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At least 55 records · Page 3Linked to original sources
PubMed1989

[Craniosynostoses in Egypt: clinical forms and treatment methods. A study of 55 surgically treated patients].

The relative incidence of the various craniosynostosis deformities in Egypt is given on a total of 55 patients who were operated upon. The most common deformity was oxycephaly, 25 cases (i.e. 45%). Various surgical methods have been applied depending on the deformity present. In isolated scaphocephalic deformities (9 cases), 3 pairs of strip craniectomies also called "the triple strip" were sufficient and gave satisfactory results. For other types of deformities, an attempt was made to improve the aesthetic results following simple forehead advancement, which was done for the first five cases. A newly suggested combined procedure of multiple craniectomies and forehead advancement was applied on 41 patients. This combined "three flap procedure" has been previously presented by the authors (Gheita and Assaad, 1987). In this study it has been further modified so as to be suitable for infantile as well as adult deformities, thus defining them as type I and type II variations. The principle of the technique consists of combining the forehead advancement flap with two pairs of strip craniectomies: a pair of para-sagittal ones and another pair of spheno-temporal ones going to the cranial base as far as the edge of the foramen ovale. This is known as type I. In the type II variety the median strip of bone overlying the sagittal sinus is broken posteriorly so as to lower the vault of the skull whenever necessary, and the spheno-temporal craniectomy is not carried as far downward to the foramen ovale but stops at the level of the zygomatic arch. The details of these techniques are demonstrated.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1977

[Craniosynostoses (17 years of experience with a new surgical technic)].

In the treatment of craniosynostosis methods have been proposed to prevent craniectomies from early reclosure. New bone is formed at the edge of the craniectomy but particularly at the outer surface of the dura. The author has developed a method to separate the edges from each other and to stop osteogenesis inside the craniectomy. The outer layer of the dura is dissected free from the inner layer, folded over the edge of the groove and sutured to the outer periosteum. The operative technique and the results in 40 patients with a follow up of up to 17 years are described.

Child↗
PubMed1984

[Craniosynostoses: importance of the measurement of intracranial pressure and the application of J Jane's surgical technic].

We report the studies, treatment and follow-up of two children with the diagnosis of craniosynostosis for early closure of sagittal and coronal sutures. We paid special attention to the intracranial pressure (ICP) monitoring. Prognosis of the mental functions (Gessell's test) is related with findings of ICP and surgical treatment. We have performed as neurosurgical approach, a modification of the Jane's technic. The advantages of this technic and our results are discussed.

Craniosynostoses↗
PubMed1994

[Remodeling the cranial vault in anterior craniosynostoses. Our therapeutic experience of trigonocephaly and plagiocephaly].

The authors propose two simple techniques to improve the adaptation of the frontal vault after remodeling the orbito-naso-frontal flap in anterior craniostenosis. In trigonocephaly: the metopic suturectomy is enlarged by lateral translation of the two half frontal bones, then to fall the place the coronal suture is used. The temporal plates are moved forward and turned to fill the failure of fronto-temporal junction due to valgisation of orbito-naso-frontal band. In plagiocephaly: the frontal flattened flap is cut with oblique radial osteotomies starting at bregma so called "Daisy petal". The first internal petal is sacrificed. The others are rotated axially in a medial direction. Internal table corticotomies must be performed at two levels to recurve the flattening and to obtain a curve in the coronal plane. In this two techniques the pericranium and dura-mater must be carefully teated to obtain a better adaptation of the new bone shape and to permit the spontaneous reossification.

Craniosynostoses↗
PubMed1976

[Value of the intracranial pressue measurement in the craniosynostoses detected after the age of 1 year].

Notwithstanding the fact that there is a general agreement on the necessity of surgery in the first year of life in craniosynostosis, the problem is more difficult for older children. Deterioration of clinical status is, in general, in relation with an increase in intracranial pressure (ICP), which may happen abruptly following different causes (slight head injury, for example). The purpose of this study was to determine, in different varieties of synostosis, if a high intracranial pressure could exist without clinical signs and consequently, if a surgical opening of the sutures would be necessary. IPC has been measured through a ventricular catheter connected with a transducer and recorder for 24 hours. In half of the cases (11 out of 22) a high ICP (above 20 mmHg) was recorded either permanently or during sleep. This increase in ICP should lead to a surgical decompression, event without clinical signs. On the other hand, psychomotor retardation, abnormal EEG, increased digitation should not be considered as an indication for surgical treatment in cases with normal ICP. The measurement of decreased ICP after operation on a long term basis would be a great value.

Adolescent↗
PubMed1998

Description of a new mutation and characterization of FGFR1, FGFR2, and FGFR3 mutations among Brazilian patients with syndromic craniosynostoses.

Dominant mutations in three fibroblast growth factor receptor genes (FGFRs1-3) cause Crouzon, Jackson-Weiss, Pfeiffer, and Apert syndromes. In the present study, 50 Brazilian patients with these four syndromes (27 Apert, 17 Crouzon, 5 Pfeiffer, and 1 Jackson-Weiss patients) were screened for mutations in the FGFR1-3 genes. Except for one, all the Apert patients had either S252W (n = 16) or P253R (n = 10) mutations. The remaining Apert case is atypical with a mutation altering the splice site of FGFR2 exon IIIc. The Pfeiffer patients had mutations in one of the FGFR genes: three in FGFR2, one in FGFR1, and one in FGFR3. In contrast, only 8 of the 17 Crouzon patients studied had a mutation in either FGFR2 (n = 7) or FGFR3 locus (n = 1). Mutations in the FGFR2 locus account for most (93%) of our syndromic craniosynostotic cases, whereas 5% had mutations in the FGFR3 locus and only 2% had mutations in the FGFR1 gene. Except for one, all the other mutations were reported previously in craniosynostotic patients from other populations. Interestingly, the mutation C278F, previously described in Crouzon and Pfeiffer cases, was here identified in a familial case with Jackson-Weiss. Also, unexpectedly, a common mutation altering the splice site of the FGFR2 exon IIIc was found in one Apert and two Pfeiffer patients. In addition, we identified a new mutation (A337P) in the FGFR2 exon IIIc associated with Crouzon phenotype.

Acrocephalosyndactylia↗