Diagnostic suggestion and surgical consideration for Hirschsprung’s disease associated with high anorectal malformation

Object: The objective of this study were to highlight the finding of vasa recta (tortuous) on the colonic wall as a diagnostic clue for suspecting an associated Hirschsprung’s disease (HD) and to draw attention to the importance of preserving the aganglionic rectum plus a retrorectal pull through in these cases.Background: The association of HD with anorectal malformation (ARM) is both diagnostically and surgically challenging.Patients and methods: Records of cases with ARM treated over 15 years were examined. Among these, five children with an associated HD were analyzed with regard to their clinical, radiological, surgical, histopathological findings, and outcome. Relevant literature was also reviewed.Results: HD was present in 1.26% cases of ARM. All were men with high-type ARM. Two groups were identified. In group 1 (two patients), associated HD was suspected after completion of all stages of ARM repair. They reported prolonged postoperative constipation, abdominal distention, and enterocolitis. Moreover, they endured additional surgeries for HD. In group 2 (three patients), HD was suspected at the time of initial colostomy for ARM in two patients on visualizing tortuous (cork screw) vasa recta on the sigmoid colon surface in a region similar to the transition zone in HD. Biopsy from the site confirmed HD. In the third patient, these vessels were visualized on the colostomy loop at the time of laparoscopic pull through for ARM. Preservation of the aganglionic rectum (to function as a fecal reservoir) as well as a retrorectal pull through was done in both groups.Conclusion: Finding prominent corkscrew vessels on the colonic surface could serve as a clinical clue for the presence of HD in cases of ARM. Moreover, preserving the aganglionic rectum and performing a Duhamel pull through helps provide acceptable continence.Keywords: anorectal malformation, Hirschsprung’s disease, rectal reservoir, tortuous vasa rect

Four-country surveillance of intestinal intussusception and diarrhoea in children

Aim: Establishment of baseline epidemiology of intussusception in developing countries has become a necessity with the possibility of reintroduction of rotavirus vaccine. The current study assessed the seasonal trend in cases admitted with intussusceptions and dehydrating acute watery diarrhoea in children aged 2 months to 10 years. Methods: In a prospective surveillance study, teaching and research hospital sites in India (Lucknow and Nagpur), Brazil (Fortazela), Egypt (Ismailia) and Kenya (Nairobi) established a surveillance where a network of hospitals with surgical facilities catered to a reference population of about 1-2 million for reporting of intussusception. One large hospital per site also recruited admitted cases of acute watery diarrhoea. Results: From April 2004 to March 2006, 173 and 2346 cases of intussusception and diarrhoea, respectively, were recruited. Cases of intussusception had no apparent seasonality. Most cases of intussusception (61.3%) (107/173) were in the ≤1 year age group, with males comprising 68.8% (119/173) of all cases. Hospital mortality of intussusception was 4.2% (4/96). Cases of diarrhoea peaked in March, with 56.6% (1328/2346) of admitted cases being males. Majority (83.1%) of cases of diarrhoea had received antibiotics, and the hospital mortality was 0.8% (18/2280). Conclusion: Intussusception in the four participating countries exhibited no seasonal trend. We found that it is feasible to establish a surveillance network for intussusception in developing countries. Future efforts must define population base before the introduction of rotavirus vaccine and continue for some years thereafter. © 2009 Paediatrics and Child Health Division (Royal Australasian College of Physicians)

Isolated cleft sternum: Neonatal surgical treatment

Isolated cleft sternum is a rare congenital defect of the anterior chest wall and is the result of failed ventral midline fusion of sternal bands. We present two cases operated 18 and 24 days after birth with satisfactory results. Surgery is indicated to protect the heart and mediastinal contents

Case Report - Isolated cleft sternum: Neonatal surgical treatment

Isolated cleft sternum is a rare congenital defect of the anterior chest wall and is the result of failed ventral midline fusion of sternal bands. We present two cases operated 18 and 24 days after birth with satisfactory results. Surgery is indicated to protect the heart and mediastinal contents

Bladder exstrophy: Comparison of anatomical bladder neck repair with innervation preserving sphincteroplasty versus Young-Dees-Leadbetter bladder neck reconstruction

Aim: To evaluate the outcome of innervation preserving sphincteroplasty along with anatomical bladder neck reconstruction (IPS-ABNR) compared to classic Young-Dees-Leadbetter (YDL) bladder neck reconstruction in exstrophy with insufficient bladder capacity requiring detubularized-ileocystoplasty. Materials and Methods: Sixteen male patients of exstrophy bladder who required ileocystoplasty from 2004 to 2010 were randomized into group A (n = 7) and group B (n = 9). After detubularized-ileocystoplasty with Mitrofanoff stoma and ureteric reimplantation in all, group A received YDL bladder neck repair while group B received IPS-ABNR repair through a midline scrotoperineal approach. Outcome measurement included operative and postoperative problems, continence, and upper tract status. Results: In group A, two had incompetent bladder neck with gross incontinence, while four had a dry interval of more than 3 h without the ability of voiding per urethra. In group B, seven patients had dry interval of more than 3 h with an ability of urethral voiding and midstream holding in five. Conclusions: Exstrophy patients requiring augmentation cystoplasty and repaired with IPS-ABNR can achieve dynamic bladder outlet resistance with adequate leak point pressure and ability to void voluntarily with midstream holding capability. The children had the satisfaction of voiding per urethra with ability to stop in midstream similar to that in normal children

Ectopic salivary gland cyst in the neck: Association with congenital plunging ranula

Congenital ranula is rare. We report a large, symptomatic, congenital plunging ranula associated with a salivary gland cyst in the neck. To the best of our knowledge, this is the first such reported case. Even though both the cysts had their origin from the sublingual gland, only the cervical cyst had a capsular covering. Herniation of a part of the immature sublingual gland anlage through a congenital mylohyoid defect, its separation, and subsequent maturation could explain this occurrence

Six-year retrospective analysis of colonic perforation in neonates and infants: Single centre experience

Background: Developing countries at tertiary referral centre. This study analysed the aetiology of colonic perforation (CP) in neonates and infants. Materials and Methods: Retrospective analyses of 60 CP cases (presented from May 2005 to May 2011) were done. Results: The mean age at presentation was 8.33 ± 0.11 days (range, 2-110 days). The aetiology were Hirschsprung′s disease (HD), necrotising enterocolitis (NEC) and idiopathic perforation in 78.33% (47/60), 6.67% (4/60) and 15% (9/60), respectively. There were 210 patients with histopathologically proven HD; 22.38% (47/210) cases of HD had CP. Most common site of perforation was mid-transverse colon (74%, 35/47) in HD patients. All HD-associated mid-transverse colonic, caecal, appendicular and ascending colon perforations (except one caecal perforation) had aganglionic recto-sigmoid region and ganglionic perforation site. Features of enterocolitis were not found in any HD patients. Two patients (3.33%) died due to sepsis. Conclusions: There was a high rate of primary HD-associated colonic perforation in this study. Colonic perforation may the initial presenting condition in HD disease. We advocate colonic biopsy to rule out HD in any neonate presenting with primary colonic perforation

Pediatric intestinal leiomyosarcoma

The paper reports an infant presenting with leiomyosarcoma of the small intestine. The patient presented with intermittent abdominal pain. Examination revealed a hard and mobile intraperitoneal mass. The tumor arose from the mid-ileum with regional lymphadenopathy. Excision of the tumor along with the involved bowel was performed followed by three cycles of chemotherapy. Histological diagnosis was that of a low-grade malignant leiomyosarcoma of the small intestine. Surgical excision was followed by three cycles of chemotherapy. After surgery and three cycles of chemotherapy, the patient was followed up for four years with no evidence of recurrence or metastasis. Surgery followed by chemotherapy was curative for leiomyosarcoma in our patient. Intestinal leiomyosarcoma should be kept as a differential diagnosis for mobile solid intraabdominal tumors in childhood