SURGICAL-MANAGEMENT OF THE CLOVERLEAF SKULL DEFORMITY

The cloverleaf skull deformity, or Kleeblattschadel, is a rare malform ation caused by synostosis of multiple cranial sutures. This anomaly h as beenreported to carry a dismal prognosis both in terms of neurologi cal outcome as well as cosmetic appearance if treatment is delayed. Du e to the paucity of data concerning the results of early operative int ervention, it remains uncertain whether aggressive craniofacial decomp ressive/reconstructive procedures are effective in ameliorating the ef fects of the malformation on both neurological development and cosmeti c appearance. This paper reports the treatment and outcome of 7 childr en with the cloverleaf skull malformation treated at our institution b etween 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 pat ients underwent near total calvariectomy whereas the 3 children treate d subsequently have undergone a staged approach with anterior followed by posterior craniectomies with bone morcellation and replacement. Su bsequent reconstructions, intended to further improve the cosmetic app earance, 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-t erm results are reported with regard to neurological outcome as well a s normalization of skull shape in terms of both the cephalic indices a nd general cosmetic appearance. Of the 4 children initially treated wi th total calvariectomy, only 1 child is neurologically normal and has a good cosmetic appearance. One child is severely impaired neurologica lly following a sagittal sinus thrombosis suffered during a secondary reconstructive procedure. Two children have died, both due to complica tions resulting from later surgical revision. In contrast, the 3 child ren who underwent staged anterior followed by posterior procedures are all alive and are without significant neurological deficits. We concl ude the following. (1) The cloverleaf skull malformation does not in i tself portend a poor outcome. (2) Early and aggressive decompressive p rocedures are necessary to ameliorate the effects of high intracranial pressure on the neurological development of these children. (3) A sta ged anterior followed by posterior craniectomy appears to result in lo wer long-term morbidity. (4) Multiple procedures are often indicated i n order to achieve a good cosmetic result. (5) Operative morbidity and mortality rise with repeated surgery.