MyPACS.net: Radiology Teaching Files > Case 11345598

previously visited 21 DAY OLD FEMALE WITH CONSTIPATION
Contributed by: Mohamed Eltomey, Radiologist, Tanta University Hospitals-Radiology & Imaging Dept., Egypt.
Patient: 21 day old female
History: A 21 day old female presented with marked abdominal distention and constipation, the patient had passed meconium in the early neoanatal peroid.
Images:[small]larger

Fig. 1: Plain film showing multiple air fluids levels denoting obstruction, note also the marked abdominal distension

Fig. 2: Contrast Enema showing a transitional zone at the splenic flexure

Fig. 3: Contrast enema showing the irregular outline of the entire colon
Findings: The plain radiograph showed multiple air fluids and distention denoting intestinal obstruction.

The contrast enema performed showed irregularity of the entire colonic contour and a transitional zone at the level of the splenic flexure.

The diagnosis of the Hirschsprung's Disease with a transitional zone "arrow" (Pseudotransitional zone, see later) at the splenic flexure was initially made and the patient was taken to surgery immediatly after the enema.

On surgery the irregular outline of the colon raised the suspescion of a Total Colonic Hirschsprung's Disease. An ileosotomy was done and multiple biopsies of the colonic were taken which proved a Total colonic Hirschsprung's Disease.
Diagnosis: Total Colonic Hirschsprung's Disease
Discussion:

The radiologic diagnosis of total colonic Hirschsprung's disease is very difficult. Findings include a normal barium enema, a short colon of normal caliber, a microcolon, or a transition zone in the ileum.

Additional findings include easy, extensive reflux far back into small bowel, a pseudotransition zone in the colon, and intraluminal small bowel calcification.

As patients with total aganglionosis may present with a microcolon, the differential diagnosis has to be made with ileal atresia. The presence of bowel contents in the dilated ileum narrows the differential diagnosis to Hirschsprung's disease or meconium ileus.

MORE ABOUT Hirschsprung's disease

*Hirschsprung's disease results from failure of relaxation of distal aganglionic bowel, which leads to a functional obstruction to the passage of intestinal contents.

*Hirschsprung's disease has an overall incidence of 1 in 4,000 live births. It accounts for 20 to 25% of the cases of neonatal intestinal obstruction. The disease affects four times as many boys as girls, and 8% of patients with Hirschsprung's disease also have Down syndrome.

*A common presentation of Hirschsprung's disease in the newborn is failure to pass meconium during the first few days of life, with subsequent passage of a meconium plug followed by sparse bowel movements. Gastrointesinal bleeding and diarrhea are danger signs for Hirschsprung's disease associated enterocolitis.

*Plain films are helpful in neonatal Hirschsprung's disease, they demonstrate a high grade distal bowel obstruction, a dilated colon proximal to the distal and smaller aganglionic segment is more typical.

*Definitive diagnosis requires a contrast enema. Stress on the point that balloon catheters should never be used: at best, they may obscure the diagnosis; at worst, they may perforate the stiff aganglionic rectum. The critical view is the lateral view of the rectum, obtained during slow filling.The important findings on contrast enema examination with Hirschsprung's disease are as follows:  
  • Denervation hyperspasticity (narrowing) of the distal segment, a highly specific fluoroscopic finding. Spot films show a spastic or "sawtooth" configuration.
  • A transition zone or an abrupt change in bowel width between the spastic segment and the innervated portion that has dilated from obstruction.
  • Decreased rectosigmoid ratio. In aganglionosis, the rectum is smaller than the dilated sigmoid, whereas normally the reverse is true. This sign is valid when aganglionosis does not involve the sigmoid. This sign is found to be exceedingly reliable sign, especially in infants, in whom a definite transition zone may beabsent.
  • Delayed and disordered evacuation. The normal colon propels the barium distally in a single, continuous bolus. In aganglionosis, the barium is interspersed with feces and, instead of being propelled distally, often appears proximal to its location of 24 to 48 hours previously. This reversed propulsion is especially pronounced in total colonic aganglionosis, in which retrograde filling of the small bowel is common.
  • Bowel shortening. Loss of redundancy of the sigmoid colon that is seen in normal infants is one of the manifestations of aganglionosis. This shortening becomes more pronounced in total aganglionosis. The colon has been described as resembling a question mark.
  • Meconium plug. Meconium plug and Hirschsprung's disease coexist in 10% to 30% of cases. Frequently, a meconium plug is assumed to be the sole cause of a neonatal obstruction, and the diagnosis of Hirschsprung's disease is missed.
References: Hernanz-Schulman M: Imaging of neonatal gastrointestinal obstruction. Radiological clinics of North America 37(6):1163-1186, 1999.

Buonomo C: Neonatal gastrointestinal emergencies. Radiol Clin North Am 35:845-864, 1997.

Klienhaus S, Boley SJ, Sheran M: Hirschsprung's disease: A survey of the members of the American Academy of Pediatrics. J Pediatr Surg 14:588-597, 1979.

Newman B, Nussbaum AR, Kirkpatrick JA Jr: Bowel perforation in Hirschsprung's disease. AJR Am J Roentgenol 148:1195-1197, 1987.

Millar KE: The child with constipation. In Hilton SW, Edwards DK (eds.) Practical pediatric radiology. W.B. Saunders Company, Philadelphia, London, Toronto, Montreal, Sydney, Tokyo. 1994.
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Additional Details:

Case Number: 11345598Last Updated: 10-18-2007
Anatomy: Gastrointestinal (GI)   Pathology: Congenital
Modality: GIExam Date: 01-01-2003Access Level: Readable by all users
Keywords: hirschsprung's disease

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