Infantile sigmoid colon stricture secondary to cytomegalovirus
On examination, the baby was undernourished, with marked abdominal distension. An abdominal radiograph demonstrated generalized, grossly dilated bowel loops. A rectal biopsy showed a negative acetylcholinesterase reaction, and ganglions were seen in the submucosa, excluding Hirschsprung disease. An upper gastrointestinal contrast study confirmed normal intestinal rotation, with contrast passing freely through markedly dilated colonic loops. A subsequent lower gastrointestinal contrast study demonstrated retained contrast material within the dilated colon, with an area of severe narrowing in the distal sigmoid colon (Fig. 1).
At laparotomy, a massively dilated descending and proximal sigmoid colon was found, with a transition point in the distal sigmoid colon. The colon was opened at this point and a fibrotic stricture approximately 6 cm in length was noted (Fig. 2a,b). This was excised and submitted for histology. An end sigmoid colostomy was fashioned and the rectal stump was oversewn. The patient received IV gancyclovir for several weeks until PCR testing of the blood for CMV was negative. Nasogastric feeding was re‐established and she was discharged 4 weeks post‐operatively.
Histological examination of the resected specimen identified an inflammatory stricture with extensive mucosal ulceration. The floor of the ulcer was composed of granulation tissue, with a dense mixed inflammatory infiltrate covered by fibrinopurulent exudates. A very occasional intranuclear inclusion body characteristic of CMV was seen, confirmed by immunohistochemistry (Fig. 3).
CMV is a member of the herpes virus group and is the commonest congenital viral infection with an incidence of 0.2–2.2%.1 Infection in the neonate may be congenital, perinatal or post‐natal. Congenital infection is due to maternal primary CMV infection or reactivation of the virus. Perinatal infection may occur during delivery. Post‐natal infection occurs most often from infected breast milk or transfusion with CMV‐infected blood products.
Congenital CMV infection may produce a wide range of clinical symptoms affecting the central nervous system, liver, spleen and eyes, but gastrointestinal involvement is uncommon.2 However, any part of the gastrointestinal tract may be involved. The association between necrotizing enterocolitis and CMV inclusion bodies on pathologic examination was first reported in 1979.3 Since then, reported surgical presentations with neonatal CMV infection have included meconium peritonitis, intestinal perforation and colonic dysmotility.4 The mechanism remains unknown, with one hypothesis suggesting CMV infection induces intestinal inflammation and a vasculitis, leading to intestinal ischaemia and destruction of the myenteric plexus.4
CMV infection causing a neonatal colonic stricture has only been described three times before, all in premature or small for gestational age neonates, as in this patient.2 This case demonstrates that clinicians should be wary of the gastrointestinal complications of CMV infection in neonates and infants. In particular, symptoms of abdominal distension, feed intolerance or decreased stool frequency in a neonate with a history of CMV should prompt evaluation for intestinal strictures.