Immaturity of Ganglion Cells - A Study of Our Own Material
Languages of publication
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.
1 - 2 - 2009
20 - 3 - 2009
- Loening-Baucke V, Kiura K: Failure to pass meconium: diagnosis neonatal intestinal obstruction. Am Fam Physician 1999; 60(7): 2043-50.
- Scharli AF: Standarization of terminology of intestinal innervation disorders. Ped Surg Int 1995; 10: 440.
- Scharli AF: Intestinal innervation disorders. Gastroenterol Polska 1996; 3: 113-18.
- Kaliciński P, Drewniak T, Ismail H i wsp.: Organiczne zaburzenia motoryki u dzieci. Medipress Pediatria 1998; 4(5): 2-10.
- Taguchi T, Masumoto K, Ieiri S et al.: New classification of hypoganglionosis: congenital and acquired hypoganglionosis. J Ped Surg 2006; 41: 2046-51.
- Spencer B: Problems in rectal biopsy due to immaturity of ganglion cells in seminar on pseudo-Hirschsprung's disease and related disorders. Arch Dis Child 1966; 41: 149.
- Heaton ND, Howard ER, Garrett JR: Small left colon syndrome: an immature enteric plexus. J R Soc Med 1991; 84: 2: 113-14.
- Puri P: Variant Hirschsprung's disease. J Ped Surg 1997; 32(2):149-57.[PubMed]
- Martucciello G, Prato AP, Puri P et al.: Controversies concerning diagnostic guidelines for anomalies of the enteric nervous system: a report from the 4 International Symposium on Hirchsprung's disease and related neurocristopathies. J Ped Surg 2005; 40: 1527-31.
- Ure BM, Holschneider AM, Schulten D et al.: Clinical impact of intestinal neuronal malformations: a prospective study in 141 patients. Ped Surg Int 1997;12: 377-82.
- Komuro H, Urita Y, Hori T et al.: Perforation of the colon in neonates. J Ped Surg 2005; 40: 1916-19.
- Hyakawa K, Hamanaka Y, Suzuki M et al.: Radiological findings in total colon aganglionosis and allied disorders. Radiation Medicine 2003; 21: 3: 128-34.
- Teitelbaum DH, Cilley RE, Sherman NJ et al.: A decade of experience with the primary pullthrough for Hirschsprung's disease in the newborn period: a multicenter analysis of outcome. Ann Surg 2000; 232: 372-80.
- De la Torre-Mondragon L, Ortega-Salgado JA: Transanal endorectal pull-through for Hirschsprung's disease. J Ped Surg 1998; 33(8): 1283-86.
- Langer JC, Fitzgerald PG, Winthrop AL et al.: One-stage versus two stage Soave pull-through for Hirschsprung's disease in the first year of life. J Ped Surg 1996; 31(1): 33-37.
- Teeraratkul S: Transanal one-stage endorectal pull-through for Hirschsprung's disease in infants and children. J Ped Surg 2003; 38(2): 184-87.
Publication order reference