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Neonatal congenital microvillus atrophy

¹ Princess Margaret and King Edward Memorial Hospitals, Neonatal Clinical Care Unit, Subiaco, Western Australia
² Department of Pathology, University of Western Australia, Western Australia

Correspondence to:
Correspondence to:
Dr Sanjay Patole
Department of Neonatal Pediatrics, King Edward Memorial Hospital for Women, Bagot Road, Subiaco, Western Australia 6008; skpatole{at}hotmail.com

Congenital microvillous atrophy (CMVA) is the leading cause of neonatal secretory diarrhoea with onset either in the first 72 hours of life (early onset) or at 6–8 weeks after birth (late onset). To date over 30 cases have been reported worldwide. The prognosis for this life threatening condition continues to be poor. Therapeutic agents like somatostatin and epidermal growth factor are either ineffective or of marginal benefit. Overall five year survival after small bowel transplantation is currently 50%. The following brief review is aimed towards helping neonatologists/perinatologists in the early diagnosis, and management of CMVA and in counselling the parents appropriately.

Keywords: congenital; diarrhoea; microvillus atrophy; neonates

Abbreviations: CMVA, congenital microvillous atrophy; PAS, periodic acid-Schiff