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

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.

Systematic review on the incidence of bisphosphonate related osteonecrosis of the jaw in children diagnosed with osteogenesis imperfecta.

OBJECTIVES: To conduct a systematic review of epidemiological literature to determine the incidence of bisphosphonate related osteonecrosis of the jaw occurring either spontaneously or after dental surgery, in children and adolescents diagnosed with osteogenesis imperfecta. MATERIAL AND METHODS: MEDLINE, HMIC and EMBASE were used to search for English-language articles published from 1946 - 2013. Inclusion criteria consisted of population based studies of children and adolescents (24 years and younger) diagnosed with osteogenesis imperfecta, only studies which included a dental examination, and patients treated with intravenous bisphosphonates were included. Articles were excluded if patients had any other co-morbidity which could affect osteonecrosis of the jaw, and those which treated patients with oral bisphosphonates only. RESULTS: Five studies consisting of four retrospective cohort studies and one case series were identified. Study populations ranged from 15 to 278 patients and number of subjects with osteogenesis imperfecta ranged from 15 to 221. Mean duration of intravenous bisphosphonate use ranged from 4.5 to 6.8 years. All patients were clinically examined and no patients were found to have osteonecrosis of the jaw. CONCLUSIONS: There is no evidence to support hypothesis of causal relationship between bisphosphonates and osteonecrosis of the jaw in children and adolescents with osteogenesis imperfecta. More prospective studies on bisphosphonate use in osteogenesis imperfecta needs to be carried out.