A Paediatric Hernia with a Twist: The Presentation, Imaging Findings and Management of a Strangulated Ovarian Hernia.

Indirect inguinal hernias are the most commonly encountered congenital abnormality in infants. They may be complicated by herniation of abdominal or pelvic viscus. In girls, a herniated ovary is a relatively common finding, however torsion of the ovary is infrequent. A tender irreducible inguinal hernia in an infant girl should raise the possibility of a strangulated herniated ovary as it requires urgent surgical attention. When in doubt, ultrasound with colour Doppler easily confirms the diagnosis. Here we present the case of an ovarian inguinal hernia which had undergone torsion and review the presentation, imaging findings and management.

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.

Batteries not included.

We report two cases of oesophageal lodgement of ingested button batteries (BB) in young children. In one case the diagnosis and subsequent treatment was made in a timely fashion and the patient suffered no sequelae. In the second case there was a delay in diagnosis and the patient subsequently suffered both early and late complications. The purpose of this report is to highlight theingestion importance of the correct management of suspected BB ingestion.

Delayed diagnosis of anorectal malformation--a persistent problem.

Delayed diagnosis of anorectal malformation (ARM) is an avoidable event associated with significant complications and morbidity. Previous studies have suggested higher than expected rates of delayed diagnosis, especially when a threshold of 24 hours of life is used to define delayed diagnosis. The aim of this study is to highlight the prevalence of delayed diagnosis of ARM in Ireland and to determine if any improvement in rates of delayed diagnosis of ARM has occurred since we previously examined this problem over a 10 year period in 2010. We compared trends in the incidence of delayed diagnosis of ARM between two cohorts, A (1999-2009) and B (2010-2012). Delayed diagnosis was defined as one occurring after 48 hours of life. Delayed diagnosis occurred in 29 cases (21.3%) in total, with no difference in the incidence of delayed diagnosis between cohort A (21 patients [21.2%]) and cohort B (8 patients [21.6%) being recorded. The rate of bowel perforation in patients with delayed diagnosis was 10.3% (3 cases). Our findings highlight the importance of a careful, comprehensive clinical examination in diagnosing ARM and suggest this is still sub-optimal. We strongly support the use of a nationally devised algorithm to aid diagnosis of ARM in order to avoid life-threatening complications.

Early experience in laparoscopic colectomy for refractory colitis in children.

There is limited literature endorsing the laparoscopic approach for the treatment of refractory colitis in children. We report our experiences of paediatric laparoscopic colectomies performed for ulcerative colitis. A retrospective review over a three year period was undertaken. Operative time, length of stay, post-operative analgesia, time to commencement of diet, and complications were recorded. Nine laparoscopic colectomies were performed. Median operative time was 320 minutes (range--240-475). Mean time to commencement of full diet was 3.9 days (range 2-8). Median length of stay was 6 days (range--5-16). In our experience, laparoscopic colectomy in children is a feasible and superior method to open colectomy and in our opinion, facilitates further restorative procedures and will become the default method of treatment in the near future.

The incidence of symptomatic malrotation post gastroschisis repair.

UNLABELLED: Gastroschisis is known to be associated with abnormal bowel rotation. Currently, the broadly accepted practice is not to perform Ladd's procedure routinely at the time of closure of gastroschisis defects. However the incidence of symptomatic malrotation and volvulus post gastroschisis repair is unknown; this incidence is important in view of the current practice of bedside gastroschisis closure. This study examined the incidence of symptomatic malrotation and volvulus following gastroschisis repair. METHOD: Patients who had undergone gastroschisis repair between 1999 and 2009 in any of 2 tertiary centers were identified using the Hospital Inpatient Enquiry system. The medical records were reviewed to obtain demographic data and postoperative outcomes. Patients were contacted for follow-up. RESULTS: 128 patients were identified with a median postoperative follow-up of 4 years (range: 6 weeks to 12 years). Upper gastrointestinal (GI) contrast studies were performed in 30 patients (23.4%), 21 (16.4%) of whom showed evidence of malrotation. Malrotation was documented during the primary repair in 12 patients (9.4%); however Ladd's procedure was performed primarily in only 3 patients. 7 patients underwent Ladd's procedure in a second laparotomy for mechanical obstruction secondary to causes not related to malrotation and volvulus. A total of 29 patients (22.7%) had either operative or radiological evidence of malrotation. None of these patients developed volvulus after being followed for a median period of 4 years. CONCLUSION: Bedside gastroschisis closure without concomitant Ladd's procedure is a safe practice. None of the patients with documented malrotation developed volvulus post gastroschisis repair.

A 9-year single center experience with circumumbilical Ramstedt's pyloromyotomy.

INTRODUCTION: Pyloric stenosis is a common cause of vomiting in infancy and is usually treated with a Ramstedt's pyloromyotomy. In this study we retrospectively reviewed our experience with the circumumbilical incision for the treatment of pyloric stenosis with a particular emphasis on the relation between postoperative emesis and postoperative time to feeds. MATERIAL AND METHOD: The medical records of all patients undergoing pyloromyotomy for IHPS from January 2000 to December 2008 were reviewed retrospectively. Patient details were recorded and statistically analyzed using SPSS version 13. We reviewed our experience looking specifically at the postoperative time to initial feeds as a way of minimizing hospital stay. RESULTS: 513 patients' notes were available for the study. There were 440 males and 73 females (M:F ratio 6:1). Median age at operation was 40 days (2-194 days) and a positive family history was obtained in 11.9%. Median duration of symptoms was 10 days (range 1-60 days). There were 31 (6%) complications related to surgery. The average number of postoperative emesis episodes was 1.9. The median postoperative hospital stay was 2 days (1-60). The average time to feeding was 20 h (1-69). CONCLUSION: This is a large single-center retrospective study where, in the era of minimally invasive surgery, Ramstedt's pyloromyotomy via the circumumbilical approach has a low rate of complications and is a safe and feasible method to treat pyloric stenosis. The establishment of feeds soon after surgery minimizes the postoperative in-hospital stay.

Anaesthetic implications of laparoscopic splenectomy in patients with sickle cell anaemia.

With the increasing immigrant population in the Republic of Ireland, the number of patients with sickle cell disease (SCD) seen in the paediatric hospitals is climbing. In this case report, we review the anaesthetic implications and outcome of the first two paediatric patients with SCD to have a laparoscopic splenectomy due to repeated splenic infarcts in the Republic of Ireland.

Laparoscopic Heller's cardiomyotomy.

Achalasia is a rare motility disorder which causes failure of relaxation of the lower oesophageal sphincter (LES) and is thought to affect 0.31/100,000 children per year in Ireland. The classic presentation is difficulty swallowing and vomiting undigested food, and children can often present with chest pain. In some instances, these symptoms can lead to considerable weight loss. In this report, we present 2 cases of patients with achalasia who have also been the first 2 cases of laparoscopic Heller's cardiomyotomy performed in children in the Republic of Ireland.

Laparoscopic appendectomy after conservative management of appendix mass.

The purpose of this study was to demonstrate the safety and efficacy of laparoscopic appendicectomy following non-operative management of appendix mass in children. Medical records of 103 consecutive patients (61 males, 42 females) who underwent non-operative treatment of appendix mass followed by laparoscopic elective appendicectomy were examined. Their ages ranged from 2 years 5 months to 15 years (mean 8.3 years). All patients were treated conservatively by close observation, antibiotics and intravenous fluids. Once the child was fit for discharge laparoscopic elective appendicectomy was booked for 4-6 weeks later. Ninety-three children responded to the initial conservative treatment and were discharged after a mean hospital stay of 5.6 days (range 3-10 days). Ten (9.7%) did not respond to initial treatment and developed appendix abscess requiring drainage. Average hospital stay in the 97 patients who had laparoscopic elective appendicectomy was 2.5 days (range 1-5 days). Three patients developed complications after elective appendicectomy, a stitch abscess in one, paralytic ileus in one and wound infection in one. Histological examination revealed fibrosed or resolving appendicitis in 52, acute or subacute appendicitis in 24, carcinoid tumour in two and normal appendix in 19. Laparoscopic elective appendicectomy is a safe and effective method of treatment following conservative treatment of appendix mass. Not only does it make the dissection and resection of the appendix easier but it also has the added advantage of performing adhesiolysis.

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.

Notochord anomalies in the adriamycin rat model: A morphologic and molecular basis for the VACTERL association.

BACKGROUND/PURPOSE: The Adriamycin rat model (ARM) is a reliable model of the VACTERL association. The notochord is structurally abnormal in the region of the foregut, midgut, and hindgut in the ARM. The authors hypothesised that notochord anomalies allow ectopic expression of molecular signals in the developing embryo and thus lead to VACTERL malformations. This study was designed to investigate this hypothesis. METHODS: Adriamycin (1.75 mg/kg) was administered intraperitoneally to pregnant rats on days 7, 8, and 9 of gestation. Control animals were given saline. Embryos were recovered on gestational days 10.5 to 14 at (1/2)-day intervals and at full term. The first group of embryos were embedded in resin, and sagittal sections stained with Toluidine blue were studied for morphologic abnormalities. The second group of embryos were examined using in situ hybridization for the expression of Sonic Hedgehog (Shh), a patterning gene implicated in the etiology of the VACTERL association. RESULTS: Twenty-seven of the 28 (96.4%) full-term embryos showed VACTERL anomalies. Forty-five of the 50 (90%) experimental embryos (gestational days 10.5 to 14) showed notochord abnormalities. Abnormal ventral branches from the notochord toward the gut were a commonly observed abnormality. These were seen to impinge on the developing foregut, midgut, dorsal aorta, and kidney. In situ hybridization for Shh showed that these branches from the notochord expressed Shh in 66.6% of experimental embryos. This abnormal Shh expression was not seen in the control embryos. CONCLUSIONS: Adriamycin diffusely induces altered notochord morphology in the rat embryo. The abnormal notochord morphology may allow ectopic expression of Sonic Hedgehog, and, thus, contribute to the malformations found in the VACTERL association.

Cell death in the early adriamycin rat model.

The adriamycin rat model (ARM) exhibits many features of the VACTERL association. Adriamycin is a cytotoxic drug used in cancer chemotherapy. Although its exact mode of action is not clear, it is presumed to have a similar cytotoxic role in the developing embryo. Lysotracker red (LT) is a dye that stains phagolysosomes and apoptotic bodies and allows entire rodent embryos to be stained for apoptosis. We hypothesised that there was increased cell death in adriamycin-exposed embryos. To investigate this hypothesis, adriamycin (1.75 mg/kg) was given intraperitoneally to rats on days 7, 8, and 9 of pregnancy. A control group was given saline on the same schedule. Embryos were recovered at 3, 12, 24, and 48 h following the last dose and also at term (21 days) to confirm that the usual incidence of congenital anomalies found in the ARM was obtained in our animal model. Embryos were embedded in resin, sectioned, and studied by light microscopy. Embryos from the 3-h and 24-h groups were studied using LT and confocal microscopy to search for evidence of apoptosis. All term newborns (100%) from the adriamycin-treated group demonstrated the typical abnormalities found in the ARM, i.e., oesophageal atresia, multiple gastrointestinal atresias, vertebral malformations, absent tails, ureterohydronephrosis, etc. In the 9.5-day adriamycin group there was no difference in appearance between the experimental and control embryos. Specifically, no cellular debris or increased cell turnover indicative of adriamycin cytotoxicity was observed in the experimental group. At day 10.5, 90% of embryos from two separate litters had evidence of notochordal distortion and tethering to the gut or gut-tube abnormalities. These findings were not observed in the control embryos. Confocal microscopy and LT examination of the embryos from litters killed at 3 and 24 h following the last dose of adriamycin demonstrated no evidence of increased cell death in adriamycin-exposed embryos compared to control embryos. The absence of significant apoptosis in the developing embryos in the immediate period following administration of adriamycin suggests that the teratogenic effect of adriamycin is not caused by cell death.

Midgut atresias result from abnormal development of the notochord in an Adriamycin rat model.

BACKGROUND/PURPOSE: Prenatal exposure to Adriamycin in a rat model (ARM) has been reported to lead to a spectrum of tracheoesophageal and associated malformations of the gastrointestinal tract, including multiple intestinal atresias. An abnormal relationship of the notochord with the foregut has been implicated in the formation of esophageal atresias. The authors hypothesised that midgut atresias arise from abnormal notochord development in the region of the midgut. This study was designed to examine the gut-notochord relationship during early embryonic development. METHODS: Timed pregnant Wistar rats were given 1.75 mg/kg of Adriamycin intraperitoneally on days 7, 8, and 9 of gestation. Embryos were recovered at 12-hour intervals from days 9.5 to 14, and at term. A control group was given saline instead of Adriamycin. Embryos were embedded in resin or wax, sectioned, and studied using light microscopy, paying particular attention to the notochord and surrounding structures. RESULTS: The notochord appeared identical in controls and experimental embryos on day 9.5. However, on day 10.5 the notochord was diffusely abnormal in ARM, distorted, and tethered to foregut as well as midgut compared with controls. This abnormality was not seen in control embryos. On day 12 the notochord abnormalities were more exaggerated in the region of the midgut in ARM embryos. Full-term ARM animals had esophageal and multiple intestinal atresias. CONCLUSIONS: The notochord is abnormal in the region of the developing midgut, and this may account for the occurrence of atresias found in this region.

Conservative management of appendix mass in children.

BACKGROUND: The management of an appendix mass in children is controversial. An experience of conservative management of appendix masses in a paediatric population over the last 19 years is reviewed. METHODS: The medical records of all children who presented with an appendix mass to one of three children's hospitals between 1982 and 2000 were reviewed. In that interval 427 children with a mean age of 7.3 years (range from 2 months to 18 years) presented with an appendix mass. Sixteen children had an immediate appendicectomy. The remaining 411 children were treated conservatively by close observation and antibiotics, with intravenous fluids and nasogastric suction if required. Once the child was well enough for discharge home, elective appendicectomy was booked for 4-6 weeks later. RESULTS: Three hundred and forty-six (84.2 per cent) of the children responded to initial conservative management and were discharged after a median stay of 6 (range 3-24) days. The children returned 4-6 weeks later for elective appendicectomy. The complication rate for elective appendicectomy was 2.3 per cent (eight patients). Those who had an uncomplicated elective appendicectomy had a median hospital stay of 3 (range 1-6) days. Histological examination demonstrated acute or subacute inflammation in 50.8 per cent of appendices removed at elective appendicectomy. CONCLUSION: Non-operative management of an appendix mass followed by elective appendicectomy is a safe and effective method of management.

Intestinal neuronal dysplasia: results of treatment in 33 patients.

PURPOSE: Intestinal neuronal dysplasia (IND) is a disease of the enteric nervous system, which clinically resembles Hirschsprung's disease. The authors reviewed their experience of IND over an 8-year period. METHODS: Between 1992 and 1999, 418 patients underwent rectal suction biopsy for persistent constipation. Thirty-three (7.8%) patients had histologic evidence of IND. There were 26 boys and 7 girls (age range, 1 week to 10 years). The diagnosis of IND was based on the presence of hyperganglionosis of the submucous plexus and giant ganglia and at least one of the following features in rectal biopsies: (1) ectopic ganglia, (2) increased acetylcholinesterase (AChE) activity in the lamina propria, and (3) increased AChE nerve fibers around the submucosal blood vessels. All patients were started on laxatives with or without enemas after the diagnosis was made. Patients have been followed up from 1 to 8 years (mean, 2.4 years). RESULTS: Twenty-one (64%) patients had a good response to conservative management and currently have normal bowel habits. Twelve patients (36%) underwent internal sphincter myectomy after failed conservative management. Seven of these patients now have normal bowel habits. Two patients are able to stay clean with regular enemas. Three patients who continued to have persistent constipation after myectomy and underwent resection of redundant and dilated sigmoid colon now have normal bowel habits. CONCLUSION: The majority of patients with IND can be treated successfully with conservative treatment.

Using a process action team to improve patient education.

To develop an effective, coordinated, and integrated process for patient education in primary care, the Department of Veterans Affairs, Western New York Healthcare System, launched a process action team. The interdisciplinary team utilized a variety of methods, including flowcharts, cause-and-effect diagrams, and variance matrices. The team developed, implemented, and analyzed a data collection plan involving chart reviews, staff interviews, and patient-staff surveys. An improvement plan was initiated in collaboration with staff. The results highlight the importance of using a data collection plan to understand the causes of problems as well as the need for staff involvement.

Outbreak of botulism in horses.

An outbreak of nervous disease in Standardbred horses occurred near Bendigo, in south-eastern Australia, in October 1980. Over a two week period 11 horses in four training stables were affected with gait abnormalities, depression and recumbency. Eight of the 11 died. The results of an investigation implicated Clostridium botulinum toxin as the cause. The toxin was food-borne as a contaminant of oaten chaff.