Radiology · 1973 · 34 citations · 0 references
MedicineClinical SciencesPediatricsGastroenterologyNeonatologyPediatric GastroenterologyGastrointestinal PathologyIntestinal ObstructionNeonatal Gastrointestinal DisordersPediatric Gastrointestinal DisordersPediatric Intestinal DisordersCongenital AganglionosisHirschsprung Disease-related ObstructionLarge Bowel ObstructionNeonatal Intestinal ObstructionPediatric Gastrointestinal Pathology
Thirteen infants with total aganglionosis coli (TAC) are described. The data from these and 128 other cases in the English literature are reviewed and summarized. TAC affects more females than males and has a higher incidence of familial rather than the usual forms of Hirschsprung's disease. Most infants (81%) require hospitalization in the first month of life. Mortality is high (68%). Radiographic findings are not diagnostic. Microcolon (38%), normal barium enema (22%), or delayed evacuation of contrast medium (21%) suggest TAC in a full-term newborn infant with signs of low, small bowel obstruction. Open biopsy establishes the diagnosis.