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Cervical cord tethering due to split cord malformation at the cervico-dorsal junction presenting with self-mutilation

L M Myles¹, A J W Steers, R Minns

¹The Royal Hospital for Sick Children, Sciennes Road, Edinburgh EH9 1UW, Scotland, UK.

Developmental Medicine and Child Neurology

|November 29, 2002

Summary

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This case study details a rare spinal cord malformation in an infant, highlighting the importance of early diagnosis for conditions like cervical spinal cord tethering and diplomyelia.

Area of Science:

Neurology
Developmental Biology
Pediatric Neurosurgery

Background:

Spinal cord tethering and diplomyelia are rare congenital anomalies.
Early identification is crucial for preventing neurological deficits.

Observation:

A 12-month-old female presented with sensory loss, absent reflexes, and motor deficits in her hands.
Physical examination revealed a deep skin dimple over the T1 spinous process.
Imaging demonstrated lower cervical cord angulation.

Findings:

Surgical exploration identified a low cervical meningocele and a split cord malformation.
One half of the spinal cord was tethered and subsequently untethered.
The case represents an unusual presentation of cervical spinal cord anomalies.

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Implications:

This case underscores the need for thorough neurological evaluation in infants with unexplained motor and sensory deficits.
Prompt surgical intervention for spinal cord tethering can prevent further neurological damage.
Understanding these rare malformations aids in improving diagnostic and treatment strategies.