Congenital mesenteric hernia in neonates: Still a dilemma.

Congenital transmesenteric hernia in neonates is a rare cause of intestinal obstruction with devastating outcomes and still remains a challenge to diagnose pre-operatively. Patients are often managed with emergency surgical exploration and may need bowel resection. We present 2 neonates with small bowel obstruction secondary to strangulated transmesenteric hernia through a congenital defect in the small bowel mesentery, which were managed successfully. We have also reviewed the literature about congenital transmesenteric hernia in neonates.

Visceral basidiobolomycosis: An overlooked infection in immunocompetent children.

Visceral basidiobolomycosis is an unusual fungal infection of viscera caused by saprophyte Basidiobolus ranarum. It is very rare in healthy children and poses a diagnostic challenge due to the non-specific clinical presentation and the absence of predisposing factors. We report a case of gastrointestinal basidiobolomycosis in a 4-year-old healthy girl who presented with a short history of abdominal pain, bleeding per rectum, fever, and weight loss. The diagnosis was based on high eosinophilic count, classical histopathology findings of fungal hyphae (the Splendore-Hoeppli phenomenon), and positive fungal culture from a tissue biopsy. Fungal infection was successfully eradicated with a combined approach of surgical resection of the infected tissue and a well-monitored course of antifungal therapy. The atypical clinical presentation, diagnostic techniques, and the role of surgery in the management of a rare and lethal fungal disease in an immunocompetent child are discussed.

Laparoendoscopic removal of peroral extrusion of a ventriculoperitoneal shunt.

INTRODUCTION: Ventriculoperitoneal shunts for hydrocephalic children are known to cause serious complications such as GI perforation. Peroral extrusion of the peritoneal part of a ventriculoperitoneal shunt is very rare, and management could be a challenge. MATERIALS AND SURGICAL TECHNIQUE: An 11-year-old girl presented with peroral extrusion of the distal end of a ventriculoperitoneal shunt tube. Endoscopy and imaging studies showed that the peritoneal end had perforated the stomach and then extruded from the mouth. We used a surgical technique that combined endoscopy and laparoscopy to manage this rare complication. DISCUSSION: Peroral extrusion of a ventriculoperitoneal shunt tube occurs secondary to perforation of the upper GI tract. Managing this condition involves removing the shunt tube, attention to the perforated viscus and associated infection. A combination of endoscopy and laparoscopy provided superior views, enabled identification of the site and size of the perforated viscus, and facilitated the uneventful removal of the tube.

Noncommunicating multiple intra-abdominal enteric duplication cysts.

A very rare case of noncommunicating multiple intra and retroperitoneal enteric duplication cysts (EDCs) is reported and discussed. Two large noncommunicating EDCs, one within the mesentery of proximal jejunum causing complete luminal obstruction and other isolated cyst in retroperitoneal area displacing duodenum and extrahepatic biliary system, were resected successfully in a 2-day-old neonate along with correction of malrotation.

Troubling toys: rare-Earth magnet ingestion in children causing bowel perforations.

Ingestion of foreign bodies in the pediatric population is common and magnet ingestion is known to cause a significant morbidity. Rare-earth magnets are small 3-6 mm diameter spherical powerful magnets that are sold as popular desk toys for adults and were previously found in construction toys in attractive colors for children to play with. We describe 2 young healthy children who ingested rare-earth magnets Buckyballs while playing with these magnetic toys and later presented in emergency with acute abdomen. Abdominal imaging revealed several (26 and 5) pieces of rare-earth magnets in the bowel loops. Emergency surgical exploration revealed multiple gastrointestinal perforations and fistula formation at sites of bowel entrapment in between strong magnets apposed to one another. We highlight the potential dangers of rare-earth magnets in children and suggest increasing public awareness about risks involved in rare-earth magnets ingestion by children to overcome this serious public health issue.

A Triad of Congenital Diaphragmatic Hernia, Meckel's Diverticulum, and Heterotopic Pancreas.

Congenital diaphragmatic hernia is a common developmental anomaly encountered by paediatric surgeons. It is known to be associated with extradiaphragmatic malformations, which include cardiac, renal, genital, and chromosomal abnormalities. Herein, we report a newborn born with concurrent congenital diaphragmatic hernia, Meckel's diverticulum, and heterotopic pancreatic tissue. This is the first case report of such a triad with description of possible mechanisms of the development.

Complicated Amyand's Hernia in a Neonate.

Amyand's hernia is a rare clinical entity in which the vermiform appendix is present within the inguinal hernia sac. Here, we report a 5-day-old neonate with dysmorphic features referred to us with a tender irreducible right inguino-scrotal swelling. Surgical exploration showed gangrenous appendix with a peri-appendicular abscess in the inguinal hernia sac. Appendectomy and right herniotomy was performed.

Infected hydrocele of the canal of nuck.

Hydrocele of the canal of Nuck in children is rare. It may present as incarcerated inguinal hernia and necessitates emergency exploration. Risk of infection in hydrocele of the canal of nuck is very rare. We present a case report of a 5-year-old girl who presented with a left tender inguinolabial region swelling with fever, tachycardia, and mild dehydration. The clinical features were suggestive of strangulated left inguinal hernia and further imaging and surgical exploration revealed it to be an infected hydrocele of the canal of Nuck. High ligation and hydrocelectomy were performed. Hydrocele of the canal of Nuck in a female child presenting with an inguinal swelling should be considered in differential diagnosis.

Congenital duodenal obstruction associated with Down's syndrome presenting with hematemesis.

Congenital duodenal obstruction is usually characterized by onset of early vomiting due to high bowel obstruction. Presentation of congenital duodenal obstruction with acute gastrointestinal bleeding is very uncommon. We present an unusual case of congenital intrinsic duodenal obstruction associated with the Down's syndrome presenting with hematemesis secondary to duodenitis. This is a rare presentation of congenital duodenal obstruction associated with Down's syndrome in addition to seven cases previously reported in the English literature.

Rising incidence of gastroschisis and exomphalos in New Zealand.

BACKGROUND: An apparent increase in the incidence of gastroschisis and exomphalos has been reported from several parts of the world. The exact mechanism of this trend is unknown. The aim of this study was to determine the regional and national trends in the incidence of gastroschisis and exomphalos in New Zealand. MATERIAL AND METHODS: This retrospective multicenter study involved collection of data from all 4 tertiary care pediatric surgical centers in New Zealand. The incidence was calculated per 10,000 live births. Data were analyzed to determine the regional and national trends. The statistical analysis was done using linear regression model and Poisson distribution. RESULTS: The incidence of gastroschisis has increased from 2.96 per 10,000 live births to 5.16 per 10,000 live births between 1996 and 2004. During the same period, the incidence of exomphalos has increased from 0.69 per 10,000 live births to 3.27 per 10,000 live births. Gastroschisis was observed more in younger mothers, whereas exomphalos was associated with older mothers. CONCLUSION: The incidence of gastroschisis and exomphalos is increasing in New Zealand, which is consistent with worldwide trends in showing the increasing incidence of anterior abdominal wall defects.

Surgical closure of patent ductus arteriosus in preterm low birth weight infants.

BACKGROUND AND AIM OF THE STUDY: In many preterm low-birth-weight infants, ductus arteriosus fails to close spontaneously. This study evaluates the results of surgical ligation of symptomatic patent ductus arteriosus (PDA) in preterm low birth weight infants. METHODS: We reviewed the medical records of all infants undergoing surgical closure of PDA from January 1987 to December 2005. Demographic data, age and weight at operation, surgical technique to close PDA and outcome were analyzed. RESULTS: One hundred and forty-five infants underwent surgical closure of PDA in which either indomethacin treatment had failed or was contraindicated. The mean gestational age was 25.5 +/- 2.3 (range 24-36 weeks) and the mean birth weight was 837.7 +/- 277.2 g (450-1000 g). The average age and weight at operation were 14.1 (+/-1.8) days and 881.7 g (+/-338.1), respectively. PDA was surgically closed by left thoracotomy using either nonabsorbable suture (73%) or hemoclips (27%). Postoperative complications occurred in 10 patients, which included intraoperative bleeding (6), pneumothorax (1), left vocal cord paralysis (1), lymphatic leak (1), and injury to left phrenic nerve (1). There was no mortality related to surgical closure of PDA. CONCLUSION: We conclude that surgical closure of hemodynamically significant PDA is safe and effective in preterm low birth weight infants when pharmacological treatment is ineffective or contraindicated. The associated morbidity is minimal and no surgery-related mortality was observed.

The contribution of the sonic hedgehog cascade in the development of the enteric nervous system in fetal rats with anorectal malformations.

OBJECTIVE: This study was designed to determine the expression of Sonic hedgehog (Shh) and its downstream genes during development of the enteric nervous system (ENS) in ethylenethiourea (ETU)-exposed fetal rats with anorectal malformations (ARMs). MATERIAL AND METHODS: Anorectal malformations were induced by 1% ETU (125 mg/kg) given on gestational day 10, and the litter was harvested at term. The fetal anorectum and rectosigmoid region, including any communication with the urinary tract, were collected for gene expression studies and immunofluorescence study of the ENS. Gene expression of Shh cascade was performed using reverse transcription and real-time polymerase chain reaction (PCR). The myenteric plexuses of the ENS in normal and ARM rats were visualized with fluorescent antibodies. RESULTS: Reverse transcription-PCR confirmed expression of Shh and its target genes in all parts of the ARMs. Quantitative PCR demonstrated that levels of expression of the genes of the Shh cascade were low in the ARMs. The immunoreactivity of neuromarkers was markedly reduced in high ARMs and slightly reduced in low ARMs. CONCLUSION: This study demonstrates reduced expression of Shh and its target genes in ARMs in ETU-exposed fetal rats. Neurons in the myenteric plexus were decreased in high and low types of ARMs. Our results support a role for the Shh cascade during development of the ENS during hindgut development.

Comparative study between window and conventional enterostomies in preterm neonates with small bowel perforations.

OBJECTIVES: Small bowel perforations in the neonatal period can be secondary to necrotizing enterocolitis (NEC), ischemic necrosis, or occlusive anomalies of the small bowel; furthermore, they may be of no discernible cause. Depending on the clinical condition of the infant and the extent of the disease, a number of surgical options are available; one of which is exteriorization. To reduce the morbidity of stoma among patients, we adopted a technique called window enterostomy (WEnt). The objectives of this study were to describe the technique and to compare WEnt with conventional enterostomy (CEnt) in preterm infants undergoing surgery for focal NEC or isolated small bowel perforation (ISBP). METHODS: We reviewed all cases of NEC and ISBP between January 1996 and March 2006 from our institution. Patients with focal NEC or ISBP who required a surgical intervention were included and categorized into the WEnt and the CEnt groups. We collected multiple data as study variables: demographics; site of perforation; operative time; need for a second operation; postoperative morbidity; duration of total parenteral nutrition; and postoperative weight gain. RESULTS: Twenty-four neonates met the criteria for study inclusion. Of these, 14 underwent CEnt and 10 underwent WEnt. The median gestational age and birth weight of the neonates were 25.4 weeks (SD = 1.4) and 814.4 g (SD = 195.1), respectively. In comparing the 2 groups, we found statistically significant differences in the operative time for the primary and secondary procedures, duration of total parenteral nutrition, time to full oral feeding, and weekly postoperative weight gain. The rate of postoperative complications was higher among the infants from the CEnt group. CONCLUSION: Our results suggest that WEnt is a quick and workable technique with minimal morbidity for preterm neonates with focal NEC or ISBP.

Congenital hernias of the diaphragm in children.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a major congenital malformation. Different types have been described. Bochdalek hernia (BH) remains most prevalent with high mortality rates. Other variants are less common and carry good prognosis. Although, the diagnosis can be made antenatally, the presentation may be delayed. There is paucity of national literature on CDHs. We present our experience with these challenging paediatric malformations. METHODS: Medical records of 18 patients (< or =14 years) treated by the group of authors between October 1998 and April 2002 were retrospectively reviewed and demographic data, clinical presentation, morbidity and outcome were studied. RESULTS: There were 13 (72%) children with Bochdalek hernia, 2 (11%) with eventration of the diaphragm, 2 (11%) with hiatus hernia and 1(6%) with a Morgagni hernia. The lesions were more common in girls and all the defects were left sided. The average age at the time of presentation of BH was 23 hours (2 to 72 hours) commonly presenting with cyanosis and respiratory distress. Associated anomalies were documented in 10 (77%) cases; six had multiple malformations and four died before surgery. In other types of CDHs, the mean age at presentation was 39 (18-60) months and they mostly presented with recurrent respiratory tract infections and/or mild gastrointestinal symptoms. Diagnosis was made on history, physical examination, plain x-ray chest, and gastrointestinal contrast study, when required. 14 (78%) children were operated and a sac was present in 5. Post-operative complications occurred in 5 (55%) patients with Bochdalek hernia, which were managed conservatively. The overall survival rate was 67% (n=12). CONCLUSION: The different types of CDHs presented from neonatal age to later childhood with distinct symptoms. Surgery was safe and effective. Higher morbidity and mortality was observed in newborns with Bochdalek hernia.

Sonic hedgehog, BMP4, and Hox genes in the development of anorectal malformations in Ethylenethiourea-exposed fetal rats.

BACKGROUND: shh signaling pathway has been shown to be involved in the morphogenesis of many organ systems. In this study, we investigated the expression of shh and its targets, BMP4 and Hox genes, in the development of anorectal malformations in Ethylenethiourea (ETU)-exposed embryos. METHODS: We used ETU murine model of the vertebral, anal, cardiac, tracheoesophageal, renal, and limb association. Ethylenethiourea 1% (125 mg/kg) was given to the pregnant females via gavage feeding on gestational day (gD) 10 and saline to control animals. Embryos were collected at gD12 to gD16 and gD21; hindguts were dissected and snap frozen. Highly purified RNA was isolated, and expression of shh, BMP4, Hoxa13, and Hoxd13 genes was confirmed with RT-PCR. Relative quantitative expression of shh and target genes at each time point was done with SYBR Green I qPCR. Normalized gene of interest expression was calculated by geNorm, and data analysis was done with 2-tail Student t test. RESULTS: shh, BMP4, Hoxa13, and Hoxd13 transcripts were detected in all samples, confirming that shh cascade is active during the process of hindgut development in fetal rats. Relative quantitation demonstrated that shh cascade expression shows time-dependent changes in the developing hindgut. CONCLUSION: This study shows that ETU disturbs the expression of shh signaling pathway during the development of hindgut. We provide evidence that shh plays a pivotal role in the hindgut morphogenesis, and its misexpression affect the expression of targets, BMP4 and Hox genes.

Counterstaining improves visualization of the myenteric plexus in immunolabelled whole-mount preparations.

Immunocytochemistry has emerged as a powerful research tool in neurobiology. One of the widely used methods is an indirect fluorescence technique that uses FITC- conjugated IgG to visualise protein expression within tissues, but a major drawback of this technique is the high background fluorescence due to non-specific antibody binding. Gut innervation is complex and best visualized in three-dimensions in whole mount preparations. We describe a simple and easy to use counterstaining procedure in conjunction with an indirect immunofluorescence technique in gut whole mount preparations that largely eliminates background fluorescence and creates a contrasting background against the bright antigen-antibody complexes. Furthermore, this technique allows the detailed qualitative and quantitative study of myenteric plexuses in whole-mount preparations.

Comparison of suture ligation and clip application for the treatment of patent ductus arteriosus in preterm neonates.

OBJECTIVE: We reviewed the experience of 2 centers performing surgical ligation of patent ductus arteriosus in preterm neonates to identify whether the choice of surgical technique--suture ligation or clip application--affected outcome. METHODS: Between 2000 and 2005, 67 newborn infants had open surgical closure of patent ductus arteriosus: 33 by suture ligation and 34 by clip application. The groups were similar in age and sex. RESULTS: The average length of the procedure was 55.8 +/- 13.7 minutes for suture ligation and 30.8 +/- 8.7 minutes for clip application (P < .05). Six neonates had intraoperative bleeding in the suture ligation group. Four patients had significant postoperative complications in the suture ligation group, compared with 2 in the clip application group. CONCLUSIONS: This study demonstrates that clip application results in a significant reduction in the operative time and, possibly, in less morbidity.

Sonic hedgehog expression in the development of hindgut in ETU-exposed fetal rats.

Sonic hedgehog (Shh) has been shown to be involved in the morphogenesis of many organ systems including the notochord, floor plate and limbs, as well as in the development of the left-right axis in vertebrates. Recent evidence suggests the Shh cascade plays a crucial role in the development of the foregut and hindgut. We have previously shown that prenatal exposure of fetal rats to ethylenethiourea (ETU) induces hindgut malformations and other abnormalities of the VACTERL association. The aim of this study was to determine the pattern of expression of Shh and its downstream genes during hindgut development in ETU-exposed embryos with anorectal malformations (ARMs). Pregnant Sprague-Dawley rats were mated together overnight and a positive vaginal plug was marked as gD0. On gD10, 1% ETU (125 mg/kg) was given to the experimental group and controls received the same volume of saline. Embryos were collected from both groups at gD12-16. The developing hindgut of each embryo was dissected under magnification and snap frozen. Highly purified RNA was isolated from each hindgut and first strand cDNA was prepared with appropriate negative controls. Reverse transcriptase (RT) polymerase chain reaction (PCR) was done to determine the transcripts of Shh in each sample and quantitative real-time PCR was carried out to show relative quantitative expression of Shh at each time point. Shh was detected in all samples confirming that Shh is active during the process of hindgut development in fetal rats. Relative quantitation demonstrated that Shh expression shows time-dependent changes in the developing hindgut of ETU-exposed rat embryos, and when results were compared with control samples, there was significant decrease in expression on gD14 and 15, when the cloaca normally separates into the rectum and urethra occurs in the rat fetus. The misregulated expression of Shh in the hindgut of ETU-exposed rat embryos suggests that ETU may interfere with Shh signalling. Downregulation at the time of cloacal separation into rectum and urethra indicates that Shh plays a crucial role in the development of hindgut.