Type I congenital multiple intraspinal extradural cysts associated with distichiasis and lymphedema syndrome

Surgical Neurology
Imad N KanaanFaisal Otaibi

Abstract

The hereditary syndrome of multiple congenital intraspinal cysts associated with distichiasis, lymphedema and other congenital deformities is extremely rare. Modern imaging techniques have promoted the non-invasive diagnosis of spinal pathology and paved the way for better surgical planning. We reviewed the clinical data, imaging studies and treatment outcomes of a 12-year-old boy with this syndrome. Progressive spastic paraparesis with signs of spinal cord compression leading to frequent falls. This was associated with bilateral double row of eyelashes and pretibial edema. The MRI of thoracic spine depicted two large elongated extradural lesions extending from D5-D10 with signal intensity compatible with cerebrospinal fluid leading to severe compression of the spinal cord dorsally. Laminotomy and complete microsurgical excision of the cysts resulted in a fast and full clinical recovery of his neurological deficit. Type I congenital intraspinal cysts is a rare etiology of cord compression syndrome and may be associated with distichiasis, lymphedema and other congenital deformaties. It has several characteristics, which include the higher incidence in thoracic spine and younger age group, disproportional sever motor deficit as c...Continue Reading

References

Mar 1, 1988·Journal of Neurosurgery·M W NaborsH V Rizzoli
Sep 1, 1986·Surgical Neurology·K S Kim, P E Weinberg
Sep 1, 1967·The American Journal of Roentgenology, Radium Therapy, and Nuclear Medicine·K Y Chynn
Apr 1, 1980·Annals of Neurology·J F SchwartzJ C Hoffman
Jul 27, 1999·American Journal of Human Genetics·J MangionS Jeffery
Jul 28, 1999·Minimally Invasive Neurosurgery : MIN·I KanaanB Sheikh
May 8, 2002·Pediatric Dermatology·Curt P Samlaska

❮ Previous
Next ❯

Citations

Jul 14, 2007·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Ricardo Santos de OliveiraHelio Rubens Machado
Oct 16, 2009·Cases Journal·Mohammad Ali BitarafZohreh Habibi
Nov 13, 2013·International Journal of Dermatology·Spring GoldenJerry D Brewer
Jul 1, 2011·Journal of Korean Neurosurgical Society·Kyung-Chul ChoiSang-Ho Lee
May 11, 2010·Ophthalmic Genetics·Antonella FabrettoPaolo Gasparini
Jun 11, 2010·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Rocío Sánchez-CarpinteroAna Patiño-García

❮ Previous
Next ❯

Related Concepts

Related Feeds

CSF & Lymphatic System

This feed focuses on Cerebral Spinal Fluid (CSF) and the lymphatic system. Discover the latest papers using imaging techniques to track CSF outflow into the lymphatic system in animal models.

Birth Defects

Birth defects encompass structural and functional alterations that occur during embryonic or fetal development and are present since birth. The cause may be genetic, environmental or unknown and can result in physical and/or mental impairment. Here is the latest research on birth defects.