Jillian Orford

Bleomycin therapy for cystic hygroma

Intralesional bleomycin injection was used as sclerosant therapy for sixteen patients with cystic hygroma. An excellent (complete clinical resolution) response was obtained in seven (44%) patients, a good (> 50% response) result in seven (44%), and a poor or no response in two (12%). Minor transient side effects (fever, vomiting, cellulitis, skin discoloration) were seen in six patients, and there were no serious side effects. The results suggest that bleomycin intralesional sclerosant is effective therapy for cystic hygroma, with response rates comparable to those of surgical removal, but with the advantage of avoiding inadvertent nerve damage and scarring.

Advances in the treatment of oesophageal atresia over three decades: the 1970s and the 1990s

Oesophageal atresia management has evolved alongside the development of paediatric surgery. An analysis of a 30-year prospective collection of oesophageal atresia cases treated at the Royal Alexandra Hospital for Children, NSW, Australia is presented. There has been a dramatic change in the surgical approach. Fewer infants are undergoing operations of cervical oesophagostomy, gastrostomy and oesophageal replacement. More, including fragile infants, are surviving with a repaired native oesophagus. Fewer infants are suffering morbidity secondary to anastomotic leak. The progression in treatment has been enabled by improved neonatal support due to advances in neonatology, neonatal anaesthesia, nutritional support and antimicrobial therapy. Oesophageal atresia treatment and outcome has changed markedly over three decades. Cooperative multi-centre database development is now required to provide data in order to further refine treatment for clinical challenges that remain.

A prospective comparison of tissue glue versus sutures for circumcision

Abstract.The safety of wound closure with tissue glue after surgery has been well established. The efficacy of its use in circumcision is poorly documented. The aim of this study was to carry out a comparative study of tissue glue versus suture closure after circumcision. The duration of the operative procedure, pain score, cosmesis and postoperative complications were evaluated. There were no complications in either group. There was no statistically significant difference in the pain score in both groups. However the mean time taken for tissue glue was 16.6 minutes and the mean time taken for sutures was 23.7 minutes. (p < 0.0001) which was statistically significant. The cosmetic appearance was found to be superior in the tissue glue group as there were no suture marks on the join of the shaft skin and foreskin base.

Dose response relationship between adriamycin and birth defects in a rat model of VATER association

BACKGROUND/PURPOSE The aim of this study was to reproduce the animal model of esophageal atresia and the VATER association. The purpose was a dose response analysis of Adriamycin teratogenicity in the rat and comparison of these anomalies with the human VATER association. METHODS Twenty time-mated Sprague-Dawley rats received intraperitoneal Adriamycin (dose range, 0 to 2.5 mg/kg/d) for 4 consecutive gestational days E6 to E9. The embryos were harvested on day E21, inspected, weighed, and dissected with a binocular dissecting microscope. Statistical analysis was performed with exact chi2. RESULTS Threshold doses of 1.25 and 1.5 mg/kg/d Adriamycin produced renal and gastrointestinal anomalies, respectively (exact chi2, P < .00001). In doses below 1.25 mg/kg/d Adriamycin, no anomalies were seen, and in above-threshold doses, the frequency of anomalies rose sharply as the dose increased. At 2.25 mg/kg/d of Adriamycin all embryos were abnormal, and Adriamycin at 2.5 mg/kg/d led to resorption of all embryos. CONCLUSIONS Adriamycin induced esophageal atresia, and VATER in the rat is a reproducible model that has many similarities to the VATER anomalies in the human. There is a relationship between dose and the frequency as well as severity of anomalies. Further studies of this model are likely to provide information relevant to the understanding of this human congenital disease.

Androgen and oestrogen receptor status of the human appendix testis.

Since the human appendix testis is a Mullerian duct derivative it has been theorised that rising oestrogen levels in puberty result in enlargement of this organ and consequent predisposition to torsion. This study sought to establish the presence or absence of both androgen and oestrogen receptors in the human appendix testis. Bilateral appendix testis specimens surgically excised from ten patients undergoing scrotal exploration for acute scrotum were stained immunohistochemically for androgen and oestrogen receptors. These were examined by light microscopy. The human appendix testis was found to express both oestrogen and androgen receptors. Marked regional variation of androgen and oestrogen receptor positivity was demonstrated. The surface epithelium and some stromal cells were androgen receptor positive, whereas oestrogen receptors were confined to ductular invaginations, gland-like structures and some stromal cells. Rising levels of androgens and oestrogens in pubertal boys may account for enlargement and the predisposition of the human appendix testis for torsion since this structure contains receptors for both.

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.

Timing of morphologic and apoptotic changes in the sheep fetal kidney in response to bladder outflow obstruction.

PURPOSE Posterior urethral valves are the main cause of bladder outflow obstruction in human fetuses. Thirty per cent of boys with valves develop end-stage renal disease, despite intervention in the postnatal period. The timing and mechanisms of renal damage in bladder outflow obstruction are unknown. We investigated the timing of changes in morphology and apoptosis in the fetal sheep kidney in response to obstruction. MATERIALS AND METHODS Thirty-three fetal lambs at day 70 of gestation underwent surgical creation of bladder outflow obstruction. Twenty-nine fetal lambs had sham surgery. Fetal kidneys were collected 2, 5, 10, 20 and 30 days after surgery. Renal histology was examined. Real-time PCR was used to quantify the renal cortical expression of the pro-apoptotic gene Bax and anti-apoptotic gene Bcl-X. The TUNEL technique was used to assess regional renal apoptosis in response to obstruction. RESULTS Changes in renal morphology were evident as early as 2 days after surgery in fetuses with bladder outflow obstruction, and progressed over 20-30 days to cystic renal dysplasia. Bladder outflow obstruction increased the renal cortical expression of Bax relative to Bcl-X. Tubular apoptosis peaked after 2 days of obstruction. Blastemal apoptosis peaked after 5 days of obstruction. CONCLUSIONS Changes in pro- and anti-apoptotic gene expression in the fetal renal cortex, and alterations in the number of apoptotic cells and renal morphology are evident soon after the onset of bladder outflow obstruction. These findings suggest that damage to the developing fetal kidney begins to occur at the onset of obstruction. Attempts to preserve renal function by antenatal interventions may best be achieved by early treatment.