Edward Shi

The radiologist says malrotation: does the surgeon operate?

Abstract The management of malrotation when it is an incidental finding is unclear. This retrospective study reports an analysis of radiological and operative findings in a series of 71 patients. There were no deaths. We report a false-positive rate of 15% for upper gastrointestinal contrast studies reported as showing malrotation. Our findings and a review of the literature demonstrate that in the asymptomatic child over 2 years of age, the evidence supporting mandatory correction of malrotation is weak.

Oesophageal atresia in twins

Abstract The twin incidence is higher in infants with oesophageal atresia (OA) than in the general population. The purpose of this study was to review the twin OA information from five institutions and evaluate possible links between the development of OA and the twinning process. Data were compared, combined, and analysed. There was a total of 1215 infants with OA, of whom 50 were from a twin pregnancy and 1 from a triplet pregnancy. Two sets of twins were concordant for OA. Mean birth weights and gestational ages were lower in the twin infants (P < 0.0005) and survival was lower in twins (65%, P < 0.005) than singletons. The anatomical variant of pure OA without fistula was seen in proportionally fewer twins (4%) than in singletons (7%). Multiple anomalies were present in 40% of twins compared with 33% of singletons, although this did not reach statistical significance. OA in our multicentre population was more common in twins. Several possible mechanisms are put forward to explain the apparent link between twinning and OA. Further analysis of this aspect of OA may aid in understanding the aetiology of this congenital anomaly.

Congenital spigelian hernia with cryptorchidism

Congenital spigelian hernia (SH) is a rarity. The authors present two cases of congenital SH with undescended testis, and examine the significance of the concurrence of these events.

Gastroduodenal intussusception due to Peutz-Jeghers Syndrome in infancy

Abstract A case of Peutz-Jeghers Syndrome (PJS) presenting in infancy with gastric-outlet obstruction is described. PJS may become symptomatic at any age and should be suspected when there are obstructive symptoms or gastrointestinal blood loss in a baby with a positive familiy history. Contrast studies and endoscopy are useful in diagnosis and surveillance. Treatment requires a combination of endoscopy and laparotomy/laparoscopy. Because of the reported cancer risk, we recommend surveillance from the age of diagnosis.

Neonatal duodenal obstruction: a review of 30 consecutive cases

Delayed return of normal duodenal function necessitating a prolonged hospital stay may often follow operative treatment of neonatal duodenal obstruction (NDO). Previously suggested ways to improve the operative result include variations in the anastomic technique, duodenal tapering, and the omission of gastrostomy. We have analysed the experience at the Prince of Wales Childrens Hospital of 30 consecutive cases of NDO treated over a 7-year period (1984–1990) in order to define factors in the treatment that might influence the establishment of oral feeding and duration of hospital stay. There were 19 males and 11 females, with duodenal atresia occurring in 10 and extrinsic or intrinsic causes of duodenal stenosis in the remaining 20. Two patients died without operation. Corrective procedures included 17 duodenoduodenostomies, 9 duodenoplasties, and 2 patients had lysis of Ladds bands that produced duodenal obstruction from the time of birth. Only 1 patient underwent duodenal tapering. There were 20 gastrostomies performed according to the preferences of the primary-care surgeons. It was shown that the duration of establishing oral feeding and hospital stay were not influenced by the type of corrective procedure, although Ladds bands duodenal obstruction was associated with the shortest hospital stay. The use of a gastrostomy was related to higher morbidity, a longer period to establish oral feeding, and a prolonged hospital stay. Prenatal diagnosis has also emerged as an important influencing factor. It is concluded that gastrostomy should be omitted in the treatment of NDO. The various types of corrective procedures were similar in final outcome in terms of morbidity and total hospitalisation time.

Isolated and multisystem hepatic trauma in children: the true role of non-operative management.

Abstract We retrospectively reviewed children with hepatic injuries (HI) admitted to our institutions from January 1982 to December 1999. Specific comparison was made of isolated (IHT) and multisystem hepatic trauma (MHT). Over the 18-year period 127 patients were identified with HI, 91 (71%) with MHT and 36 with IHT. The median age was 8 years (range 13 months to 14 years). Motor vehicle injury was the most common mechanism in both groups, but bicycle injuries were more common in IHT (P<0.001). Shock (P=0.02), the requirement for blood transfusion (P<0.001), and operative intervention for the HI (P=0.001) were all significantly more common in MHT. The distribution of liver injury grades was similar between the two groups. Twenty-two (17.3%) children died and in 18 the HI was the main cause or contributed significantly. There were 19 and 3 deaths in the MHT and IHT groups, respectively, a difference that was not significant. After excluding children with minor HI, 27 (39%) required operative intervention within 24 h of their injury. This suggests that the high success rate for non-operative management of HIs in the literature may have been biased by the inclusion of a significant proportion of subclinical injuries.

Early Posthepatoportoenterostomy Predictors of Native Liver Survival in Biliary Atresia

Objectives: Most infants with biliary atresia (BA) require liver transplantation (LT) after hepatoportoenterostomy (HPE), including those who initially clear jaundice. The aim of the present study was to identify clinical and routine laboratory factors in infants with BA post-HPE that predict native liver survival at 2 years. Methods: A retrospective cohort study was conducted in 217 patients with BA undergoing HPE in Sydney, Australia and Toronto, Canada between January 1986 and July 2009. Univariate and multivariate logistic regression using backwards-stepwise elimination identified variables at 3 months after HPE most associated with 2-year native liver survival. Results: Significant variables (P < 0.05) on univariate analysis included serum total bilirubin (TB) and albumin at 3 months post-HPE, bridging fibrosis or cirrhosis on initial liver biopsy, ascites of <3 months post-HPE, type 3 BA anatomy, age at HPE of >45 days, change in length z scores within 3 months of HPE, and center. On multivariate analysis, TB (P < 0.0001) and albumin (P = 0.02) at 3 months post-HPE, and center (P = 0.0003) were independently associated with native liver survival. Receiver operating characteristic analysis revealed an optimal cut-off value of TB <74 &mgr;mol/L (4.3 mg/dL; area under the receiver operating characteristic curve 0.8990) and serum albumin level >35 g/L (3.5 mg/dL; area under the receiver operating characteristic curve 0.7633) to predict 2-year native liver survival. TB and albumin levels 3 months post-HPE defined 3 groups (1: TB ⩽74 &mgr;mol/L, albumin >35 g/L; 2: TB ⩽74 &mgr;mol/L, albumin ⩽35 g/L; 3: TB >74 &mgr;mol/L) with distinct short- and long-term native liver survival rates (log-rank P < 0.001). Length z scores 3 months post-HPE were poorer for group 2 than group 1 (−0.91 vs −0.30, P = 0.0217) with similar rates of coagulopathy. Conclusions: Serum TB and albumin levels 3 months post-HPE independently predicted native liver survival in BA when controlling for center. Serum albumin level <35 g/L in infants with BA who were no longer jaundiced at 3 months post-HPE was a poor prognostic indicator. Poorer linear growth and absence of significant coagulopathy suggest a role for early aggressive nutritional therapy in this group.

Cisapride and caesarean section: their role in babies with gastroschisis.

Objective: The objective of this study was to compare the neonatal postoperative course and morbidity for patients with gastroschisis who received cisapride with those who did not receive cisapride.