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Imaging of terminal myelocystoceles
S E Byrd1, C Harvey, D G McLone
1Division of Neuroimaging, Children's Memorial Hospital, Chicago, IL 60614, USA.
Insights
Terminal myelocystoceles, a congenital spinal defect, often present at birth as a mass and are frequently associated with cloacal exstrophy. Magnetic resonance imaging is the optimal diagnostic tool for evaluating these complex spinal malformations.
Area of Science:
- Pediatric Neurosurgery
- Medical Imaging
- Developmental Biology
Background:
- Terminal myelocystocele is a rare congenital spinal malformation.
- It involves the herniation of the terminal central canal cyst through a spinal dysraphism.
- Associated anomalies are common, impacting patient management and prognosis.
Purpose of the Study:
- To retrospectively analyze the clinical presentation, imaging findings, and associated anomalies in children with terminal myelocystocele.
- To evaluate the diagnostic efficacy of various imaging modalities for terminal myelocystocele.
- To highlight the characteristic imaging features of terminal myelocystocele.
Main Methods:
- Retrospective analysis of 20 pediatric patients with surgically and histologically confirmed terminal myelocystocele.
- Review of clinical presentations, including associated conditions like cloacal exstrophy.
- Assessment of imaging studies: plain radiographs, myelography-computed tomography, ultrasound, and magnetic resonance imaging.
Main Results:
- All 20 patients presented with a sacral mass at birth; 13 had concurrent cloacal exstrophy.
- Associated findings included Chiari I malformation (8 patients), Chiari II malformation (1 patient), hydromyelia (3 patients), hydrocephalus (3 patients), and vertebral segmentation anomalies (6 patients).
- Magnetic resonance imaging (MRI) proved superior in diagnosing and evaluating terminal myelocystocele, demonstrating the characteristic tethered terminal cyst with cerebrospinal fluid herniation.
Conclusions:
- Terminal myelocystocele requires a multidisciplinary approach due to frequent associated anomalies.
- MRI is the gold standard imaging modality for characterizing terminal myelocystocele and associated spinal cord abnormalities.
- Early and accurate diagnosis through advanced imaging is crucial for appropriate surgical planning and management.
Abstract:
This article presents a retrospective analysis of the presentation, imaging studies, and associated findings in 20 children with surgically and histologically proven terminal myelocystoceles. All 20 children presented at birth with a black mass; 13 had cloacal extrophy. The patient population was comprised of 15 girls and 5 with ambiguous genitalia: Of the imaging studies, 8 had plain radiographs, 6 myelography-computed tomography, 11 ultrasound, and 14 magnetic resonance. The associated findings included Chiari I (eight patients), Chiari II (one patient), hydromyelia (three patients), hydrocephalus (three patients), and vertebral segmentation anomalies (six patients). Magnetic resonance imaging was the best imaging modality to diagnose and evaluate children with a myelocystocele. Magnetic resonance imaging demonstrated the classic findings: a terminal cyst of the central canal of the spinal cord that is tethered and herniated with arachnoid and cerebrospinal fluid through an area of spinal dysphria onto the back as a mass.