Normocephalic Pancraniosynostosis Resulting in Late Presentation of Elevated Intracranial Pressures

Foo, Roy; Whitáker, Linton A.; Bartlett, Scott P.

doi:10.1097/prs.0b013e3181d62b48

Cited by 23 publications

(33 citation statements)

References 15 publications

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“…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.…”

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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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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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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“…A recently published series of 4 patients with normocephalic pancraniosynostosis had an average age at presentation of 3.5 years. 1 Our cases had delayed presentation at 18 and 24 months of age, respectively. Although our patients' presentations were not as delayed as those presented by Foo et al, 1 their presentations were considerably later than those typically seen with other types of craniosynostosis.…”

Section: Discussionmentioning

confidence: 86%

“…1 The late presentation associated with normocephalic pancraniosynostosis is likely a result of the absence of significant cranial dysmorphology leading to an initial low index of suspicion for craniosynostosis. A recently published series of 4 patients with normocephalic pancraniosynostosis had an average age at presentation of 3.5 years.…”

Section: Discussionmentioning

confidence: 99%

Normocephalic Pancraniosynostosis

Killion

McEwan

Muzaffar

2013

Journal of Craniofacial Surgery

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“…4Y6 Besides the cosmetic facial deformity, severe problems can arise with the associated micromyelia, high intracranial pressures, proptosis, papilledema, hydrocephalus, hindbrain herniation, and skull base dysplasias. 7 Those who survive early infancy have a high incidence of neurological impairment that can continue throughout life. The neurological sequelae arise from hydrocephalus, hindbrain herniation, and venous hypertension.…”

Section: Discussionmentioning

confidence: 99%

Use of Osteogenesis Distractors in Cloverleaf Skull Reconstruction

Sargent

Griner

2013

Annals of Plastic Surgery

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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.

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Normocephalic Pancraniosynostosis Resulting in Late Presentation of Elevated Intracranial Pressures

Abstract: Patients with normocephalic pancraniosynostosis have an insidious clinical course. Because of their indistinct cranial morphology, they present late with significant symptoms of elevated intracranial pressure requiring urgent surgical decompression.

Cited by 23 publications

References 15 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

Normocephalic Pancraniosynostosis

Use of Osteogenesis Distractors in Cloverleaf Skull Reconstruction

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