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Klippel-Feil syndrome associated with persistent trigeminal artery
Yahya Paksoy1, Muzaffer SEker, Erdal Kalkan
1Ultra Görüntüleme Merkezi, Konya, Turkey. yahyapaksoy@yahoo.com
Spine
|April 24, 2004
Summary
Klippel-Feil syndrome, a congenital spinal malformation, can co-occur with a persistent trigeminal artery and other anomalies. This rare presentation highlights the importance of searching for related developmental defects.
Area of Science:
- Neurology
- Radiology
- Developmental Biology
Background:
- Klippel-Feil syndrome involves congenital cervical vertebral fusion, often with other organ system defects.
- Persistent trigeminal artery is a fetal anastomosis between carotid and vertebrobasilar circulations.
Observation:
- A rare case of Klippel-Feil syndrome presented with a persistent trigeminal artery.
- Additional anomalies included spina bifida occulta, cervical spondylosis, atlanto-occipital assimilation, and bilateral cervical ribs.
Findings:
- Magnetic resonance angiography confirmed the persistent trigeminal artery and revealed absent flow in the right vertebral artery's C4 segment.
- Radiologic review detailed vertebral fusion and other associated skeletal abnormalities.
Implications:
- The co-occurrence of Klippel-Feil syndrome and persistent trigeminal artery suggests a potential link to embryologic developmental dysfunction.
- This case underscores the need for comprehensive evaluation for associated anomalies in such presentations.
- Magnetic resonance angiography is a valuable noninvasive tool for diagnosing persistent trigeminal artery.