Use of Osteogenesis Distractors in Cloverleaf Skull Reconstruction

Abstract

Cloverleaf skull deformity (Kleeblattschädel-Syndromen, trilobular skulls) results from synostosis of multiple cranial sutures. The number of sutures involved, the pathogenesis of the synostosis, and the associated anomalies and syndromes are variable. All forms of cloverleaf skull are associated with a high morbidity and mortality. Management of surviving infants requires multiple decompressive and reconstructive operative procedures. Maximal advancement of the forehead/brow at the initial surgery is usually not enough to correct the associated proptosis and a second brow advancement must be done. We present a patient with Apert syndrome and cloverleaf skull deformity that required early (1 month old) cranial vault decompression due to severe proptosis and papilledema. Our management included the placement of osteogenesis distracters on the forehead/brow to gain additional advancement and expand the soft tissue.

Superior Sagittal Sinus Thrombosis as a Treatment Complication of Nonsyndromic Kleeblattschädel

“…15 If symptoms of increased ICP appear early, a VP shunt or an endoscopic third ventriculostomy (ETV) must be considered. 4,16 We and other authors 7 believe that the placement of a shunt in these patients merely redistributes the balance of the CSF without dealing with the underlying anatomic abnormalities, so its use is justified merely to gain time until a more definitive surgery can be performed.…”

Early Posterior Vault Distraction Osteogenesis for the Treatment of Syndromic Craniosynostosis

“…In 2009, White et al published the first case series reporting the results of PCVD for the treatment of increased intracranial pressure in children with syndromic, multisuture craniosynostosis 1. Since that time, PCVD has gained popularity as the initial procedure in this patient population to increase intracranial volume and improve the aesthetic appearance of the posterior cranium 2345678. The ability to treat intracranial hypertension, which 35 to 80% of children with syndromic craniosynostosis will develop, has also made PCVD valuable surgical option 10…”

“…While posterior vault distraction has been shown to adequately treat increased intracranial pressure by significantly improving intracranial volume, there was initial concern about performing this operation in children younger than 6 months secondary to lack of adequate bone thickness for placement and retention of the distractor devices 2. However, more recent studies have shown that distraction can be performed adequately, without significant increase in device failure, on patients younger than 6 months 456…”

“…This technique has been shown to increase intracranial volume, treat intracranial hypertension, and improve the aesthetic contour of the posterior cranium 1234567. When compared with conventional cranial vault remodeling, distraction osteogenesis can provide a greater degree of expansion, partially due to the ability of the technique to progressively stretch the soft-tissue envelope 4.…”

Distraction Osteogenesis Technique for the Treatment of Nonsyndromic Sagittal Synostosis

“…Since calvarial distraction for craniosynostosis was first described by Sugawara et al 13 in 1998, it has been gaining popularity for treatment of syndromic and multiple suture synostosis. 11 , 12 , 14 – 17 Few studies have addressed its usefulness in the management of patients with single-suture synostosis. 18 , 19 Techniques employing distraction osteogenesis have shown several advantages over vault reconstruction.…”