craniosynostoses/edema

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Adverse facial edema associated with off-label use of recombinant human bone morphogenetic protein-2 in cranial reconstruction for craniosynostosis. Case report.

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The authors present a case of scalp and facial edema following craniofacial reconstruction for metopic craniosynostosis in which recombinant human bone morphogenetic protein-2 (rhBMP-2) was used to treat cranial defects related to the frontoorbital reconstruction. The extent of swelling, the onset,

The association of Alagille syndrome and craniosynostosis.

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Alagille syndrome is associated with various ocular abnormalities, including pseudopapilledema or optic disk edema due to increased intracranial pressure. Several mechanisms have been proposed to explain the mechanism of intracranial hypertension in Alagille syndrome. Craniosynostosis is an unusual

Subgaleal Drain Placement Improves Surgical Outcomes After Primary Cranioplasty in Craniosynostosis Patients.

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BACKGROUND There is no published data addressing the use of postoperative subgaleal drains in patients undergoing primary cranioplasty for craniosynostosis. We conducted a retrospective chart review in this population of patients, comparing outcomes of those who received postoperative drains with

Severe craniosynostosis with Noonan syndrome phenotype associated with SHOC2 mutation: clinical evidence of crosslink between FGFR and RAS signaling pathways.

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Dysregulation in the RAS signaling cascade results in a family of malformation syndromes called RASopathies. Meanwhile, alterations in FGFR signaling cascade are responsible for various syndromic forms of craniosynostosis. In general, the phenotypic spectra of RASopathies and craniosynostosis

Mini-invasive surgical technique for sagittal craniosynostosis.

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BACKGROUND Several techniques are currently available for the surgical correction of sagittal craniosynostosis. The most recently introduced ones have been specifically designed to perform a mini-invasive approach in order to reduce the postoperative morbidity. Herein, the surgical steps of a

Sudden death associated with syndromic craniosynostosis.

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In this paper we report the autopsy findings of a 7 year old girl who presented with headache, nausea and repeated vomiting and died unexpectedly at home. She had no previous history of major illnesses and no history of epileptic seizures. External examination revealed ocular abnormalities. Internal

Case report of optic atrophy in pansynostosis: an unusual presentation of scalp edema from hair braiding.

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Pansynostosis (fusion of all cranial sutures) and optic atrophy were found as incidental CT scan and ophthalmological findings in an 8-year-old who presented to the emergency room with scalp edema from tight 'cornrow' hair braiding. Cranial vault expansion was successfully performed.

Diagnosis, management and outcome in 9 children with unilateral posterior synostotic plagiocephaly.

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Posterior synostotic plagiocephaly (PSP) represents a very rare challenging type of craniosynostosis, often misdiagnosed as a simple posterior positional plagiocephaly (PPP). Under-diagnosed forms may result in delayed diagnosis and neurosurgical management, with potential

Cerebral salt wasting syndrome after calvarial remodeling in craniosynostosis.

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Hyponatremia and increased urine output after calvarial remodeling have been noted in pediatric patients with craniosynostosis. If not treated properly, patients develop hypoosmotic conditions that can lead to cerebral edema, increased intracranial pressure, and collapsed circulation. Postoperative

Two cases of cerebral salt wasting syndrome developing after cranial vault remodeling in craniosynostosis children.

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Hyponatremia has been recognized as an important postoperative metabolic complication after central nervous system (CNS) operations in children. If not appropriately treated, the postoperative hyponatremia can cause several types of CNS and circulatory disorders such as cerebral edema, increased

Does perioperative steroid use improve clinical outcomes in open repair of craniosynostosis?

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BACKGROUND The benefits of routine perioperative steroid use to decrease facial edema, ecchymosis, pain, and reduced length of hospitalization have been reported for many procedures. The role of perioperative steroids after open craniosynostosis surgery remains understudied. The purpose of our study

Ophthalmic features of craniosynostosis.

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With the possible exception of aesthetic considerations, the visual system is more involved in craniosynostosis than any other. Changes may be an inherent feature of the pathologic process or occur as a secondary complication. Several of these are potentially devastating, resulting in substantial

Bilambdoid and sagittal synostosis: Report of 39 cases.

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UNASSIGNED Bilambdoid and sagittal synostosis (BLSS), also called "Mercedes Benz synostosis," is a multisutural craniosynostosis that has been described as a specific entity. However, this synostotic pattern can also be found in syndromic craniostenosis. To better define this entity we reviewed our

[Surgical correction of 34 patients with hypertelorism].

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In 34 patients with hypertelorism aged on the average 12.6 years, 24 underwent intracranial surgery (combined intra- and extracranial approach), 2 (moderate) U-osteotomy (subcranial approach), and 8 (mild) canthoplasties. Improved results were obtained in the patients with various types of

White matter abnormality in Jacobsen syndrome assessed by serial MRI

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Introduction: Jacobsen syndrome (JS) is caused by a deletion at the terminus of the long arm of chromosome 11. There are few reports of JS associated with cerebral white matter abnormalities (WMA), and the etiology, pathophysiology, and time-dependent changes in

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