography and/or esophagoscopy should be performed. Once TEF is identified, conservative treatment is the initial treatment of choice. Delayed surgical closure is necessary if spontaneous closure does not occur. The authors present a case of TEF secondary to discbattery ingestion with review of the current literature and discuss the management of this potentially life-threatening complication. —Thomas A. Angerpointner Post-traumatic duodenopancreatectomy in a child

Congenital diaphragmatic hernia: associated anomalies and antenatal diagnosis Bedoyan JK, Blackwell SC, Treadwell MC, et al. Pediatr Surg Int 2004 (March);20:170-176. The neonatal mortality rate of all antenatally diagnosed isolated congenital diaphragmatic hernia (CDH) is still ranging from 50% to 76%, but survival is enhanced by ECMO, NO and in utero fetal surgery. The authors reviewed CDH cases from their area’s healthcare facilities to get sufficient database to

Salman FT, Emre A, Abbasoglu L, et al. Eur J Pediatr Surg 2004 (August);14:287-289. The authors report on an 5-year-old boy who complained of severe abdominal pain after a car accident. He was sitting on the lap of the driver and his abdomen was pressed closely between the pregnant driver and the steering wheel. At laparotomy, associated injuries of the duodenum and the pancreas were detected which could not be repaired. The transsected pancreas and extensive duodenal injury necessitated a