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Immaturity of Ganglion Cells - A Study of Our Own Material

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Markiewicz-Kijewska M., Kowalski A., Bacewicz L., Drewniak T., Ismail H., Kluge P., Kaliciński P.

Polish Journal of Surgery

2009

vol. 81

issue 2

95-102

The most common causes of clinical symptoms of bowel obstruction in newborn are the various dysmotylity disorders.The aim of the study was a retrospective analysis of the newborn's case history with the diagnosis of ganglion cells immaturity, their clinical course, diagnostic imagine and treatment in our own material.Material and methods. In the years 1981-2007 we treated in the Department of Pediatric Surgery in Children's Memorial Health Institute 420 children with various dysmotylity disorders so-called dysganglionosis. Among them were 15 infants, who finally were diagnosed ganglion cells immaturity. In all clinical symptoms associated with impaired motoric function of large bowel occurred in the first week of life. We performed a retrospective analysis of the case history of these children's including: demographic data, perinatal anamnesis, the dominant clinical symptoms, how to conduct an emergency and a final treatment, and long term follow-up.Results. The children were born at 28-40 week of pregnancy (mean 35,9 weeks) with the body mass between 0.95 and 4.15 kg (mean 2,72 kg), 7 of them were premature infant in 28-36 week of pregnancy and body mass 950-2900 g (mean 1970 g). All infants after birth were evaluated by the Apgar's scale of the 1-10 point (mean 8.5). The first meconium in the first 24 hours of life passed only 1 infant, the remaining 12 meconium passed delayed from 2 to 4 day of life. In 5 children were present concomitant diseases and malformations making it difficult to establish early correct diagnosis: congenital gastroschisis (1 child), intestinal volvulus (1 child) and necrotizing enterocolitis (NEC) (3 children). Diagnosis of ganglion cells immaturity was found at age from 1 to 365 day on the basis of the clinical course, radiological imagine (11 children) and histopathological test of rectal biopsy specimens (9 children) or surgical biopsy specimens (6 children). Four children were treated medically; the others require the temporary emergence colostomy or ileostomy. Functional maturity of ganglion cells identified between 2 and 16.5 month on the basis of electromanometric study (12 children), and/or rectal biopsy specimens (7 children) and in 3 only on clinical course. GI tract reconstruction was performed in the other 11 children at aged from 4.2 to 20.3 month of life, mean 10.6 month. After GI tract reconstruction in 1 child it was mechanical adhesive ileus and malabsorption of unknown origin requiring partial parenteral nutrition. In another child remained ileus and chronic cholestasis, this patient died after next laparotomy because of bleeding and failure liver function. Other patients live in a good general condition with a normal motoric function of the digestive tract from long term follow-up 1.8 to 17.8 years (mean 6.7 years).Conclusions. Early differential diagnosis of dysganglionosis is difficult, but possible subject to the full panel of diagnostic (radiological imagine, electromanometric and histopathological study) already in newborn with impaired bowel motoric function. Only in such cases with correct diagnosis patient can be adequately treated and to guarantee good initial and definitive treatment.

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1 Polish Journal of Surgery

1 2009

1 Bacewicz L.

1 Drewniak T.

1 Ismail H.

1 Kaliciński P.

1 Kluge P.

1 Kowalski A.

1 Markiewicz-Kijewska M.

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Immaturity of Ganglion Cells - A Study of Our Own Material