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Seckel syndrome and moyamoya
Patrick J Codd1, R Michael Scott, Edward R Smith
1Department of Neurosurgery, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts.
Journal of Neurosurgery. Pediatrics
|April 3, 2009
Summary
This report details the first case of Seckel syndrome and Moyamoya syndrome co-occurring, treated with surgical revascularization. The study highlights the importance of considering Moyamoya syndrome in dwarfism patients with ischemic symptoms.
Area of Science:
- Neurology
- Genetics
- Pediatrics
Background:
- Seckel syndrome is a rare genetic disorder causing severe growth delay and microcephaly.
- Moyamoya syndrome is a progressive cerebrovascular disease characterized by intracranial artery stenosis.
- Both conditions have been independently linked to neurological complications.
Observation:
- A 16-year-old girl with Seckel syndrome presented with symptoms of cerebral ischemia.
- Imaging confirmed Moyamoya syndrome, including cerebral infarcts and stenosis.
- The patient underwent successful surgical revascularization via bilateral pial synangiosis.
Findings:
- This is the first documented case of concurrent Seckel and Moyamoya syndromes.
- Surgical revascularization provided temporary symptom relief.
- An intracranial aneurysm rapidly developed and led to complications.
Implications:
- The findings suggest a potential association between Seckel syndrome and Moyamoya syndrome.
- Physicians should consider Moyamoya syndrome in dwarfism patients exhibiting ischemic symptoms.
- Early diagnosis and surgical intervention for Moyamoya syndrome may improve outcomes in affected individuals.
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