Related Experiment Videos
MR of terminal myelocystoceles
S E Byrd1, C Harvey, C F Darling
1Department of Radiology, Children's Memorial Hospital, Chicago, IL 60614, USA.
Insights
Terminal myelocystocele, a rare spinal dysraphism, presents as a back mass in infants. Early diagnosis via MRI is crucial for favorable neurologic outcomes and identifying associated conditions like cloacal exstrophy.
Area of Science:
- Pediatric Neurology
- Medical Imaging
- Developmental Biology
Background:
- Spinal dysraphism encompasses a spectrum of congenital anomalies.
- Terminal myelocystocele is an uncommon variant with potential for good prognosis if identified early.
- Accurate diagnosis is essential for appropriate management and intervention.
Purpose of the Study:
- To characterize the clinical and Magnetic Resonance (MR) imaging findings in children diagnosed with terminal myelocystocele.
- To emphasize the importance of early diagnosis through understanding MR features.
- To correlate clinical presentation with imaging findings.
Main Methods:
- Retrospective analysis of medical records and MR imaging studies.
- Inclusion of 15 pediatric patients with surgically and histologically confirmed myelocystocele.
- Detailed review of imaging for spinal cord abnormalities and associated central nervous system findings.
Main Results:
- MR imaging consistently revealed a terminal cyst of the spinal cord's central canal, tethered and herniated through dysraphia as a dorsal mass.
- Commonly associated findings included Chiari malformations, hydromyelia, hydrocephalus, vertebral segmentation anomalies, and sacral agenesis.
- Clinical presentation frequently involved a dorsal mass, with a high incidence of cloacal exstrophy and ambiguous genitalia, while patients remained neurologically intact.
Conclusions:
- Terminal myelocystocele typically presents with a dorsal mass and shows a strong association with cloacal exstrophy.
- Magnetic Resonance (MR) imaging is the optimal non-invasive tool for diagnosing terminal myelocystocele and its associated central nervous system anomalies.
- Early and comprehensive diagnosis via MR imaging facilitates timely intervention and improves patient outcomes.
Purpose:
To delineate the clinical and MR findings in children with an unusual type of spinal dysraphism, the terminal myelocystocele. Infants with a terminal myelocystocele carry a favorable neurologic prognosis if the entity is diagnosed early. Understanding the MR characteristics of this entity will allow for earlier and more accurate diagnosis.
Method:
Analysis of the medical charts and MR studies in 15 children with surgically and histologically proven myelocystocele.
Results:
In all 15 children, MR demonstrated the primary findings of a terminal cyst of the central canal of the spinal cord which is tethered and herniated with arachnoid and cerebrospinal fluid through an area of spinal dysraphia onto the back as a mass. Of these children, 10 had additional findings (one or more) on MR of Chiari I (five cases), Chiari II (one case), cervicothoracic hydromyelia (two cases), lumbar hydromyelia (two cases), hydrocephalus (2 cases) segmentation anomalies of vertebrae (3 cases) and partial agenesis of sacrum (six cases). Of the clinical findings, all 15 children had a back mass, 10 also had cloacal exstrophy. One had imperforate anus, 10 were girls, five had ambiguous genitalia and all were neurologically intact.
Conclusion:
Children with a terminal myelocystocele present with a back mass and there is a high association with cloacal exstrophy. MR is the best noninvasive modality to diagnose all of the components of a terminal myelocystocele and the associated central nervous system findings.