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At least 505 records · Page 28Linked to original sources
PubMed1986

The use of Zenker's solution in linear craniectomy for craniosynostosis: technical modification and reappraisal.

Regrowth of bone after craniectomy for craniosynostosis is still a problem, despite the advent of newer and extensive surgical techniques. A clinical study on 25 consecutive patients was undertaken to determine whether a modification of previous routines could retain the advantages of a tissue fixative, Zenker's solution, while eliminating the drawback of convulsive activity. After brief coagulation, Zenker's solution was sparingly applied to the dural surface for maximally one minute followed by copious irrigation of the surgical field. The results were satisfactory and postoperative complications unrelated to the fixative. The present procedure seems safe, effective, and easy to use.

Bone Regeneration↗
PubMed1994

Jackson-Weiss syndrome registered in four successive generations. The facies of Crouzon's syndrome with foot abnormalities.

A family with 15 individuals in four successive generations affected by Jackson-Weiss syndrome, craniosynostosis with Crouzon-variant-like phenotype and feet's abnormalities, is presented. An autosomal dominant inheritance pattern with complete penetrance, variable expressivity, and wide intrafamilial variation, more among, less within the same generation, was observed. Concerning the frequency and severity of complications, the evolution of craniofacial deformities seems to parallel those described with Crouzon syndrome, suggesting the similar evaluation and management.

Adult↗
PubMed1982

Cranioplasty for premature metopic suture closure (trigonocephaly).

A surgical procedure for instant and strong correction of the four factors (midline ridge, narrow bifrontal dimension, backward slant of right and left forehead, recession of frontal bone at supraorbital ridge) contributing to deviate appearance in trigonocephaly is described.

Child, Preschool↗
PubMed1994

Phenotypic variability in the Baller-Gerold syndrome: report of a mildly affected patient and review of the literature.

We report a patient with a mild form of the Baller-Gerold syndrome (craniosynostosis-radial aplasia syndrome). The patient, a 3-year 3 month-old boy, has trigonocephaly with bilateral absent radii and thumbs. His growth parameters and psychomotor development have been normal. No visceral anomalies were found. This patient represents a new case of the rare mild form of the syndrome.

Abnormalities, Multiple↗
PubMed1994

Teratogenic effects in a case of maternal treatment for acute myelocytic leukaemia--neonatal and infantile course.

Experience with the use of cytotoxic drugs in the first trimester of pregnancy is limited. We report on the clinical phenotype and infantile development of a girl born to a 36-year-old mother. Before recognition of pregnancy, the latter had been treated for acute myelocytic leukaemia receiving cytarabine, daunorubicin and doxorubicin at conception and cytarabine and thioguanine at about 35-37 days post conception. At delivery, there were severe brachycephaly, hypoplasia of the anterior cranial base and the midface as well as synostoses of both coronal and metopic sutures. Further findings included bilateral four-finger hands with hypoplastic thumbs and absent radii. This phenotype is reminiscent of the Baller-Gerold syndrome. The child, at present 15 months old, has had to undergo two operations for fronto-orbital advancement because of insufficient growth of the mid-face, nasal airway hypoplasia and increased intracranial pressure. Motor milestones are slightly retarded--neurodevelopment is otherwise normal. These findings are discussed in the context of the few previous reports and are particularly important for future genetic counselling.

Abnormalities, Drug-Induced↗
PubMed1993

Neuropsychological evaluation in a series of 17 cases operated on for craniosynostosis.

In a study of patients suffering from craniosynostosis various pre- and post-operative parameters were identified that may affect subsequent intellectual capacity. In particular, facial malformations appeared to be closely correlated with impaired intellectual development. Other parameters included measurement of cranial circumference and volume; their subaverage values correlated with slight mental insufficiency. However, in the pre-operative period they cannot be used as prognostic indices because these relations were statistically not significant. The remaining parameters (neurological and electroencephalographic findings, post-operative re-ossification of the skull, pre-operative digital impressions, CT findings) did not seem to be related to intellectual development.

Adolescent↗
PubMed1993

T-bone plastique for treatment of brachy-turricephaly.

The "T-Bone Plastique", which is presented in this paper, allows a surgical correction even of extreme cases of brachy-turricephaly together with malformations of the occipital region in one operative session. Brachy-turricephaly is characterized by abnormal vertical height of the skull and a shortening of its anterior-posterior length, frequently combined with malformations of the occipital region. Resection of the prematurely closed coronal suture, bi-parietal trepanations with 90 degrees rotation and side-exchange of the parietal bone flaps, double transverse trepanation of the occipital bone and outward bending and shifting of the bone fragments enable a bony remodeling and normalization of the deformed skull. Using this operative technique in three children we achieved a significant improvement of the skull form with an aesthetically pleasing result, without any neurologic sequelae and with normal development of the children during follow-up.

Cephalometry↗
PubMed1991

[Osteotomies for treating developmental disorders of the neurocranium and visceral cranium].

Craniofacial deformities are primarily caused by premature synostosis of cranial sutures. Depending on the involved sutures, typical deformities of the neuro- and visceral cranium are produced. They result in severe functional and aesthetic disturbances. Several osteotomies, which do not only make an aesthetic improvement possible but a correction of severe malfunction, are available for therapy. Preoperative planning and postoperative treatment require the combination of orthodontics and maxillofacial surgery.

Craniosynostoses↗
PubMed1985

Imaging modalities of craniosynostosis with surgical and pathological correlation.

Premature fusion of the sutures of the skull has been extensively analyzed and several diagnostic imaging modalities have been proposed. Radiographic, scintigraphic and computed tomographic features of craniosynostosis have been reported. This study was initiated (a) to determine the value and accuracy of plain skull radiography, skull scintigraphy and cranial CT; and (b) to determine the optimal imaging modality for the evaluation of a clinically suspected craniosynostosis. The study is based on 36 patients with clinical suspicion of craniosynostosis who underwent craniectomy and cranioplasty. One hundred and three sutures were pathologically and/or surgically evaluated. The overall accuracy rate for skull radiography was 89%, skull scintigraphy, 66%, and cranial CT, 94%. Routine skull series are adequate in the majority of cases of craniosynostosis. Skull scintigraphy is less accurate, more costly, and has a limited role in the evaluation of craniosynostosis. Cranial CT should be reserved for those cases in which the findings in the routine skull series are not clearly positive or negative, or in cases of more complex craniofacial anomalies.

Craniosynostoses↗
PubMed1985

Multisynostotic osteodysgenesis.

A sporadic case of multisynostotic osteodysgenesis is reported in a 15-day-old female infant with urogenital abnormalities. The main radiological findings were craniosynostosis, radiohumeral synostosis, femoral bowing and fractures. The typical combination of clinical and radiologic findings allow the ready recognition of this syndrome. Seven earlier cases have been reported, five sporadic and one familial; in two sisters.

Abnormalities, Multiple↗