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At least 19 recordsLinked to original sources
PubMed1989

[Diagnosis and treatment of craniosynostoses: the usefulness of CT combined with 3-dimensional reconstruction].

Craniosynostoses are craniocerebral and craniofacial dysmorphic states, characterized by early closure of one or more cranial sutures. In this pathology the perpendicular growth of the bone to the involved suture is disturbed (Virchow's theory). Craniosynostoses can be divided into: 1) single-suture synostoses (scaphocephaly, plagiocephaly, trigonocephaly), 2) multiple-suture synostoses (brachycephaly, oxycephaly) and 3) craniofacial dysostoses. From 1976 to 1987, 63 children with craniosynostosis were studied in our Institute. All patients underwent radiologic and neuroradiologic research with CT scans both before and after surgery; in the past year three-dimensional reconstructions of CT images were also employed. Major advantages of 3-DCT have been obtained in craniofacial malformations. In fact, this technique helps reduce surgical risks and allows the surgeon to evaluate partial results and to make eventual corrections in the last phase. The processing of images is useful to simulate the operation, thus allowing the surgeon to take the best therapeutic choice by computer. This technique is especially useful for postoperative follow-up. In craniosynostoses, early surgical treatment (within the first 6-8 months of life) is necessary in order to obtain excellent functional and cosmetic results.

Cranial Sutures↗
PubMed1975

Remarks on the surgical treatment of the craniosynostoses.

Technical aspects and results of a surgical procedure for craniosynostoses are exposed. Fragmentation-recomposition of the anterior part of the cranial vault in cases of brachi-, acro-, turri- and trigono-cephaly gives both an effective decompression and a satisfactory cosmetic result.

Adolescent↗
PubMed1991

Pitfalls in counselling: the craniosynostoses.

We describe three families to highlight the variability of expression and penetrance that can occur in the craniosynostoses. In two of the families, gene carriers were only identified in retrospect by looking at photographs of other family members. In the third family, identical twins were initially thought to be discordant for sagittal craniosynostosis until early skull x rays were examined and both were found to be affected. The dilemmas faced when counselling these families are discussed.

Adult↗
PubMed1992

Aggressive surgical management of sleep apnea syndrome in the syndromal craniosynostoses.

Obstructive sleep apnea syndrome (OSAS) frequently develops in patients with craniosynostosis and associated midfacial stenosis. In the past, conservative measures or tracheostomy have been used to manage this condition. Although the course of OSAS in these patients is multifactorial, a major factor is the marrow nasopharyngeal space. Aggressive surgical intervention to enlarge the nasopharyngeal space can reduce the severity of OSAS and therefore avoid the need for tracheostomy. Surgical approaches include adenotonsillectomy, uvulopalatopharyngoplasty, and midface advancement.

Child, Preschool↗
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↗
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↗
PubMed1978

Normal intelligence in two children with Carpenter syndrome.

Previous reports have noted a constant association between the Carpenter syndrome (acrocephalopolysyndactyly, type II) and mental retardation. We report two patients with this condition with normal intelligence. These observations indicate that mental deficiency is not necessarily a component of the Carpenter syndrome and that early surgical correction of the craniosynostoses may improve the chances of normal mentality.

Acrocephalosyndactylia↗
PubMed1975

A girl with karyotype 46,XX,del(7)(qter-p 15:).

A girl with partial deletion of the short arms of one chromosome 7 is described. Among many other symptoms she has craniosynostoses. Early closure of cranio-sutures has previously been described in 2 of 3 patients with partial deletion 7. Investigation of a number of genetic marker systems shows that the HL-A, MN, AcP, and GPT loci are not located in the deleted segment.

Adult↗
PubMed1991

Advancement-onlay: an improved technique of fronto-orbital remodeling in craniosynostosis.

Eighteen patients with nonsyndromic craniosynostosis underwent fronto-orbital remodeling with an advancement-onlay technique. The mean age of the infants was 5 months (range = 2-11 months) when the procedure was performed for the following indications: unilateral coronal synostosis (n = 10); bilateral coronal synostosis (n = 3); metopic synostosis (n = 2); and multiple craniosynostoses (n = 3). The technique consists of (1) unilateral or bifrontal craniotomy, (2) superior orbital rim recontouring and advancement, and (3) frontal bone graft rotation and onlay. Posteriorly, the frontal bone graft is left "floating," while anteriorly, rigid fixation with microplates and screws has supplanted wire osteosynthesis. The use of rigid fixation prevents uncontrolled "float" of the forehead and eliminates the need for temporal struts. Follow-up time ranged from 6 to 60 months (mean = 2.6 years). There were no serious postoperative complications. Surgical results were good to excellent in 94% of cases and poor to fair in 6%. Only 1 patient with a Kleeblattschädel deformity required major revision, while another patient with trigonocephaly underwent a minor, extracranial recontouring procedure. Supraorbital rim and/or forehead recession suggestive of relapse or initial inadequacy of anterior projection occurred in 3 patients (17%). Residual, mild contour abnormalities of the forehead and/or temporal regions were found in 5 cases. To date, no gross disturbances in craniofacial growth related to our method of rigid fixation have been observed and no clinically detectable resynostosis has occurred.

Craniosynostoses↗
PubMedDate not supplied

Craniosynostosis.

Craniosynostosis affects approximately one infant out of one thousand. Increase of intracranial pressure and risks of functional problems are more frequent than previously thought, especially in single-suture synostosis. Frontocranial remodeling will correct both functional and esthetic consequences of craniosynostosis. The best time for surgery is the first year of life, 2-3 months of age for the brachycephalies, and 6-9 months of age for the other craniosynostoses. Not only does growth not deteriorate after forehead remodeling, but the adjacent orbitonasal areas improve with time. In Crouzon's disease and Apert's syndrome, early frontal advancement does not prevent the midface retrusion, and a radical frontofacial advancement may be occasionally indicated in very severe cases. Frontocranial remodeling is also indicated in children presenting with sequelae of classical neurosurgical treatment or those who have had no treatment.

Adult↗
PubMed1990

Delayed and progressive multiple suture craniosynostosis.

A considerable amount of information is available on various types of craniosynostoses. The patient exhibiting single suture synostosis that progresses to involve multiple sutures is distinctly uncommon, as is the patient exhibiting delayed synostosis involving all of the calvarial sutures. We report a group of 11 such patients with progressive and delayed holocalvarial synostosis. Most patients exhibited features of raised intracranial pressure or developmental delay, and in all patients symptoms were relieved after surgery. The diagnostic and therapeutic implications of this type of presentation in craniosynostosis are discussed.

Child↗
PubMed1991

The cranial base in normal and abnormal skull growth.

In the normal growth of the cranial base, an outline of prenatal development stressed the common vertebrate plan, the patterning of mesenchymal tissues formed from and influenced by migrating neural crest cells. Details are given of the sequences of chondrification and ossification. Postnatal growth is approached in two ways--sagittal growth and transverse growth--involving detailed consideration of sutural growth and of resorptive expansion and remodeling in the whole base. New conclusions have been reached about the growth of the ethmoid, which continues for a longer time than was generally thought, and of its relation to orbital growth. The dependence of fossa growth upon the early, almost explosive growth of the brain is shown in the sequences of basicranial growth, with the maturation of the fossae in a rostrocaudal direction. Of all the suture systems affecting the base, the coronal ring is most important (with the spheno-occipital synchondrosis next in importance). An off-shoot of the coronal ring is noted, the pterygoid buttress, and its importance stressed in relation to maxillary and midface growth. These considerations of normal morphology are widened to explore the dysmorphologies of the nonsyndromic and syndromic craniosynostoses. The abnormal patterning of these synostoses is shown to lie partly in the common pathways of expressivity of the calvarial and basicranial suture systems, again particularly the coronal ring, and partly in the development and responsiveness of the bony units themselves, whether normal or hypoplastic, and of their soft-tissue matrices.

Craniosynostoses↗
PubMed1977

[Carpenter's syndrome].

A newborn boy presented with an acrocephaly characterized by a coronal craniosynostoses, open sagittal sutures and abnormally high and straight forehead. He was the only child of young, unrelated, healthy parents; there was no familial history of dysmorphy. Facial asymmetry was important and associated with posterior cleft palate, syndactylia of the tips and polydactylia of feet, due to a splitting of the first metatarsus. The child also had a congenital heart disease, like in half of the 15 published cases. In older children, mental retardation is usually observed, often associated with obesity and hypogonadism. Polydactylia permitted to exclude Apert's acrocephalosyndactylia in which there is a normal number of finger arms and which seems to be a dominant mutation, while the transmission of Carpenter's syndrome appears autosomal recessive, thus requiring restrictive genetic counselling.

Abnormalities, Multiple↗