Nathan E. Wiseman

Congenital duodenal obstruction: The impact of an antenatal diagnosis

In a series of 34 infants with congenital duodenal obstruction, 15 were diagnosed by antenatal ultrasound between the fifth month of gestation and term. Fourteen patients were available for follow-up. In 10 of 14 cases, patients were available for follow-up. In 10 of 14 cases, parents were informed of the diagnosis antenatally and, in 9, were advised of the possibility of surgery in the neonatal period. Parents felt the information helped them prepare for the surgical and medical interventions necessary in the postnatal management of their infants. The mean birth weight and gestational age of these infants were comparable with those diagnosed postnatally. Associated congenital anomalies were more common in the patients with a postnatal diagnosis. In the total group of 34 infants with duodenal obstruction, 32 had surgical repair with a survival rate of 94%. Surgical procedures were comparable between the two groups. Surgical intervention occurred earlier in the neonatal period when an antenatal diagnosis was made. Overall survival in the entire series was 88% with four deaths attributed to severe associated congenital anomalies. We conclude that (1) the antenatal diagnosis of duodenal obstruction influences parents positively in coping with the anomaly, and (2) although surgery was performed sooner, the outcome of infants with duodenal obstruction was not changed by providing an antenatal diagnosis.

Incarcerated congenital lumbar hernia associated with the lumbocostovertebral syndrome

Congenital lumbar hernias are uncommon, with only thirty-three reported cases. They have been associated with the lumbocostovertebral syndrome. This report describes a patient with a congenital lumbar hernia and manifestations of the lumbocostovertebral syndrome. The diagnosis of the hernia was delayed due to its juxtaposition to a posterior meningomyelocele, resulting in incarceration. The patients course was also complicated by recurrence of the lumbar hernia requiring prosthetic repair.

Lethal short-bowel syndrome

Infants with short-bowel syndrome are difficult to manage. Despite supportive measures with parenteral nutrition and surgery to lengthen remaining bowel or increase functional absorptive surface area, the outcome for many of these infants is poor. We have reviewed a series of seven infants diagnosed with severe short bowel. Causes included volvulus (3), multiple atresias (2), and total intestinal aganglionosis (2). Survival time ranged from 15 days to 8 months. During the hospital course, each infant underwent one to three operative procedures to diagnose and manage the short bowel and all received total parenteral nutrition (TPN) ranging from 10 days to 6 months. One infant died of liver failure and two others developed significant liver dysfunction secondary to TPN. Most infants remained hospitalized until their death. Death occurred at an average of 9 weeks following the diagnosis of short-bowel syndrome. This review suggests that infants with less than 6 cm of small bowel beyond the Ligament of Treitz will inevitably die of their disease or treatment complications. Until bowel transplant becomes a viable alternative, operative intervention and nutritional support may prolong survival but will not change the outcome of these infants and will only contribute to additional morbidity. A decision to withhold further therapy would be reasonable at the time the diagnosis is established.

A familial disorder with duodenal atresia and tetralogy of Fallot.

We report on two sibs with tetralogy of Fallot (TOF) and duodenal atresia (DA). The first child, a 6-year-old girl, had a right facial palsy in addition to the TOF and DA. Her brother, age 10 months, was born with bilateral microtia without facial palsy. The children are the product of an apparently non-consanguineous union between clinically normal parents. The pertinent family history includes a paternal aunt with TOF and a cleft lip and palate who died in childhood and another paternal aunt with a supernumerary thumb. This family has anomalies found in several syndromes, but does not meet the diagnostic criteria for any of them. The genetic basis for this condition remains unknown, but the pattern of inheritance is likely either autosomal recessive, or autosomal dominant with variable expression and reduced penetrance. The pathogenesis is unknown, but either a disturbance in neural crest cell migration or familial predisposition to vascular disruption might explain this pattern of malformations.

Rigid bronchoscopy in the pediatric age group: Diagnostic effectiveness

Over a 15-year period, 277 diagnostic bronchoscopic procedures were carried out at the Winnipeg Childrens Hospital using rigid bronchoscopic instrumentation. The objective of the review is to examine contribution to final diagnosis made by the procedure. The patient population included 60% male and 40% female patients ranging from the first day of life to 18 years (mean age, 6 years). Indications for bronchoscopy fell into two large groups, including 60% of patients with evidence of lower airway disease and 30% of patients with evidence of upper airway obstruction. In patients with upper airway obstruction, half were found to have a congenital underlying cause and half were due to an acquired lesion. In 85% of patients, a specific diagnosis was reached and this proved to correlate positively with the preoperative diagnosis in 80% of patients and negatively in 20%. Definitive treatment in patients with upper airway obstruction included surgical intervention in one third of patients. Patients with lower airway disease were diagnosed as having consolidation in 43%, atelectasis in 39%, and bronchiectasis in 18%. Disease localized most frequently to the left lower and right upper lobes and in only 10 of 168 patients was a congenital cause determined. Among 168 patients, 30 had surgical treatment as the definitive management with the majority of patients treated medically. Bronchoscopy was shown to contribute to diagnosis in 88% of patients examined. Bronchoscopy was carried out with a complication rate of 3% and no mortality.

Applying vacuum to accomplish reduced wound infections in laparoscopic pediatric surgery

INTRODUCTION The prevention of surgical site infections has received little attention in pediatric surgery. Negative pressure wound therapy is used to treat complex wounds. We hypothesized that this principle could reduce wound infection rates following laparoscopic surgery. We tested this in a randomized controlled trial. MATERIALS AND METHODS We randomized pediatric patients with an umbilical port site to a standard dressing or a vacuum dressing. The dressings were removed 48h after surgery. A nurse blinded for the treatment inspected the umbilical wound between post-operative days 7-10 for infection. Data comparison was performed using a Fisher exact test with p<0.05 defined as significant. RESULTS We recruited 90 patients over 2 years and randomized 44 to the vacuum dressing arm and 42 to the control arm. We observed a 2.8% (n=1/35) infection rate in the vacuum dressing group and 3.3% (n=1/30) in the control group (p=1.0). DISCUSSION We ended our study early when an interim analysis showed an impractical number of patients would be required to achieve sufficient power. We did not find a significant difference between the control and vacuum dressings in reducing post-operative wound infections. LEVEL OF EVIDENCE 3.

Antenatal Diagnosis, Perinatal Management, and Pulmonary Complications as a Result of Laryngeal Compromise in Fraser Syndrome

This report describes an infant with laryngeal obstruction as a part of Fraser syndrome. The antenatal findings, perinatal management, and pulmonary complications of this condition are presented with a review of the literature.