|Year : 2013 | Volume
| Issue : 1 | Page : 29-31
Sigmoid volvulus in a young patient: Beware of Hirschprung's disease
Chandan R Choudhury1, Dipak Ghosh2, Sarabarni Biswas1
1 Department of General Surgery, Medical College, Kolkata, India
2 Department of Paediatric Surgery, Medical College, Kolkata, India
|Date of Web Publication||13-Sep-2013|
Chandan R Choudhury
Swapna Villa (3rd Floor), 23 Avenue First Road, Santoshpur, Kolkata - 700 075
Source of Support: None, Conflict of Interest: None
Sigmoid volvulus is a rare and potentially life-threatening complication of Hirschsprung' disease. A young patient presenting with sigmoid volvulus is not common and Hirschsprung's disease should be suspected in patients in this age group presenting with otherwise unexplained colonic volvulus. Here we report a rare and interesting case of Hirschsprung's disease presenting as sigmoid volvulus in a 15-year-old girl and the sufferings of the patient due to this uncommon entity.
Keywords: Aganglionosis, volvulus, young
|How to cite this article:|
Choudhury CR, Ghosh D, Biswas S. Sigmoid volvulus in a young patient: Beware of Hirschprung's disease. Saudi Surg J 2013;1:29-31
| Introduction|| |
Sigmoid volvulus is a rare and potentially life-threatening complication of Hirschsprung's Disease.  While sigmoid volvulus is commonly seen in older patients, it is rarely encountered in children and younger adults. Among the pediatric and adult cases of colonic volvulus reported, 23 of the affected individuals have also been diagnosed with Hirschsprung disease (HD).  The volvulus usually involves the sigmoid colon, and less commonly, the transverse colon or caecum. The cause is thought to be related to torsion of an enlarged colonic segment filled with meconium or faeces. Excessive mobility of the sigmoid mesentery may also have a role.  Despite advances in the diagnosis of Hirschsprung's Disease, occasionally, there is still a delay in presentation.  Mostly such patients have typical historical features, such as failure to pass meconium (in the neonate) or chronic constipation (in older children), suggesting the underlying disease. But often such relevant history in the neonatal and childhood period is either missed or neglected and patient is inappropriately treated.
Although uncommon, adult Hirschsprung's disease is a cause of chronic constipation and can present acutely with a sigmoid volvulus. Mortality in cases with sigmoid volvulus is greater than in cases without (15.4% vs. 0%).  Here we report a rare and interesting case of Hirschsprung's disease presenting as sigmoid volvulus in a 15-year-old girl and the sufferings of the patient due to this uncommon entity.
| Case History|| |
A 15-year-old girl was referred to us with a functioning colostomy (operated in district hospital) with an intention of colostomy closure. Her abdominal examination revealed multiple scar of three surgeries and a end colostomy in left iliac fossa [Figure 1]. Her past operative history was quite interesting. It revealed that was she was admitted in the emergency ward of a district hospital with chief complaints of obstipation and huge distension of abdomen with diagnosis of acute intestinal obstruction. Exploratory laparotomy was performed on the same day that confirmed sigmoid volvulus without any gangrene. De-rotation and release of volvulus with excision of dilated redundant sigmoid loop was done along with proximal colostomy and closure of distal end. Post operative period was uneventful, colostomy was functioning normally and the patient was discharged.
|Figure 1: Preoperative view showing colostomy and multiple scars of previous surgery|
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She was followed up regularly and colostomy closure was planned for re-establishment of colorectal continuity. After 3 months, patency of the distal stump was confirmed by pushing contrast through anal opening and a second laparotomy was performed and anastomosis of colon with distal stump was performed.
Patient started passing liquid faecal matter through anus from fifth postoperative day and discharged after 10 days. At 2 weeks of follow-up, patient developed mild faecal leak with progressively increasing constipation and distension of abdomen.
After the patient did not improve, colonoscopy was attempted for suspected anastomotic narrowing and patient landed up with inadvertent colonic perforation without any conclusive diagnosis. Thereby patient underwent third laparotomy and a Hartmann's procedure was performed and the girl recovered with a good functioning stoma at proximal sigmoid colon [Figure 1]. Thus, once again, normal bowel motion was established through colostomy and the patient was referred to us for closure of colostomy.
All these facts raised a high degree of suspicion and before undertaking any further surgery, a definitive diagnosis was sought for. Further, detailed enquiry about her past history revealed that she developed hugely distended abdomen soon after her birth and passed meconium for the first time after 4 days, which was managed conservatively in local hospital. Frequently she needed rectal stimulation (betel leaf stalk, suppository etc.) for evacuation although mild distension of abdomen was persistent.
Later in childhood, she was a frequent user of enemas and laxatives because of habitual constipation although the evacuation was always incomplete and dissatisfying. Another important fact observed by us was that the patient always had normal bowel function with a proximal colostomy but was developing constipation when the bowel continuity was being established.
Hence, on the basis of history and observation, a full-thickness rectal biopsy was taken. On histopathological examination, no ganglion cell was found and there was hypertrophy of nerve fibres, thereby establishing it to be a case of Hirschsprung's disease. Therefore, for definitive management, Duhamel's procedure (abdomino-anal pull-through) was done [Figure 2] and [Figure 3]. Patient started passing stool per anus after fifth postoperative day and subsequently discharged. Further follow-up up to 1 year revealed no more complaints and patient is passing normal bowels presently with complete evacuation.
|Figure 3: Coloanal anastomosis in progress with catheter and ureteric stent|
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| Discussion|| |
Hirschsprung's disease was first described by Harald Hirschsprung in 1886. Though adult onset HD was thought to be rare, Hirschsprung himself had suggested as early as 1900, that the disease was not confined to children and several cases of adult HD have been reported since then.  About 5% of patients with less severe disease may not be diagnosed until early adulthood, most of them between the second and third decade of life, and are frequently misdiagnosed as chronic constipation; these may not be truly diagnosed until an acute presentation with complications such as obstructive colitis, sigmoid volvulus, or subacute obstructions. 
The definitive treatment for sigmoid volvulus is grossly different from that of HD (Hirschprung's disease) and failure to recognize HD as the primary cause in a patient presenting with sigmoid volvulus would result in inappropriate and inadequate treatment. Hence, it is of paramount importance that whenever a young adult presents with sigmoid volvulus, rectal biopsy must be done to rule out HD. There are also reports of inappropriate treatment of this condition and patient death. 
Consequently, heightened awareness of this entity is required to avoid a delay in diagnosis.  Hence, unnecessary and multiple interventions and surgeries can be avoided in such patients, and thus reduce morbidity as well as mortality, giving them a better quality of life. It would be a wise decision for a surgeon to keep in mind about Hirschprung's disease before managing any case of colonic volvulus irrespective of age of patient.
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[Figure 1], [Figure 2], [Figure 3]