Split Notochord Syndrome With Dorsal Enteric Fistula In A Male Neonate: A Case Report
Dr Rajendra K Ghritlaharey,MS (Gen Surg), M. Ch. (Paed Surg), FAIS Associate Professor,Department of Paediatric Surgery,Gandhi Medical
College & Associated Kamla Nehru &
Hamidia Hospitals Bhopal, Madhya
Pradesh 462 001(INDIA)Phone No. 91-55-4050571(R), 4050261(O)E-mail:
Split notochord syndrome (SNS) is an extremely rare congenital anomaly and 33 such cases have been reported in literature, till date. Of these 33 cases of SNS; 20 presented with associated dorsal enteric fistula. We are reporting here a 2.5 kg male neonate, born normally at hospital, to a 24 yrs old Gravida II Para I mother. Clinical examination and investigations revealed SNS associated with hydrocephalus (Arnold Chiari malformation), thoracic meningomyelocele and dorsal enteric fistula just below meningomyelocele. He also had associated imperforate anus, reducible right inguinal hernia and penoscrotal transposition. Staged correction of the disease was planned. Right inguinal herniotomy and sigmoid loop colostomy was done on 14th day of age as a first stage of the operation. His father was reluctant for further operation at present therefore he was discharged on request after a week of operation. The reason for reporting the case is its rarity.