Diagnosi precoce e trattamento di un caso di disrafismo spinale occulto con "diastematomielia" e doppio seno dermico

Translated title of the contribution: Early diagnosis and treatment of a case of occult spinal dysraphism with diastematomyelia and double dermal sinus

Mario Crispino, C. Zambelloni, A. Fontana, S. Savasta, C. Poggiani, L. Olivetti

Research output: Contribution to journalArticle

Abstract

The malformations characterising split cord malformation are rare forms of occult spinal dysraphism which develop early in foetal life. Affected patients are at high risk of developing severely invalidating neurological deficits due to the tethered cord. At birth, most babies present skin abnormalities (hypertrichosis, naevi, angiomas, foveolas or fistulae and subcutaneous lipomas) usually in the lumbosacral region which are often the only sign of underlying disease. We describe the neuroradiological and neurosurgical features of a rare case of split cord associated with tethered cord, double dermal sinus and multiple vertebral abnormalities. A newborn girl delivered spontaneously at the 38th week had a birth weight of 3100g and an Apgar score of 8/10. She presented cutaneous dysraphic stigmata and modest bilateral patellar hyperreflexia with mild hypotonia of the lower limbs. Brain and spine neuroradiological investigation by magnetic resonance and computed tomography on the second day of life disclosed a double spinal cord with a single dural sheath (Pang's type II), a double dermal sinus at L3-L4 and at the coccyx with tethered cord and vertebral abnormalities (butterfly vertebra at T12 and multiple schisis). At 22 days of life the infant underwent successful surgery to untether the cord. Operative findings confirmed the neuroradiological diagnosis. Serial clinical and neuroradiological follow-up in subsequent months including urodynamic testing failed to disclose neurological or spincter deficits. One year later the child had normal psychomotor development and neurological examination. Spinal dysraphism, often including rare complex malformations, requires early diagnosis and prompt treatment to avoid the onset of severe irreversible neurological deficits. Skin abnormalities are often the only sign of the malformation, especially in neonates. Neuroradiological diagnosis (namely magnetic resonance) is highly reliable in pinpointing the causes of tethered cord thereby allowing successful treatment.

Translated title of the contributionEarly diagnosis and treatment of a case of occult spinal dysraphism with diastematomyelia and double dermal sinus
Original languageItalian
Pages (from-to)683-690
Number of pages8
JournalRivista di Neuroradiologia
Volume16
Issue number4
Publication statusPublished - Aug 2003

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Clinical Neurology
  • Radiological and Ultrasound Technology

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