Idiopathic megacolon and megarectum in an adult treated with laparoscopic modified Duhamel procedure

Idiopathic megacolon (IMC) and idiopathic megarectum (IMR) describe an abnormality of the colon or rectum, characterised by a permanent dilatation of the bowel diameter in the absence of an identifiable cause. We present a 23-year-old woman with chronic constipation and excessive straining during defecation who presented at the emergency department in partial gut obstruction with a palpable fecaloma. Manual faecal disimpaction and a sigmoid loop colostomy was initially done. A full thickness rectal biopsy was positive for ganglion cells. Further workup led to the diagnosis of chronic IMC and IMR. The patient underwent laparoscopic modified Duhamel procedure, with an uneventful postoperative course.

Muscle splitting sigmoid loop colostomy: a viable option to prevent colostomy prolapse

Background: The purpose the study was to assess the incidence of Colostomy prolapse with sigmoid loop colostomies performed through rectus muscle splitting Incision.Methods: Colostomy prolapse may present serious problems for patient care and stoma function6. Sigmoid loop colostomy was done for babies with High anorectal malformation (HARM) and Hirschsprung’s disease (HD) from Jan2012 to Dec2014 in two referral hospitals. All colostomies were sigmoid loop colostomies and done by a single surgeon through rectus muscle splitting incision. All patients were followed up for colostomy prolapse for at least 6 months.Results: A total of 110 babies managed with colostomy during the study period. The patients comprised of 68 males and 42 females. High anorectal malformation accounted for 66 babies [males 48, females18] while Hirschsprung’s disease was the surgical indication in 44 [males20, females24]. The age at colostomy ranged from 1day to 4 years. A total of 32 complications were recorded in 25patients [29%]. There were 4 deaths [3.6%]. Early complications like Haemorrhage and septicaemia was seen 4 patients. Colostomy stenosis was observed in 3 cases requiring only dilatation. Colostomy prolapse was seen only in 3 patients [3.24%]. Skin excoriation was the commonest complication, noted in 10 patients. Failure to thrive secondary to colostomy diarrhoea was observed in 3 patients. Superficial wound dehiscence occurred in 3 babies. Urinary tract infection seen in 5 and Intestinal obstruction requiring laparotomy occurred in one baby.Conclusions: Colostomy prolapse is very common after loop colostomies. Rectus muscle splitting incision for loop colostomies is safe and is associated with low incidence of colostomy prolapse.

Postoperative Megarectum in an Adult Patient with Imperforate Anus and Rectourethral Fistula

This report presents a surgical case of postoperative megarectum in an adult patient with imperforate anus/anorectal malformations. A 71-year-old Japanese male presented with a mass in the lower abdomen which was 15 × 12 × 8 cm in diameter, edema in the right lower extremity, and frequent urination. He had undergone sigmoid loop colostomy for an imperforate anus as a newborn infant. At 28 years of age, the sigmoid loop colostomy was changed to sigmoid divided colostomy in the left lower abdomen. Computed tomography revealed a large cystic mass in the lower abdomen. Retrograde urethrography indicated a rectourethral fistula and megarectum with stones. A small laparotomy incision was created in the right lower abdomen, and the wall of the megarectum was identified. Approximately 2,300 mL of gray muddy fluid was identified and drained. A mucous fistula of the upper rectum was created in the right lower abdomen. This is an extremely rare case of postoperative megarectum in an adult patient with an imperforate anus and rectourethral fistula.

Variants of Currarino Syndrome: Embryological Association and Review of Pertinent Literature

Currarino syndrome is a triad of sacral defect, anorectal malformation and a presacral mass. The diagnosis is usually made late in childhood and about 50% of cases are familial with autosomal dominant inheritance. We present two neonates (one with vestibular fistula, and another with cloacal malformation) with the features consistent with Currarino syndrome, but with Altman’s type II sacrococcygeal teratoma, that is, presacral mass having an external sacrococcygeal component also. We believe that this triad should be considered a variant of Currarino syndrome. In first case, excision of the mass along with coccyx, followed by primary Posterior Sagittal AnoRectoPlasty was performed in the same setting. The patient succumbed to death due to septicemia as a result of wound sepsis. Learning from the previous experience, we decided to do a diverting sigmoid loop colostomy followed by posterior sagittal excision of the mass along with coccyx, in same sitting in the second case. There was no recurrence. Though HLXB9 has been identified as the major causative gene in Currarino syndrome, exact pathogenesis is still unclear. We herein highlight the significance of this variant of Currarino syndrome and propose a theory on the basis of an embryological association between the malformation complex.