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At least 397 records · Page 22Linked to original sources
PubMed1978

Ear morphology in Treacher Collins', Apert's, and Crouzon's syndromes.

Size, proportions, level, inclination and shape of the ears were assessed by anthrompometric methods in eight patients with Treacher Collins' syndrome, eleven with Apert's, and 25 with Crouzon's syndrome. The ears were most severely damaged in Treacher Collins' syndrome, in which they were microtic or disproportionally long; many were low-set and with great inclination. All patients with Apert's syndrome had low-set ears and a tendency to disproportion, with widening and small inclination of the longitudinal axis. The ears were least affected in Crouzon's syndrome; in two-thirds of the patients there were mild growth variations leading to disproportion in width (wide ears), or low-set ears, or both.

Acrocephalosyndactylia↗
PubMed1975

Evaluation of craniofacial surgery in the treatment of facial deformities.

Surgical access to the cranial, orbital, and facial areas, as developed by Tessier, has produced not only definitive repair of previously uncorrectable congenital deformities such as orbital hypertelorism and facial stenosis (e.g., Crouzon's, Apert's syndromes) but also has improved markedly the treatment of traumatic and neoplastic defects. The surgical approach allows complete dissection of facial soft tisses including the orbits from the underlying bones followed by corrective osteotomies and fixation. Mobilization of the frontal lobes through a frontal bone flap exposure may be required. The ramifications of this latest intrusion by surgeons into a previously inviolate anatomic area have involved neurosurgeons, ophthalmologists, anesthesiologists, and dental and psycho-social disciplines. The disciplines of genetics and embryology are being influenced by this new field of surgery, much as the study of immunology was influenced by transplantation surgery two decades ago. This report analyzes a 10 year experience with over 100 patients with emphasis on patient selection by disease, age, intellectual status, morbidity, complications, and the psycho-social reactions of patient and family. Procedures initially planned to correct dental and aesthetic defects are proving beneficial for other functions including hearing, taste and smell, articulation and tongue movement, respiratory function, vision, and possibly bone growth. The development of self image, a normal process always in operation, is also strikingly altered. These operations may last as long as 14 to 16 hours. We have had no deaths or postoperative blindness. One postoperative cerebrospinal fluid leak was successfully repaired. Three partial losses of bone grafts and four instances of late cellulitis have occurred. Prevention of infection seems related to avoidance of dead spaces and primary closure of all mucosal, dural, conjunctival and skin surfaces.

Adolescent↗
PubMed1989

The psychosocial adjustment of pediatric craniofacial patients after surgery.

The postsurgical psychological status of 25 craniofacial patients, 6 through 16 years of age, was studied using self, teacher, and parent report measures. In contrast to earlier reports of more positive adjustment following corrective surgery, the present study identified several concerns for craniofacial patients, including low self-esteem, impaired peer relationships, and greater dependency on significant adults. Problems were more clearly identified using projective techniques rather than self-report measures. The findings suggest that many children having craniofacial surgery should have supportive psychotherapeutic services. Variables were explored relative to psychosocial functioning. Although few significant correlations were demonstrated, positive psychological adjustment was found to be related to greater physical attractiveness, lower parental stress, and younger age.

Adolescent↗
PubMed1978

Congenital and acquired deafness in clefting and craniofacial syndromes.

A study of 284 craniofacial defect patients determined factors associated with or predisposing to acquired or congenital hearing loss. Complete otolaryngic and audiologic evaluation was done at the initial assessment of a large group of patients. From there, a smaller number who had had adequate workup and followup was selected. The patients were classified into seven clefting categories, microtia/artresia, facial defects, cranial defects and miscellaneous anomalies of the head and neck. X-ray findings, those present at middle ear surgery and those studied at autopsy, are summarized. The incidence of hearing loss is 88 per cent. The likelihood of congenital hearing loss increases with the number of defects. Certain unifying concepts that may help identify a specific child as being in urgent need of otologic and audiologic testing are presented.

Abnormalities, Multiple↗
PubMed1975

Correction of severe facial deformity.

A craniofacial team has been developed to corrdinate the treatment of patients with severe facial deformity and to minimize the surgical risks. Two hundred patients have been evaluated in the last 4 years and more than 100 treated by this team to correct orbital hypertelorism, oxycephaly and plagiocephaly with exophthalmos, features of Crouzon's disease and Apert's and Treacher Collins syndromes, hemifacial microsomia, and severe lower facial deformity and malocclusion. Surgical principles include extensive subperiosteal stripping of bone, osteotomy as necessary, and repositioning maintained by bone grafts. Postoperative evaluation is being maintained for 5 years or to maturity in younger children. Although many of the results are less than perfect, there has been sufficient improvement, especially psychologic and functional, to warrant continuation of the surgical program under closely controlled conditions.

Acrocephalosyndactylia↗