Intestinal atresia and agenesis of the gallbladder in 2 siblings

Intestinal atresia and gallbladder agenesis are rare congenital malformations usually presenting as isolated and sporadic. We present and discuss the case of 2 sisters affected by a previously unreported association of these 2 anomalies

Right congenital diaphragmatic hernia: an 18-year experience

BACKGROUND: Prenatal diagnosis and outcome of right congenital diaphragmatic hernia (RCDH) are far less well known than the more common left CDH (LCDH). In addition, onset of RCDH beyond the neonatal period with a spectrum of atypical symptoms is not unusual. A retrospective analysis of RCDH treated at a single center over 18 years has been reviewed with regard to outcome after the introduction of a new treatment protocol for CDH. METHODS: All charts of patients with CDH between 1987 and 2004 were reviewed. Twenty-nine patients with RCDH were identified. The patients were divided into 2 historical groups: group 1, from 1987 to 1998 (16 patients), and group 2, from 1999 to 2004 (13 patients). From 1999, at the Department of Pediatric Surgery of Padua, the management of a baby born with CDH has been standardized and includes planned delivery at term, high-frequency oscillatory ventilation since birth, inhaled nitric oxide if required, extracorporeal membrane oxygenation as a "last resort," and delayed repair once the infant is hemodynamically stable. Patients with either prenatal diagnosis of RCDH or early onset of symptoms (6 hours) were considered "low risk." RESULTS: Of 29 patients, prenatal diagnosis was available in 8 (27.5%) and major associated malformations in 8 patients (27.5%). Eight (50%) of 16 cases from group 1 and 9 (69.2%) of 13 cases from group 2 were high-risk patients. There was a trend in favor of a higher survival among high-risk patients from group 2 (25% vs 44%), although this was not statistically significant. As expected, all low-risk patients survived (P = .0001). Plain thorax x-ray was diagnostic in 23 (82.1%) cases, initially normal in 3, not performed in 1, and misinterpreted as right lower lobe pneumonia in 2. At operation, the prosthetic patch was required in 2 (9%) of 22 cases and the peritoneal sac was found in 4 (13.7%). CONCLUSIONS: (1) The rate of prenatal diagnosis of RCDH was low and remained stable throughout the examined period. (2) The introduction of a treatment protocol, using high-frequency oscillatory ventilation since birth, improved the survival of high-risk patients with RCDH, although the data did not reach statistical significance. (3) The majority (75%) of low-risk patients presented beyond the first week of life with a variety of aspecific gastrointestinal or respiratory symptoms that accounted for initial misdiagnosis. (4) Even in these cases, the outcome was excellent

Congenital tracheobiliary fistula

Congenital respiratory tract-biliary fistula, including tracheo- and broncho-biliary fistulae, are rare developmental anomalies. To date, only 18 cases have been reported. We present two additional cases that came to our attention after a long and difficult attempt to make a diagnosis. After surgical excision of the tract both children have remained symptom-free for 6 and 4 years, respectively. Bronchoscopy allows an early diagnosis, but patients also have to be investigated for associated biliary tree malformations

Laparotomic versus laparoscopic Duhamel pull-through for hirschsprung disease in infants and children

PURPOSE: The aim of this study was to analyze the results from laparotomic and laparoscopic Duhamel pull-through in the treatment of Hirschsprung disease. MATERIALS AND METHODS: In our department, the Duhamel pull-through technique was adopted in most patients and the laparoscopic technique used since 1999 follows the original Duhamel procedure. Patients were identified retrospectively and followed up in our outpatient clinic. Preoperative, operative, and postoperative data were collected and analyzed. RESULTS: Between January 1992 and March 1999, 21 children with Hirschsprung disease underwent primary surgical correction using the classic open Duhamel pull-through. The mean age at operation was 14 months, the median operating time was 297 minutes, and the average postoperative stay was 10 days. From April 1999 to December 2003, 22 children underwent laparoscopic Duhamel pull-through. The mean age at operation was 14.6 months, the mean operative time was 253 minutes, and the mean postoperative stay was 6.8 days. The rates of early (4.7% vs. 4.7%) and late (19% vs. 23.8%) complications were similar in the two groups, but in the laparoscopic group the mean operative time and postoperative hospital stay were lower. CONCLUSION: With these encouraging results, laparoscopic Duhamel pull-through has become our procedure of choice in the surgical management of Hirschsprung's disease