S. W. Beasley

The response of phimosis to local steroid application

The effectiveness of topical steroid application in relieving phimosis was studied in 63 boys treated with local application of steroid ointment to the foreskin. Betamethasone valerate 0.05% (42 patients), hydrocortisone 1% (18 patients), or hydrocortisone 2% (3 patients) was applied three times daily for 4 weeks. Thirty-seven of the patients treated with 0.05% betamethasone valerate ointment (half-strength Betnovate) showed an initial improvement and circumcision was performed on 5 non-responders. Six patients showed initial improvement but later redeveloped phimosis: they were given a further course of treatment, resulting in 2 satisfactory responses and 4 failures requiring circumcision. Two patients using 2% hydrocortisone and 16 using 1% hydrocortisone ointment showed improvement, but 2 of the latter group ultimately required circumcision. Overall, a permanent improvement was achieved in 51 of the 63 patients, with the ability to retract the foreskin after one or more treatments. The remaining 12 boys required circumcision. Local application of steroid ointment to the foreskin results in resolution of phimosis in the majority of cases, but if the foreskin has a circumferential white scar, it is slightly less likely to respond. Following cessation of steriods, phimosis redevelops in a proportion of patients.

Oesophageal atresia without fistula — anastomosis or replacement?

At the Royal Childrens Hospital, Melbourne, 553 babies with oesophageal atresia and/or tracheo-eesophageal fistula have been admitted during the past 39 years; 36 (6.5%) of these had oesophageal atresia without a tracheo-oesophageal fistula. Definitive surgery was performed in 27 patients: the primary definitive procedure was oesphageal anastomosis in 15 and oesophageal replacement in 12. Aspects of diagnosis and selection of the most appropriate treatment modality are discussed, with the results of treatment presented. Our current policy is to perform an oesphhageal anastomosis whenever possible, and this has been successful in 7 of the last 11 patients.

Timing and embryology of esophageal atresia and tracheo-esophageal fistula.

The embryology of tracheo‐esophageal anomalies is controversial. The development of an adriamycin‐treated animal model has enabled improved understanding of the embryogenesis of these anomalies. Using this model, we aimed to describe the events leading to esophageal atresia and tracheo‐esophageal fistula.

Cardiovascular malformations in rat fetuses with oesophageal atresia and tracheo-oesophageal fistula induced by adriamycin

Associated congenital anomalies have emerged as the most significant prognostic factor in babies born with oesophageal atresia and/or tracheo-oesophageal fistula (OA-TOF). The most frequently encountered groups of anomalies are cardiovascular (CV) and gastrointestinal, the former being more significant from a prognostic point of view. Some, such as a right-sided aortic arch (RAA), vascular ring, or major heart defects, may alter the timing and surgical approach for the repair of OA-TOF or adversely affect the prognosis. The rat fetal OA model induced by adriamycin (Adr) has been described previously. In the present experiments, information was sought regarding the incidence and type of CV abnormalities in fetal rats obtained from Adr-treated dams. OA-TOF was induced in 24 of 36 fetal rats from Adr-treated dams. DV abnormalities were found in 18 (75%) OA-TOF fetuses and 10 (83%) Adr-treated fetuses without OA-TOF. The difference was not significant (P > 0.05). The most frequently found anomalies were ventricular and atrial septal defects. A RAA was present in 8/36 fetuses and a double aortic arch in 2/36. A patent ductus arteriosus was present in all treated fetuses compared with two-thirds of controls. The findings in the present study emphasise the importance of CV anomalies in association with OA, and reinforce the value of the Adr-induced rat fetal OA model by adding to our knowledge of the basic embryogenesis of both OA and CV anomalies.

Renal function and vesicoureteric reflux in children with ureteroceles

A total of 215 ureteroceles were present in 41 children with single-system and 154 children with double-system ureteroceles. Males were predominant in the single-system group while the reverse was true in patients with double-system ureteroceles. Single-system ureteroceles occurred more commonly on the left side. In double-system ureteroceles there was minimal renal function in 74% of the ureterocele-bearing moieties, 26% of the ipsilateral lower moieties, and 3% of the contralateral kidneys. Vesicoureteric reflux (VUR) was present in 17% of the ureterocele-bearing ureters, 54% of the ipsilateral second ureters, and 28% of the contralateral ureters. In the single-system ureteroceles, 20% of the ipsilateral kidneys had minimal renal function and 9% of both the ipsilateral and contralateral ureters had VUR. Differences in laterality, VUR, and function of the subservient renal tissue suggest that single-system ureteroceles are a different disease entity from the double-system type and should be considered separately in any discussion of the management of ureteroceles.

The surgical management of neonatal necrotizing enterocolitis, 1975–1984

A review was conducted of 202 neonates with necrotizing enterocolitis (NEC) seen at the Royal Childrens Hospital, Melbourne, over a 10-year period. The study population was biased towards the more severe cases and those requiring surgical intervention for complications. Most cases had one or more obstetric or perinatal stress factors present. Radiology was important in confirming the diagnosis and identifying those who required surgery. The indications for surgical intervention and the selection of the appropriate surgical procedure are discussed. Surgery was required for acute disease in 72 cases. In most of these, necrotic bowel was excised and temporary ostomies constructed. During the period of study the overall mortality decreased from 41% to 24%. Post-NEC strictures occurred in 18 patients, of whom 6 presented with bowel obstruction. The transverse and left colon seemed particularly susceptible to stricture formation. The diagnosis and management are described in detail.

Oesophageal atresia and associated anomalies : a plea for uniform documentation

Although associated congenital anomalies are the most significant factor affecting prognosis in babies with oesophageal atresia (OA), there is considerable variation in the methods by which they have been documented. To facilitate a uniform approach to the reporting of associated anomalies, we have analysed the data from our own experience and studied the methods used by other workers. During the period 1948–1991, 618 patients with OA and/or tracheo-oesophageal fistula were admitted to the Royal Childrens Hospital, Melbourne. Concurrent congenital abnormalities were present in 344 (56%), the most frequent being cardiovascular (20.7%); urinary (21.6%); gastrointestinal (22.7%); and orthopaedic (15.7%). Not only have reports of the incidence of associated anomalies in OA involved a wide variety of classifications, but also they have varied in the extent to which co-existent lesions were sought by investigation. These factors, in addition to the criteria used for inclusion and the method of their documentation, have resulted in marked discrepancies in the apparent incidence of specific anomalies, and made valid comparisons between series difficult or impossible. We advocate a simple approach whereby: (1) data should be collected prospectively; (2) routine investigation should include renal ultrasound and echocardiography (as a minimum requirement); (3) the number of associated anomalies should be reported as a percentage of the total cohort of patients, rather than as a percentage of the anomalies themselves; (4) true congenital anomalies should be recorded separately from acquired conditions; and (5) the wide variation in the impact of associated abnormalities on the management and long-term outlook in OA should be recognized.

Intussusception of vitello-intestinal tract through an exomphalos in trisomy 13

Intussusception of a patent vitello-intestinal tract through the dome of an exomphalos is extremely rare, but appears to have an association with trisomy 13. It can be distinguished from a ruptured exomphalos by: (1) its single limb (no loop of bowel); (2) its mucosal surface; and (3) an opening at its end.

Bezoar formation and malabsorption secondary to persistent dilatation and dysmotility of the duodenum after repair of proximal jejunal atresia

Bezoar formation is a rare but well-recognised complication of previous gastric surgery and persimmon ingestion. We present a case of bezoar formation in the duodenum of a child with a grossly dilated, dysmotile duodenum following surgery for jejunal atresia. The presenting symptoms were vomiting, diarrhoea, and growth failure, with evidence of malabsorption secondary to bacterial overgrowth in the bezoar. Plication of the duodenum to reduce its size and improve its motility may reduce the likelihood of future bezoar formation.

Crohn's disease : the Melbourne experience

During the years 1979–1988, 63 children (38 male: 25 female) were first diagnosed as having Crohns disease on the basis of histological findings. Twenty-nine per cent were less than 10 years old at the onset of symptoms; 40% of these cases took more than 1 year to diagnose. Most patients (59%) presented with abdominal pain and diarrhoea, but 40% presented with: a non-specific illness (14%); upper gastro-intestinal tract symptoms (14%); growth failure (6%); systemic disease (5%), or peri-anal disease (1%). There were signs of abdominal tenderness, distension, or an abdominal mass in over 50% of cases. Peri-anal Crohns disease was present in 41% and a height less than the 10th centile was recorded at diagnosis in 45%. Children were treated with a combination of prednisolone and sulphasalazine. In 16 cases a bowel resection was performed. Surgery was indicated for localised stricture formation causing obstruction and for persistence of severe disease poorly responsive to steroids, particularly in the prepubertal child with growth failure. The extent and distribution of disease influenced outcome. Twenty-four of 39 children followed for more than 3 years are either well-controlled or disease-free at present.