Occult spinal dysraphism

Case contributed by Dr Varun Babu

Presentation

Chronic constipation, with a sacral dimple - to rule out spinal abnormalities.

Patient Data

Age: 2
Gender: Male
Modality: X-ray

Winking owl sign or the absent pedicle sign of left posterior element of L3 vertebra. 

MRI of whole spine

Modality: MRI

See below

Case Discussion

This baby was admitted with long standing history of constipation under evaluation. The child had no neurological deficits and was symptomatically treated with milk of magnesia. The persistent symptoms led to a detailed clinical and neurological evaluation which identified a sacral dimple. This prompted the clinician to send the patient for a screening MRI.

Prior to the MRI the previous radiographs were evaluated and this identified a subtle yet key pointer to the condition. The absent pedicle sign or the winking owl sign are pathognomic of posterior element affliction that can be identified on frontal radiographs of the spine. 

A detailed MRI study including screening of the whole spine is a good pointer to identify any underlying congenital neural tube defects. 

This baby had dorsal and lumbar cord hydromyelia extending to conus with vertebral defect and intrathecal lipomatous component - all fitting in with a picture of occult spinal dysraphism. Babies may present with only constipation and this is one of the final diagnoses considered if no cutaneous markers or any neurologic deficits are identified. 

Take home message: Every pediatric case with constipation - careful assessment of the spine and cutaneous markers will enable early diagnosis and management. 

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Case Information

rID: 42433
Case created: 20th Jan 2016
Last edited: 21st Apr 2017
Systems: Paediatrics, Spine
Inclusion in quiz mode: Included

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