Circumcision Rates after the Release of Preputial Adhesions

Aim We aim to determine if the release of preputial adhesions (RPA) successfully managed symptoms related to physiological phimosis and prevented the need for circumcision. Methods We performed a retrospective review and analysed data on RPA procedures performed between January 2005 and December 2017. Results 534 RPA's were performed. Median age at RPA was 52.7 months (range: 3-197 months). Mean follow-up was 108 months (range: 4.7 to 152.4 months). 44 children and 1 child subsequently required a circumcision or preputioplasty respectively (8.4% incidence). There was no statistical difference in the circumcision rates in children who had RPA over 5 years vs those that had RPA under 5 years old (6.6% vs 9.8%; p = 0.21). The histology of the 44 excised foreskins showed BXO in 2 (4.5%) and chronic inflammation in 11 (25%). Conclusion RPA is an effective alternative to circumcision where either reassurance on the benign and self-limiting nature of physiologic phimosis or steroid treatment are unsuccessful in managing symptoms.

The national incidence and outcomes of gastroschisis repairs.

The birth prevalence of gastroschisis worldwide has increased over the past decades. We aim to determine the Irish national incidence of gastroschisis repairs (NIGR) over a 5 year period (2007- 2011) and clinical outcomes by a retrospective cohort review of cases admitted to all Irish paediatric surgical units. Seventy patients were identified. The NIGR per 10,000 live births was 1.96 (SD 0.51) per year. Fifty eight (82%) were antenatally detected. Twenty eight (40%) had primary repair day 1 with the remaining repaired in a median of 3(2-5.75) days. Thirty three (47%) experienced a central catheter related infection. Duration of stay was significantly correlated with decreasing gestational age (p = 0.016), decreasing birthweight (p = 0.005), increasing numbers of blood transfusions (p < 0.001) and co-morbidity or complication (p < 0.001). This study provides individual centres with patient outcomes and national data that can be provided to parents and clinical staff regarding the clinical course of gastroschisis.

Animal attack: an unusual case of multiple trauma in childhood.

A 2½ year old girl attended our facility following attack by a tapir at a city zoo. She sustained multiple injuries including a forearm laceration and multiple perforating wounds to her abdominal wall. She had several procedures, including bowel resection, performed under the care of the General Paediatric Surgery and Plastic Surgery teams and was treated with a course of IV antibiotics. She recovered well and to date has suffered no long-term adverse outcome.

Patterns of internet use by parents of children attending a pediatric surgical service.

PURPOSE: The internet has revolutionised the way we search for information. We determined the level of internet use by parents of children attending general surgical services and identified trends in online information-seeking behaviour. METHODS: A questionnaire based on the work by Boston and Tassone was distributed to parents attending both the day surgical units and surgical outpatients department in a paediatric tertiary referral centre. RESULTS: There were 214 (82.3 %) questionnaires returned, with 82 (38.3 %) of respondents having searched the internet regarding their child's surgical issue. Access to a smartphone, a university education and private health insurance were factors that positively influenced online searching (p < 0.005). Of those respondents who searched the internet, 42 (51 %) felt that information they found online was understandable, while only 14 (17 %) admitted to online sourced information influencing the treatment decisions they had made for their children. When asked to rank information sources on Likert-type scales in terms of importance; parents ranked the surgeon as most important (mean = 4.73), whilst the internet ranked lowest (mean = 3.02). CONCLUSION: We demonstrated significant use of the internet amongst those attending paediatric general surgical services. Clinician sourced information remains important, however we should engage with patients to utilise this vast resource effectively.

The Adriamycin rat/mouse model and its importance to the paediatric surgeon.

The teratogenic effect of Adriamycin (doxorubicin) in the rat model, and more recently in the mouse, has provided paediatric surgeons with a reliable, easily reproducible method of studying the embryology and molecular biology for a range of complex congenital anomalies. Concomitantly these animal models have stimulated interest among embryologists for the effect on the notochord, shedding more light on the important organizational role of this structure in the developing embryo. Finally, as more is learnt of the pathogenesis of the various malformations induced by Adriamycin, future therapeutic interventions involving gene therapy, drugs or surgery may arise. This article reviews the establishment of the Adriamycin rat and mouse models, examines their impact on various congenital malformations, and suggests targets for further research.

Penile malignant peripheral nerve sheath tumour (schwannoma) in a three-year-old child without evidence of neurofibromatosis.

Malignant schwannoma (malignant peripheral nerve sheath tumour, MPNST) is a rare high-grade tumour arising from peripheral nerves. We report the case of a 3-year-old male who presented with a non-tender lesion on the dorsum of his penis. The lesion was excised and a formal circumcision performed. Histology of the lesion revealed a spindle cell tumour. Immunohistochemistry showed the tumour cells to be strongly positive for S100 and Vimentin. A diagnosis of intermediate grade malignant peripheral nerve sheath tumour was made. Malignant schwannoma is rare in children and is previously unreported in the penis in the paediatric age group without evidence of neurofibromatosis.

Mucinous cystadenoma of the ovary in perimenarchal girls.

Ovarian masses in children are an uncommon occurrence. They represent less than 2% of all tumours in girls less than 16 years of age. Mucinous tumours of the ovary occur principally in middle adult life and are extremely rare prior to menarche. To the best of our knowledge, there are only 13 previous cases of benign mucinous cystadenoma (MCA) of the ovary in perimenarchal girls reported in the literature. We present six cases of this rare tumour. We reviewed the charts of six patients who presented with large MCA of the ovary. The patient's ages ranged from 13 to 14 years (mean 13.6 years). Two were premenarchal and four were within 1 year of menarche. All children presented with marked abdominal distension and discomfort. Except for one child who had ultrasound scan alone, all the others had either CT or MRI scan as well. Ultrasound demonstrated a large multiloculated cystic mass arising from the pelvis reaching the level of the xiphoid. CT demonstrated an enormous mass occupying almost the entire abdomen. The mass was partly solid, partly cystic and the cystic elements were multiloculated in all patients. Three patients demonstrated contralateral hydronephrosis on imaging. Laparotomy revealed a tumour arising from the left ovary in five patients and from the right ovary in one. Several litres of fluid were aspirated in order to deliver the tumour from the abdomen. All patients underwent oophorectomy or salpingo-oophorectomy. Histology revealed benign MCA of the ovary in all cases. On follow up, ranging from 2.4 to 5 years, all patients were well with no evidence of recurrence. MCA in perimenarchal girls usually affects the left ovary. Although this tumour is rare, this diagnosis should be considered in 11 to 15-year-old girls presenting with a very large abdominal mass.

Bilateral megaureters in the Adriamycin rat model.

Congenital obstructive uropathy is associated with significant morbidity and mortality in the human neonate. The pathophysiology of congenital obstructive uropathy is poorly understood. There are very few experimental models of prenatal obstruction of the urinary tract, except in the fetal lamb or inbred rats. Prenatal exposure to Adriamycin in a rat model leads to a spectrum of malformations including urinary tract anomalies. We hypothesized that Adriamycin administration during a particular time frame could yield a high incidence of urinary tract anomalies and therefore designed this study to investigate the rates of urinary tract anomalies at different windows of Adriamycin injection in rat embryos. Adriamycin (1.75 mg/kg) was administered intraperitoneally to pregnant rats at different times from days 6 to 10 of gestation. Control animals were given saline. Embryos recovered on gestational day 21 by cesarean section were examined for urinary tract anomalies, and malformations were noted. Sections were then processed for paraffin embedding, sectioned at 5 mum, and stained with hematoxylin and eosin for histological examination. Anomalies of the urinary tract occurred maximally following Adriamycin administration on days 7, 8, and 9 of gestation (91.6%) compared with 16% of controls. The most common urinary tract anomaly in the Adriamycin group was bilateral megaureters with a hypoplastic bladder (81%). Other anomalies included unilateral or bilateral ureterohydronephrosis with a normal-sized bladder, duplex kidney, and unilateral or bilateral renal agenesis. In conclusion, the critical embryologic window for the development of bilateral megaureters with a small bladder in the Adriamycin rat model occurs following Adriamycin administration on gestational days 7-9. This simple experimental model of bilateral megaureter may allow further research into the pathophysiology of this condition.

Adriamycin induces notochord hypertrophy with conservation of sonic hedgehog expression in abnormal ectopic notochord in the adriamycin rat model.

BACKGROUND/PURPOSE: The Adriamycin rat model (ARM) is a well-established model of the Vertebral, Anorectal, Cardiac, Tracheoesophageal, Renal, Limb (VACTERL) association. The notochord, which expresses Sonic Hedgehog (Shh), has been found to be grossly malformed with ventral ectopic branches in the foregut region of embryos in the ARM. The authors designed this study to test the hypothesis that Shh-expressing ectopic notochord could contribute to an increased volume of notochord relative to total embryo volume, resulting in an increased concentration of Shh in the notochord of affected embryos. METHODS: Adriamycin was administered intraperitoneally to rats on days 7 (E7), E8, and E9 of gestation and saline to control animals. Embryos recovered at E12 and E14 were examined immunohistochemically for Shh expression. Quantitative morphology using the Cavalieri technique was performed to determine embryo and notochord volume. RESULTS: Embryos in both Adriamycin and control groups at E12 and E14 showed comparable levels of Shh expression in notochord at all locations. The percentage of notochord per embryo was significantly increased in Adriamycin embryos at E12 and E14 compared with equivalent controls. CONCLUSIONS: These data suggest that Adriamycin induces notochord hypertrophy. With all regions of the notochord secreting Shh, this could result in a higher concentration gradient of Shh in close abnormal proximity to the foregut, possibly contributing to the malformations found in the VACTERL association.

Increased fibronectin expression in developing embryos is associated with abnormal notochord in the Adriamycin rat model.

BACKGROUND: The VACTERL association is a spectrum of clinical conditions, including esophageal atresia (EA) and tracheoesophageal fistula (TEF), which affects approximately 1 in 5,000 live human births. The administration of intraperitoneal Adriamycin to pregnant rats reliably induces anomalies, such as EA and TEF, in their offspring, in what is known as the Adriamycin rat model (ARM). In affected embryos the presence of gross notochord abnormalities is commonly found, with typical features being ectopic ventral branches and adherence of the notochord to the foregut. Fibronectin (FN) is an extracellular matrix (ECM) glycoprotein present on most cell surfaces, in extracellular fluids and in plasma. FN is involved in various functions, including cell adhesion, cell motility and wound healing. Previous studies in rats have shown that a single dose of Adriamycin can produce an appreciable rise in FN levels in various organs such as kidney and heart. We hypothesised that Adriamycin administration could promote upregulation of FN expression contributing to increased gut-notochord adherence and the development of abnormal ventral notochordal branching in the ARM. This study was designed to investigate FN expression in ARM embryos. METHODS: Adriamycin (1.75 mg/kg) was administered intraperitoneally to pregnant rats on days 7,8 and 9 of gestation (E7, E8 and E9 respectively). Control animals were given saline. Embryos recovered on E10-E14 were fixed, embedded in paraffin and sectioned. Immunohistochemistry using an anti-FN rabbit polyclonal antibody was performed. RESULTS: FN expression in both Adriamycin and control embryos on E10, E11 and E12 was comparable. However, the levels of FN expression in Adriamycin embryos on E13 and E14 were significantly greater in embryos with abnormal notochords than in equivalent control embryos. CONCLUSION: Adriamycin-induced increased expression of FN, in the ARM, may contribute to abnormal notochord development leading to the VACTERL association.

The timing of enteric neural crest cell colonisation of the chick embryo cloaca.

Neural crest cell (NCC) migration and formation of the enteric nervous system (ENS) is an essential process in the development of the normal human gut. Abnormalities of the ENS lead to a number of neurochristopathies. In avian embryos, the cloaca acts as a common chamber into which gastrointestinal, urinary and genital tracts emerge. Previous studies have elucidated the specific timeframes at which NCCs reach the various regions of the developing chick gut but, to date, none have looked at NCC colonisation of the cloaca. The aim of our study was to investigate the exact timing of the appearance of NCCs in the cloaca of chick embryos. Chicken embryos were harvested on embryonic days (E) 8-12. Whole embryos were fixed, embedded in paraffin and sectioned. Fluorescent immunohistochemistry, using an anti-HNK-1/N-CAM monoclonal antibody, was performed and images were obtained by confocal microscopy. There was no evidence of NCCs in the cloaca of embryos from E8 to E11. Intense immunoreactivity to HNK-1 first appeared in the cloaca of E12 embryos, demonstrating a profuse circumferential colonisation by NCCs at this time. Our study is the first to show the exact timing of enteric NCC colonisation of the chick embryo cloaca. Further studies, involving quail-chick chimeras, are required to establish the true origin of cloacal NCCs and to establish the relationship between NCCs and persistent cloaca.

Adriamycin effects on the chick embryo.

Adriamycin is an anthracycline, anti-neoplastic drug with known teratogenic effects on foetal rats in what is known as the Adriamycin rat model (ARM). This includes conditions similar to those in newborn humans, known collectively as the VACTERL association. This comprises vertebral (V), anorectal (A), cardiac (C), tracheoesophageal (TE), renal (R) and limb (L) anomalies. We designed this study to test the hypothesis that the administration of Adriamycin to chick embryos would cause similar anomalies to those in the VACTERL association seen in the ARM. Fertilized Ross eggs received Adriamycin doses from 2-50 microg into the air sac and from 0.9-6 microg into the albumin. Administration varied from day 0-3 (D(0-3)) with D(0) being the first day of incubation. Control eggs received saline. Embryos were incubated at 38 degrees C and a relative humidity of 70%. Embryos were recovered on D(14), paraffin-embedded and transverse sections studied for morphological abnormalities. In the air sac group ( n=142), 71% of Adriamycin embryos survived versus 86% of controls (n=29). In the albumin group (n=121), 42% of Adriamycin embryos survived versus 55% of controls (n=69). No embryos demonstrated anomalies consistent with the VACTERL association. Ventral defects affected 1% of surviving Adriamycin embryos versus 4% of controls in the air sac group. In the albumin group, 19.8% of surviving Adriamycin embryos had ventral defects compared to 15.7% of surviving controls. Anophthalmia affected 1% of the surviving embryos in the Adriamycin air sac group and 2% of the Adriamycin albumin group. No controls developed anophthalmia. Exencephaly affected 2% of the survivors in the Adriamycin air sac group but none of the albumin group or controls. The administration of Adriamycin to chick embryos in comparable doses and times to those used in the ARM does not appear to produce comparable effects in relation to developmental anomalies, such as the VACTERL association. Despite examining different administration routes and mimicking the ARM, by giving Adriamycin to embryos at gastrulation, we were unable to re-create the anomalies seen in the ARM.