Pseudomeningocele With Orbital Extension as a Complication of Fronto-Orbital Advancement and Remodeling in Craniosynostosis.

ABSTRACT

AIM: The authors present a series of patients who developed a pseudomeningocele following fronto-orbital advancement and remodeling (FOAR), describing clinical presentation, investigations, and management. Risk factors are identified and preventative strategies suggested. MATERIALS AND METHODS: From 2002 to 2012, all patients who underwent FOAR at our unit were identified. Those who developed a pseudomeningocele were selected and case notes, scan imaging and photographs were reviewed. RESULTS: Two hundred thirty-six FOAR operations were carried out over 12 consecutive years. Sixty-one of these patients were syndromic. A pseudomeningocele occurred in 6 patients. All affected cases were syndromic. Clinical features of presentation with orbital pseudomeningocele included orbital swelling, ptosis, proptosis, and/or hypoglobus. Raised intracranial pressure (ICP) was managed before pseudomeningocele repair in 2 patients, at the time of pseudomeningocele repair using an extra-ventricular drain (EVD) or lumbar drain in 4 patients. Decompression of the pseudomeningocele with excision and duraplsty was carried out in 5 patients, 1 patient required excision of gliotic brain and obliteration of dead space. Four patients had a calvarial graft to manage the bony defect and a further 2 had a titanium mesh. None of the patients had a recurrence of the pseudomeningocele or any long-term ocular or aesthetic complications. CONCLUSION: Pseudomeningocele has not previously been described in FOAR, but in a large series of consecutive patients, we have identified a 2.5% incidence. This incidence increases to 10% in the syndromic population of patients undergoing FOAR. The risk factors include a diagnosis of syndromic craniosynostosis, dural tear, hydrocephalus or raised ICP, infection, persistent cerebrospinal fluid (CSF) leak, or presence of dead space. Preventative strategies include CSF management before or post-FOAR. The ultimate treatment of the pseudomeningocele and growing fracture involves surgical decompression of the collection, a duraplasty, reconstruction of the orbital roof, and temporary CSF diversion.