A Case Report of Caudal Regression Syndrome Associated with an Intraspinal Arachnoid Cyst

We report here a rare case of caudal regression syndrome associated with an intraspinal arachnoid cyst. The patient was a 6-month-old baby girl with multicomplex congenital abnormalities: sacrococcygeal dysgenesis and ventral curvature, large terminal cyst (myelocystocele), spinal arachnoid cyst, cerebellar hypertrophy (suspected), high imperforate anus, partial dysgenesis of the large intestine, omphalocele, atresia of the vagina, bilateral incomplete ureter duplication, incomplete pseudoduplicated bladder and bilateral talipes equinovarus. We performed plastic repair of the myelocystocele and perineal lesion for caudal regression syndrome and partial removal of the cyst wall for the intraspinal arachnoid cyst. She has been well for 3 years postoperatively, and her mental development is normal.

[1]  D. Subtil,et al.  Early detection of caudal regression syndrome: specific interest and findings in three cases. , 1998, European journal of obstetrics, gynecology, and reproductive biology.

[2]  D. Pretorius,et al.  Terminal myelocystocele: important differential diagnosis in the prenatal assessment of spina bifida. , 1998, Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine.

[3]  V. Capra,et al.  Multidisciplinary management of caudal regression syndrome (26 cases). , 1996, European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie.

[4]  D. S. Martin,et al.  Imaging of the Pediatric Head, Neck, and Spine , 1996 .

[5]  R. Nievelstein,et al.  Abnormal anatomy of the lumbosacral region imaged by magnetic resonance in children with anorectal malformations. , 1996, Archives of disease in childhood.

[6]  D. Barlev.  Imaging of the Pediatric Head, Neck, and Spine , 1996 .

[7]  A. Adra,et al.  Caudal regression syndrome: etiopathogenesis, prenatal diagnosis, and perinatal management. , 1994, Obstetrical & gynecological survey.

[8]  R. Nievelstein,et al.  MR of the caudal regression syndrome: embryologic implications. , 1994, AJNR. American journal of neuroradiology.

[9]  J. V. van Gool,et al.  Urodynamic evaluation of children with the caudal regression syndrome (caudal dysplasia sequence). , 1994, The Journal of urology.

[10]  R. Nievelstein,et al.  Embryonic development of the mammalian caudal neural tube. , 1993, Teratology.

[11]  D. Pang,et al.  Sacral agenesis and caudal spinal cord malformations. , 1993, Neurosurgery.

[12]  T. Naidich,et al.  Congenital Anomalies of the Spine and Spinal Cord , 1992 .

[13]  A. Barkovich,et al.  The wedge-shaped cord terminus: a radiographic sign of caudal regression. , 1989, AJNR. American journal of neuroradiology.

[14]  T. G. Pait,et al.  Updated assessment and current classification of spinal meningeal cysts. , 1988, Journal of neurosurgery.

[15]  F. Müller,et al.  Somitic-vertebral correlation and vertebral levels in the human embryo. , 1986, The American journal of anatomy.

[16]  P. Frérebeau,et al.  [Spinal intradural arachnoid cyst]. , 1985, Archives francaises de pediatrie.

[17]  R. Galzio,et al.  Spinal intradural arachnoid cyst. , 1982, Surgical neurology.

[18]  A. Cass,et al.  Sacral agenesis: an analysis of 11 cases and review of the literature. , 1979, The Journal of urology.

[19]  R. White,et al.  Sacral agenesis. Analysis of 22 cases. , 1976, Urology.

[20]  D. Price,et al.  Caudal dysplasia (caudal regression syndrome). , 1970, Archives of neurology.

[21]  E. Passarge,et al.  Syndrome of caudal regression in infants of diabetic mothers: observations of further cases. , 1966, Pediatrics.

[22]  B. Duhamel.  From the Mermaid to Anal Imperforation: The Syndrome of Caudal Regression* , 1961, Archives of disease in childhood.

[23]  N. Tomura,et al.  Caudal regression syndrome: MR appearance. , 1998, Computerized medical imaging and graphics : the official journal of the Computerized Medical Imaging Society.

[24]  M. Nelson,et al.  Anorectal malformations: Evaluation of associated spinal dysraphic syndromes , 1988 .

[25]  P. Barnes,et al.  Neuroradiologic evaluation of sacral abnormalities in imperforate anus complex. , 1987, Journal of pediatric surgery.

[26]  J. Carey,et al.  The OEIS complex (omphalocele, exstrophy, imperforate anus, spinal defects). , 1978, Birth defects original article series.