Complications
Acute episodes of dehydration and metabolic decompensation are common complications. Neurological and psychological symptoms (eg, developmental delay) may be related to the hypovolemia-related temporary ischemia. [19] Impaired renal function and nephrocalcinosis may also occur. Infectious complications of the central line that result in sepsis are the most frequent causes of death, followed by liver failure. [12]
Multiple hepatic adenomas have recently been described in a child with microvillus inclusion disease. [13]
Prognosis
The prognosis is poor. If patients are untreated, the disease is rapidly fatal because of dehydration and malnutrition.
If patients are treated with total parenteral nutrition (TPN), their prognosis entirely depends on the complications of this approach. These complications include cholestasis with subsequent liver damage leading to cirrhosis, catheter-related sepsis due to infection with bacterial or fungal agents, and progressive lack of vascular access.
The limited experience accumulated in a few centers worldwide reflects an overall survival rate of approximately 50% at 5 years after small-bowel transplantation; this is a much better outcome than is seen with other indications for intestinal transplantation. [9]
Children with late-onset congenital microvillous atrophy usually have less severe diarrhea; with age they can reduce the requirements of TPN to 1-2 per week.
