Fibrous Osteodysplasia of Bones of the Skull Base and Fornix in Children: Surgical Treatment

L.A. Satanin, V.V. Roginsky, M.V. Ananov, S.K. Gorelyshev, A.G. Korshunov, V.I. Ozerova, V.A. Cherekaev, M.S. Zubairaev, L.A. Lazareva

(Burdenko Research Institute of Neurosurgery, Moscow;
The Moscow Center of Children’s Maxillofacial Surgery (Central Research Institute of Stomatology-Children’s Municipal Clinical Hospital of Saint Vladimir), Moscow)

A problem of surgical treatment of fibrous osteodysplasia consists in closeness of functionally eloquent structures, extensive spread of pathologic bone tissue and necessity of osteoplasty and correction of cranial deformities.

There were 69 children, aged 1-15 with fibrous osteodysplasia of bones of the skull base and fornix, who were operated in the Burdenko Research Institute of Neurosurgery and the Moscow Centre of Children’s Maxillofacial Surgery (Central Research Institute of Stomatology-Children’s Municipal Clinical Hospital of Saint Vladimir) in 1985-2001. Mono- and polyossesous forms of the disease were watched in 35% and 65% of cases respectively. Albright’s syndrome was revealed in 2 cases. There were pain syndrome (30% of cases), craniofacial deformities (85%), failing sight of a different degree (9%), diminished hearing (5%), development of secondary craniofacial deformities (34%).

All the cases were operated for removal of pathologic foci. Total, subtotal and partial removal was performed in 46%, 44% and 10% respectively. Defects of the skull base were eliminated with the help of plasty by soft tissues (free grafts of fatty tissue, a periosteal-aponeurotic graft, a pedicle graft of a temporal muscle, etc.). Elimination of bone defects was carried out, using a split autobone of a skull fornix, polymeric substances (palakos, codubix, protacryl, etc.), titanium devices. Decompression of an optic nerve canal was performed in 1 case. When lesions spread on a facial skeleton, surgical treatment was characterized by two stages.

Correction of secondary craniofacial deformities was carried out simultaneously or during a repeated operation. The orbit hypertelorism and its vertical dystopia were treated, using surgical methods, which had been developed at the Institute.

Postoperative complications included nasal liquorrhea, seroma, diplopia, failing sight, autograft rejection (1 case).

A follow-up period varied from 1 up to 16 years. As for the group with polyosseous fibrous osteodysplasia, partial removal of pathologic tissue resulted in a prolonged growth in 32% of cases, including those with Albright’s syndrome. There were no relapses or prolonged growth in the majority of patients.

Despite evident advantage of plasty with a split autobone, one could watch its resorption and development of a cosmetic defect in an operation area 1-4 years later. It demanded reoperation. There were no complications in cases with plasty by polymeric materials.

The obtained results show, that extensive fibrous osteodysplasia of bones of the skull base and fornix can be treated by maximum possible removal of affected tissue. Plasty with a periosteal-aponeurotic graft is effective in extensive defects of the skull base bones, as it allows to prevent liquorrhea. Use of polymeric materials is optimum for elimination of extensive defects of the skull fornix bones.