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Surgical decompression for symptomatic Chiari II malformation in neonates with myelomeningocele
W P Vandertop1, A Asai, H J Hoffman
1Division of Neurosurgery, Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Early decompression surgery for Chiari II malformation in infants significantly improves outcomes. Prompt surgical intervention leads to complete neurological recovery in most cases, highlighting the role of compressive forces.
Area of Science:
- Pediatric Neurosurgery
- Neurology
- Developmental Biology
Background:
- Chiari II malformation is a congenital condition affecting the cerebellum and brainstem.
- Infants present with diverse neurological symptoms, including swallowing difficulties and apneic spells.
- The exact etiology, particularly the role of compressive forces versus intrinsic brainstem abnormalities, remains debated.
Purpose of the Study:
- To evaluate the clinical presentation and outcomes of infants with symptomatic Chiari II malformation.
- To assess the efficacy of early decompressive surgery in promoting neurological recovery.
- To investigate the etiological role of compressive forces in symptomatic Chiari II malformation.
Main Methods:
- Retrospective analysis of 17 infants under 1 month of age admitted with Chiari II malformation signs and symptoms between 1981 and 1991.
- All patients underwent decompressive laminectomy.
- Clinical outcomes and follow-up data were collected and analyzed.
Main Results:
- Common symptoms included swallowing difficulty (71%), stridor (59%), and arm weakness (53%).
- Fifteen of 17 patients (88%) survived with complete recovery after surgery, with a mean follow-up of 65 months.
- Two patients died, one from respiratory arrest and another from shunt-related complications.
Conclusions:
- Compressive forces play a critical etiological role in symptomatic Chiari II malformation.
- Early recognition and immediate decompressive laminectomy are crucial for prompt and full neurological recovery in affected infants.
- Surgical decompression is an effective treatment for improving outcomes in infants with Chiari II malformation.
Abstract:
Between January, 1981, and July, 1991, 17 infants under 1 month of age were admitted to The Hospital for Sick Children with the signs and symptoms of a Chiari II malformation. These patients' presentation included swallowing difficulty (71%), stridor (59%), apneic spells (29%), aspiration (12%), weakness of cry (18%), and arm weakness (53%). Decompression of the Chiari II malformation was performed in all patients, with a time interval between onset of symptoms and surgery ranging from 1 to 121 days. Fifteen patients (88%) remain alive, all of whom have shown a complete recovery. The mean follow-up period in this group of patients was 65 months. Two patients died, one due to respiratory arrest 8 months after decompression and the other because of shunt infection and peritonitis 7 years after decompression. These results support the concept that compressive forces, rather than a primary intrinsic disorder of the brain-stem nuclei, play a crucial etiological role in the development of a symptomatic Chiari II malformation. Early recognition of the symptoms of Chiari II malformation should be followed by immediate decompressive laminectomy in order to promote a prompt and full neurological recovery.