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Diagnosis, Management, and Outcome in 9 Children with Unilateral Posterior Synostotic Plagiocephaly
Hilal Abboud1, Loubna Rifi1, Adyl Melhaoui1
1Neuropediatric Unit, Neurosurgery Department, Mohammed V University Hospital, Rabat, Morocco.
Insights
Posterior synostotic plagiocephaly, a rare craniosynostosis, often presents with male predominance and right-sided lambdoid suture issues. Early diagnosis and neurosurgery can prevent long-term cognitive and aesthetic problems.
Area of Science:
- Pediatric Neurosurgery
- Craniofacial Surgery
- Developmental Pediatrics
Background:
- Posterior synostotic plagiocephaly is a rare craniosynostosis type, frequently misdiagnosed as positional plagiocephaly.
- Delayed diagnosis can lead to significant ophthalmologic, cognitive, and aesthetic complications in children.
Purpose of the Study:
- To analyze clinical data and outcomes of infants with posterior synostotic plagiocephaly.
- To highlight diagnostic challenges and the importance of timely neurosurgical intervention.
Main Methods:
- Retrospective analysis of 9 infants with posterior synostotic plagiocephaly treated between 2000-2009.
- Inclusion of clinical presentation, CT scan findings, ophthalmoscopic examination, and surgical outcomes.
Main Results:
- Patients averaged 10 months old with a male predominance (8:1).
- Right-sided lambdoid suture synostosis was common (77.77%), with occipitomastoid bulge and occipital flattening in all cases.
- Ophthalmoscopic findings included papilledema in 6 cases; all patients underwent successful neurosurgical correction with full ophthalmic recovery and satisfactory aesthetic results.
Conclusions:
- Male predominance and right-sided lambdoid suture involvement are notable findings.
- Early and accurate diagnosis coupled with appropriate neurosurgical treatment is crucial to prevent neurocognitive and aesthetic sequelae.
Background:
Posterior synostotic plagiocephaly represents a rare challenging type of craniosynostosis, often misdiagnosed as a simple posterior positional plagiocephaly. Underdiagnosed forms may result in delayed diagnosis and neurosurgical management, with potential ophthalmologic, cognitive, and aesthetic sequelae in children.
Methods:
Here we retrospectively analyzed data of 9 posterior synostotic plagiocephaly infants treated in our center over a 10-year period (January 2000-December 2009).
Results:
Patients averaged 10 months of age, and there was a clear male predominance (8 males/1 female). Cerebral computed tomography scan was performed in all patients, and the abnormal suture was located at the right side in 7 cases (77.77%). Ipsilateral occipitomastoid bulge and occipital flattening were found in all cases (100%). Posterior ear displacement was found in 7 cases (77.77%), and there was minimal facial asymmetry in 4 cases (44.44%). The ophthalmoscopic examination found a papillary edema grade 1 in 2 cases and grade 2 in 4 cases. There were no deaths or reoperation in our series. All of our patients underwent a neurosurgical correction, with total ophthalmic recovery in all patients with preoperative papillae edema. Eight patients had an early satisfactory aesthetic aspect. Long-term follow-up in treated children found a normal childhood, with no major school dysfunctions and normal social integration.
Conclusions:
Interesting findings in our series were male gender predominance and predilection of the right-side synostotic lambdoid suture. We think that early correct diagnosis and appropriate neurosurgical treatment may prevent potential complications such as neurocognitive and aesthetic sequelae in children with posterior synostotic plagiocephaly.
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