Cloverleaf skull

Arseni, C; Horvath, Lenke; Ciurea, V

doi:10.1007/bf01401883

Cited by 13 publications

(8 citation statements)

References 3 publications

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“…In 1972 Arseni et al 2 first described surgical treatment in the form of linear craniotomies along the coronal, lambdoid, and temporoparietal sutures in 2 stages at intervals of 4 and 7 weeks. These patients had survived to 6 years and 3 months of age because of a milder form of hydrocephalus.…”

Section: Surgical Strategies In Kleeblattschädel: Evolution In the Pamentioning

confidence: 99%

“…1 The condition is further characterized by cosmetic facial deformity and micromyelia and can be further complicated by increased intracranial pressure, hydrocephalus, hindbrain herniation, skull base dysplasias, and impaired neurological function. 2,10,28,38 The etiology of Kleeblattschädel syndrome is unknown; it has been attributed to abnormalities of both the calvaria and the skull base, making it one of the most complex craniosynostoses to treat. The Kleeblattschädel anomaly has been reported to occur in patients with both syndromic and nonsyndromic forms of craniosynostosis.…”

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confidence: 99%

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

Manjila¹,

Chim²,

Eisele

et al. 2010

FOC

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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.

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Section: Surgical Strategies In Kleeblattschädel: Evolution In the Pamentioning

confidence: 99%

mentioning

confidence: 99%

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

Manjila¹,

Chim²,

Eisele

et al. 2010

FOC

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“…Two children were reported to have a good cosmetic outcome and 1 child was described as having an acceptable cosmetic result. Neurological out come data and cosmetic results from several smaller series are variably reported (see table 1) [3,4,12], Although sev eral case reports provide excellent follow-up data on sin gle patients, details regarding the timing of surgery, the nature of the initial procedures performed, and the fol low-up periods vary considerably, precluding a compari son of treatment modalities [6,8,11].…”

Section: Discussionmentioning

confidence: 99%

“…The natural history of nonsurgically treated children with the cloverleaf deformity has been well described. These children have a grim prognosis regarding survival past infancy, and those who do survive into childhood almost invariably suffer severe neurologic impairment [1,[3][4][5]9], The first attempt at surgical correction of the deformity was reported by Angle et al [3] in 1967. Subse quently, Arseni et al [4], Zuleta et al [12], Rohatgi [10] and Lodge et al [7] each reported small series of children with the cloverleaf deformity who had undergone some attempt at surgical correction.…”

Section: Introductionmentioning

confidence: 99%

“…These children have a grim prognosis regarding survival past infancy, and those who do survive into childhood almost invariably suffer severe neurologic impairment [1,[3][4][5]9], The first attempt at surgical correction of the deformity was reported by Angle et al [3] in 1967. Subse quently, Arseni et al [4], Zuleta et al [12], Rohatgi [10] and Lodge et al [7] each reported small series of children with the cloverleaf deformity who had undergone some attempt at surgical correction. In addition, there have been anecdotal reports which describe variable correction of the cosmetic deformity and preservation of neurologi cal function in children who underwent decompressive surgery in early childhood [6,8,11], However, the lack of a consistent surgical philosophy as well as the paucity of information regarding postoperative neurological func tion and cosmetic outcome has hampered attempts to determine the efficacy of aggressive surgical intervention in the management of this complex craniofacial disorder.…”

Section: Introductionmentioning

confidence: 99%

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Surgical Management of the Cloverleaf Skull Deformity

Resnick

Pollack²,

Albright³

1995

Pediatr Neurosurg

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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. Two children have died, both due to complications resulting from later surgical revision. In contrast, the 3 children who underwent staged anterior followed by posterior procedures are all alive and are without significant neurological deficits. We conclude the following. (1) The cloverleaf skull malformation does not in itself portend a poor outcome. (2) Early and aggressive decompressive procedures are necessary to ameliorate the effects of high intracranial pressure on the neurological development of these children. (3) A staged anterior followed by posterior craniectomy appears to result in lower long-term morbidity. (4) Multiple procedures are often indicated in order to achieve a good cosmetic result. (5) Operative morbidity and mortality rise with repeated surgery.

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The relationship of cloverleaf skull syndrome to hydrocephalus

et al. 1991

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In 1960, Holtermüller and Wiedemann described chondrodystrophic hydrocephalus as Kleeblattschädel syndrome. In the present paper, we review 23 cases of this syndrome associated with hydrocephalus, including 2 cases of our own. Of these 23 cases, 8 involved communicating hydrocephalus and 15 non-communicating hydrocephalus. It has been speculated that the associated hydrocephalus results from basilar impression, compression of infratentorial structures, aqueductal stenosis in non-communicating hydrocephalus, and impairment of venous drainage and cerebral spinal fluid flow at the level of the constrictive cranial ring in communicating hydrocephalus. Our present cases also showed this cranial ring, as demonstrated by skull X-rays and computed tomography scans. Since cloverleaf skull syndrome often has other general anomalies, we consider the hydrocephalus in this syndrome to have causes associated with the osteocartilaginous system.

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Cloverleaf skull

Cited by 13 publications

References 3 publications

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

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

Surgical Management of the Cloverleaf Skull Deformity

The relationship of cloverleaf skull syndrome to hydrocephalus

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