Surgical management of the cloverleaf skull deformity

The cloverleaf skull deformity, or Kleeblattschädel, is a rare malformation caused by synostosis of multiple cranial sutures. This anomaly has been reported to carry a dismal prognosis both in terms of neurological outcome as well as cosmetic appearance if treatment is delayed. Due to the paucity of data concerning the results of early operative intervention, it remains uncertain whether aggressive craniofacial decompressive/reconstructive procedures are effective in ameliorating the effects of the malformation on both neurological development and cosmetic appearance. This paper reports the treatment and outcome of 7 children with the cloverleaf skull malformation treated at our institution between 1981 and 1993. All children underwent an initial decompressive craniectomy with the removal of at least 50% of the cranial vault for relief of high intracranial pressure in early infancy. Our first 4 patients underwent near total calvariectomy whereas the 3 children treated subsequently have undergone a staged approach with anterior followed by posterior craniectomies with bone morcellation and replacement. Subsequent reconstructions, intended to further improve the cosmetic appearance, were performed later in infancy or in early childhood. Follow-up ranges from 17 months to 9 years, with a mean of 61 months. Long-term results are reported with regard to neurological outcome as well as normalization of skull shape in terms of both the cephalic indices and general cosmetic appearance. Of the 4 children initially treated with total calvariectomy, only 1 child is neurologically normal and has a good cosmetic appearance. One child is severely impaired neurologically following a sagittal sinus thrombosis suffered during a secondary reconstructive procedure.(ABSTRACT TRUNCATED AT 250 WORDS)