title: Rachischisis totalis without acrania in a newborn male.
abstract: BACKGROUND
  Rachischisis totalis is a rare open neural tube defect often associated with lethal acrania. When acrania is not present, mortality remains high and morbidity for survivors is extreme.
  
  
  METHODS
  We describe is a term newborn with in-utero diagnosed rachischisis totalis without acrania and Chiari II malformation with subsequent hydrocephalus.
  
  
  RESULTS
  At birth, clinical examination revealed rachischisis totalis without acrania. The spinal defect extended from the mid-cervical to the lower sacral vertebrae. Serial cranial ultrasonography and brain MRI revealed Chiari II malformation with severe hydrocephalus. The spinal defect was closed and a ventriculoperitoneal shunt placed to relieve the hydrocephalus. The clinical course was complicated by significant central apnea, often necessitating aggressive resuscitation and chest compressions. The infant has survived with expected severe developmental delay but has graduated from hospice care.
  
  
  CONCLUSIONS
  This report demonstrates that survival with variable function and quality of life is possible with severe open neural tube defects. A multidisciplinary team approach including family-centered care must be used early to overcome the many ethical challenges that such patients present.
authors.affiliation: Division of Neonatology, University of Tennessee Medical Center, Knoxville, Tennessee. Electronic address: jmeadows@mc.utmck.edu.
authors.forename: John Thomas
authors.initials: JT
authors.lastname: Meadows
authors.affiliation: Department of Pediatrics and Internal Medicine, The Ohio State University, Nationwide Children's Hospital, Columbus, Ohio.
authors.forename: Don
authors.initials: D
authors.lastname: Hayes
mesh_terms: D001139:Arnold-Chiari Malformation / Q000150:complications; D001921:Brain / Q000473:pathology; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology* / Q000601:surgery; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D009436:Neural Tube Defects / Q000175:diagnosis / Q000601:surgery; D013116:Spinal Cord / Q000000981:diagnostic imaging; D016135:Spinal Dysraphism / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D014463:Ultrasonography; D017287:Ventriculoperitoneal Shunt
keywords: Chiari II malformation; neural tube defect; rachischisis; spina bifida