Hirschsprung's Disease With Impending Abdominal Compartment Syndrome
Article Outline
- Answer to the Clinical Challenges and Images in GI Question: Image 5: Hirschsprung's Disease With Impending Abdominal Compartment Syndrome
- References
- Copyright
Question: A 29-year-old man underwent a pull-through procedure for imperforate anus at birth. He had been bothered by difficulty with defecation and irregular bowel habits for >20 years. Because he had remained in good health, he did not seek further medical investigation. About 5 days before admission, he noticed progressive abdominal distention and swelling of the lower limbs. On admission he was thin and had an ovoid abdomen, which was dull on percussion. Laboratory data were notable for liver function impairment (aspartate aminotransferase, 789 U/L; alanine aminotransferase, 1277 U/L) and renal insufficiency (blood urea nitrogen, 29.6 mg/dL; creatinine, 1.3 mg/dL). Computed tomography of the abdomen revealed hepatodiaphragmatic colonic interposition (Chilaiditi syndrome) and marked distension of the colon and rectum with massive fecal retention (Figure A). Passive compression with displacement of the liver, spleen, and both kidneys with bilateral hydroureteronephrosis were evident (Figures B and C).
What is the most likely diagnosis? What is the proper management?
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Answer to the Clinical Challenges and Images in GI Question: Image 5: Hirschsprung's Disease With Impending Abdominal Compartment Syndrome
Because of organ dysfunction and worsening symptoms, decompressive laparotomy with near total proctocolectomy and fecal material removal (10 kg) were performed. Grossly, the resected megacolon measured 57 × 14 × 26 cm (Figure D). Histopathology (stain, hematoxylin and eosin original magnification, ×100; Figure E) and immunohistochemical staining (stain, synaptophysin; original magnification, ×200; Figure F) showed decompensated hypertrophic neurofibers (area between the arrows) and aganglionosis. After surgery, there was good improvement in hepatitis, renal disturbance, limbs edema, and hydroureteronephrosis. Although the coexistence of imperforate anus and Hirschsprung disease is extremely rare, associations between these 2 disorders have been reported.1 Our patient is extraordinary in that he had an imperforate anus at birth and was diagnosed with Hirschsprung disease as an adult. Owing to the rarity of the disease and the higher incidence of short or ultra-short segment aganglionosis in adults, prompt diagnosis of Hirschsprung disease in adults is much more difficult.2 As a result of chronic constipation and long-term fecal accumulation, he presented to our hospital with a tense, distended abdomen and symptoms of elevated intra-abdominal pressure. A deteriorating condition and intra-abdominal organ dysfunction implies impending abdominal compartment syndrome and surgical decompression is indicated.3 Being highly alert to the association of these 2 disorders is mandatory for initiation of early management.
References
- Imperforate anus, malrotation, and Hirschsprung's disease: a rare and important association. J Pediatr Surg. 2006;41:1335–1337
- Hirschsprung's disease in adults: report of a case and review of the literature. J Nippon Med Sch. 2005;72:113–120
- . Abdominal compartment syndrome: a concise clinical review. Crit Care Med. 2008;36:1304–1310
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Conflicts of interest The authors disclose no conflicts.
PII: S0016-5085(09)00510-1
doi:10.1053/j.gastro.2009.03.049
© 2009 AGA Institute. Published by Elsevier Inc. All rights reserved.
