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Terminal Myelocystocele: Surgical Observations and Theory of Embryogenesis

Pang, Dachling MD, FRCS(C), FACS*,‡; Zovickian, John MD, FACS; Lee, Ji Yeoun MD§; Moes, Greg S. MD‡,‖; Wang, Kyu-Chang MD, PhD§

doi: 10.1227/NEU.0b013e31824c02c0
Research-Human-Clinical Studies: Editor's Choice
Editor's Choice

BACKGROUND: The structural complexity of terminal myelocystocele (TMC) precludes a recognizable link to spinal cord development and therefore a plausible embryogenetic theory.

OBJECTIVE: To demystify TMC using clinical, imaging, surgical, electrophysiological, and histopathological data and to propose a theory of embryogenesis.

METHODS: Our series consisted of 4 newborns and 6 older children. All had preoperative magnetic resonance imagings and surgical repair of the myelocystocele with electrophysiological monitoring.

RESULTS: TMC can be deconstructed into essential and nonessential features. Essential features are present in all TMCs and constitute the core malformation, comprising an elongated spinal cord extending extraspinally into a cerebrospinal fluid--filled cyst that is broadly adherent to the subcutaneous fat. The functional conus resides in the proximal cyst or within the intraspinal cord, and the caudal myelocystocele wall is nonfunctional fibroneural tissue. Nonessential features include variable measures of hydromyelia, caudal meningocele, and fat, present in only some patients. The core structure of TMC strikingly resembles a transitory stage of late secondary neurulation in chicks in which the cerebrospinal fluid--filled bleblike distal neural tube bulges dorsally to fuse with the surface ectoderm, before focal apoptosis detaches it from the surface and undertakes its final dissolution. We theorize that TMC results from a time-specific paralysis of apoptosis just before the dehiscence of the cystic distal cord from the future skin, thereby preserving the embryonic state.

CONCLUSION: Besides tethering, the myelocystocele may show early rapid expansion causing precipitous deterioration. We recommend early repair with resection of the nonfunctional caudal cyst wall, reconstruction of the proximal neural placode, and duraplasty.

ABBREVIATIONS: HH, Hamburger-Hamilton

OEIS, omphalocele, bladder exstrophy, imperforate anus, and spinal anomaly

TM, terminal myelocystocele

TUNEL, terminal deoxynucleotidyl transferase--mediated deoxyuridine triphosphate nick-end labeling

*Department of Paediatric Neurosurgery, University of California, Davis

Regional Centre of Paediatric Neurosurgery and

Neuropathology, Oakland Medical Centre, Kaiser Foundation Hospitals of Northern California, Oakland, California

§Division of Paediatric Neurosurgery, Seoul National University Children's Hospital, Seoul, Korea

Department of Neuropathology, Stanford University School of Medicine, Stanford, California

Correspondence: Dachling Pang, MD, FRCS(C), FACS, Kaiser Permanente Medical Center, Department of Paediatric Neurosurgery, 280 W MacArthur Blvd, Oakland, CA 94611. E-mail:

Received May 17, 2011

Accepted January 4, 2012

Copyright © by the Congress of Neurological Surgeons