History of the Kleeblattschädel deformity: origin of concepts and evolution of management in the past 50 years

Abstract

<jats:p>The history and evolution of surgical strategies for the treatment of Kleeblattschädel deformity are not well described in the medical literature. Kleeblattschädel anomaly is one of the most formidable of the craniosynostoses, requiring a multidisciplinary team for surgical treatment. The initial descriptions of this cloverleaf deformity and the evolution of surgical treatment are detailed in the present report. Two illustrative cases of Kleeblattschädel deformity, syndromic and nonsyndromic craniosynostoses treated by the senior authors, are also described along with insights into operative strategies.</jats:p>

Cloverleaf Skull Syndrome (Kleeblattschädel Deformity): Findings on Cone Beam Computed Tomography

“…All the clinical manifestations and radiographic findings as noted in our patient were consistent with features described in patients associated with cloverleaf skull syndrome in the literature [2][3][4][6][7][8].…”

“…Despite the unfavorable outcome of many cases, advances in surgical, anesthetic and intensive care techniques have transformed a condition previously considered untreatable into a potentially treatable condition, with acceptable neurological and aesthetic results [7].…”

“…Extreme forms of this condition result in poor neurological outcome and high mortality in infancy [7].…”

Superior Sagittal Sinus Thrombosis as a Treatment Complication of Nonsyndromic Kleeblattschädel

“…5Y7,13 Hydrocephalus is characterized by ventricular dilation and periventricular edema, and its origin is still debated whether it is noncommunicating (basilar impression, aqueductal stenosis, and posterior fossa deformities) or communicating (venous hypertension). 6,13 As stated by Thompson et al,7 the effect of venous hypertension on a ventricular size seems to be related to the state of the cranial sutures, which explains some situations where patients with high venous pressure but with even higher ICP (allowing the absorption of cerebral spinal fluid) have no ventricular enlargement and where, after the sutures release, the ICP drops (no more absorption of the cerebrospinal fluid [CSF] is possible) and the patient develops hydrocephalus. However, we believe that the 2 conditions coexist once, even in treating the noncommunicating causes, the hydrocephalus persists and needs to be treated with a VP shunt.…”

Use of Osteogenesis Distractors in Cloverleaf Skull Reconstruction

“…The neurological sequelae arise from hydrocephalus, hindbrain herniation, and venous hypertension. 8 Surgical correction of the cloverleaf skull has evolved since the first reported treatment in 1972 involving craniotomies along fused suture lines. 9 Subtotal craniectomy (the suggested procedure in Plastic Surgery by Mathes 10 ) has been the treatment of choice even with the expectant poor outcomes.…”