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At least 865 records · Page 48Linked to original sources
PubMed1997

Airway management in children with craniofacial anomalies.

Craniofacial anomalies (CFA) predispose children to airway obstruction. A retrospective study was conducted to describe airway intervention required to manage patients with craniofacial syndromes and diseases involving the midface and mandible (i.e., Pierre Robin, Apert, Treacher Collins, Saethre-Chotzen, CHARGE, Nager, Stickler, Goldenhar, and Pfeiffer). The type of airway intervention, duration of intervention, and associated physical and medical conditions were evaluated. One hundred nine patients had charts available for review and met inclusion criteria. Sixty-five of these patients required airway management, most commonly in the first month of life, ranging from positioning to tracheotomy. Nineteen patients required a tracheotomy. Associated medical conditions and feeding difficulties were associated with airway obstruction. This study evaluates factors that predispose children with CFA to have airway problems that need treatment, as well as the types of airway management that are necessary.

Acrocephalosyndactylia↗
PubMed2003

Cephalometric analysis of the consolidation phase following bilateral pediatric mandibular distraction.

OBJECTIVE: The goal of the consolidation phase of mandible distraction is to maintain the improvement in maxillomandibular form and relationship while the generated tissue ossifies. During this period, external deforming forces can act on the healing generated bone. The purpose of this study was to describe the potential cephalometric changes that occur following pediatric bilateral mandibular distraction using external devices. DESIGN: Retrospective lateral superimposition cephalometric analyses. PARTICIPANTS: Thirty-five cases of pediatric mandible distraction were reviewed. Seven of these cases were included in the study after exclusion criteria were applied. These cases represented a group with severe congenital dysmorphology and a mean device activation of 26.5 mm. MAIN OUTCOME MEASURES: Changes in pogonion position, symphyseal plane rotation, mandible length, and mandible length relative to maxillary length during the 18 to 36 days of activation, the eight weeks of consolidation, and the 1-year period following removal of the distraction device were measured. RESULTS: All patients demonstrated variable changes in position of the mandible during the consolidation phase. The most common were retrusion of pogonion, a decrease in mandible length, and a clockwise rotation of the symphyseal plane. In some cases the changes that occurred during consolidation were greater than those that occurred on 1-year follow-up. CONCLUSIONS: The consolidation phase of distraction osteogenesis is a dynamic phase and should not be assumed to be static. Multicenter use of this cephalometric technique would help to identify potential risk factors associated with postactivation changes.

Cephalometry↗
PubMed2003

Increased fistula risk following palatoplasty in Treacher Collins syndrome.

OBJECTIVE: Patients with Treacher Collins syndrome have abnormal vascular supply to the palate, yet it is unknown whether there are increased postoperative healing problems following palatoplasty. This study investigated the correlation between Treacher Collins syndrome and postoperative palatal fistula formation. DESIGN: Retrospective chart review was performed. PATIENTS: Children undergoing palatoplasty at Children's Hospital Los Angeles from 1987 to 2000 were evaluated. Ten children with Treacher Collins syndrome, 92 children with other syndromes and cleft palate, and 458 nonsyndromic patients with isolated cleft palate were studied. INTERVENTIONS: All children were treated with a one-stage, double-reversing Z-plasty cleft palate repair. MAIN OUTCOME MEASURES: Outcome measures included intraoperative observations of surgical anatomy and postoperative clinic follow-up of fistula formation. Palatal fistula rates between patients with Treacher Collins syndrome, other syndromes, and no syndrome were compared with chi-square analysis. RESULTS: Children with Treacher Collins syndrome had significantly greater palatal fistula rates (50%) than children with other syndromes (8.7%) or no syndrome (4.1%). Treacher Collins patients demonstrated large palatal fistulas and poor flap vascularity. CONCLUSIONS: Children with Treacher Collins syndrome and cleft palate have significantly higher palatal fistula risk than other children with cleft palate when double-reversing Z-plasty palate repair is performed. Our findings suggest that children with Treacher Collins syndrome and cleft palate may have poor vascularity to palatal flaps created during palatoplasty. Furthermore, we recommend that surgeons performing palatoplasty minimize the dissection of mucoperiosteal flaps around the greater palatine arterial pedicle and utilize closure techniques creating the least vascular disruption of palatal tissue.

Chi-Square Distribution↗
PubMed1997

Use of extinction and reinforcement to increase food consumption and reduce expulsion.

Extinction and reinforcement contingencies were used to treat 2 children with feeding disorders. Positive reinforcement and avoidance extinction effectively increased food acceptance but also increased food expulsion. Reduced expulsion and increased swallowing were achieved by repeated presentation of expelled food, a second extinction component

Behavior Therapy↗
PubMed1995

Abnormal craniofacial growth.

Treatment of patients with craniofacial (CF) anomalies necessitates knowledge about normal CF growth and how it deviates in the abnormal state. There are different basic types of CF anomalies and various kinds of aberrations that influence CF development. These factors might help to explain why patients display growth variations. The effect of surgery on subsequent development is significant, but the heterogeneity among patients with regards to the morphology, etiology, and pathogenesis of the anomalies could also explain certain growth results. Roentgencephalometric findings related to CF growth in three different groups of anomalies are discussed.

Cephalometry↗
PubMed1978

Progressive hearing loss in Goldenhar's syndrome.

A family with Goldenhar's syndrome is presented. The pattern of inheritance is autosomal dominant with incomplete penetrance. The mutant gene in question has variable phenotypic expressivity. Evidence of progressive hearing loss is reported. Congenital facial palsy is suggested to be a part of the syndrome.

Facial Paralysis↗
PubMed1985

Classification and treatment of hemifacial microsomia.

On the basis of 37 surgically treated patients with hemifacial microsomia, an anatomical-surgical classification was developed which divides these patients into five categories to facilitate surgical planning and help standardize treatment. Treatment was founded on the cooperation of a large craniofacial team. Osteotomies of the jaws, construction of the TM joint with costochondral grafts, onlay bone grafts, and genioplasty were performed to improve facial form and function. Facial scars were avoided. After three-dimensional skeletal alignment, there was rarely a compelling need for subsequent soft tissue augmentation.

Adult↗
PubMed1987

General body growth in children with clefts of the lip, palate, and craniofacial structure.

This paper discusses general body growth in children with craniofacial clefts. Body growth is important in such patients because morphology reflects the cumulation of metabolism over time. The same hormones that direct general body growth also govern the ontogeny of the head and face. Body growth varies in children with different types of clefts. We found no average differences from US norms for those with isolated clefts of the lip alone or those with bilateral clefts of the lip and palate. Children with unilateral clefts of the lip and palate and with isolated cleft palate were significantly shorter than their unaffected peers. Males with these defects were also thinner than normal based on average standard deviation scores for body mass indices. Both unilateral and bilateral clefts of the lip and palate predominated in males, while isolated cleft lip was more frequent in females. Our results indicate that congenital metabolic variation contributes to the development of orofacial clefting and influences postnatal development in certain types of cleft. Accordingly, cleft type is important to growth prognosis, and growth status is relevant to optimization of therapy in orofacial cleft patients.

Adolescent↗
PubMed1990

Prenatal diagnosis by isoenzymic differentiation of Treacher Collins' syndrome induced by retinoids in rats.

A series of branchial arch malformations was induced in 618 embryos from 72 pregnant rats by a single intraperitoneal injection of 10 mg/kg etretinate at 8.5 days of gestation. The litters developed several malformations, including microtia, low set and dorsally placed outer ears, defective middle ear ossicles, short cochleas, defectively differentiated Meckel's cartilages, micrognathia, rudimentary malar bones, lateral facial clefts, fistulas and skin tags, all of which were similar to Treacher Collins' syndrome in man. The defects were accompanied by a pathological differentiation pattern of various isoenzymes in maxillary and mandibular processes. These isoenzymes could be detected in amniotic fluid from the 9th to the 20th days of pregnancy and showed a pathological differentiation pattern here as well. We conclude that a teratogenically induced syndrome affecting the first and second branchial arches is accompanied by a pathological differentiation pattern that can be traced by determinations of isoenzymes in the branchial arches as well as in amniotic fluid.

4-Nitrophenylphosphatase↗
PubMedDate not supplied

Diamond-Blackfan anemia associated with Treacher-Collins syndrome.

We describe a 2-year-old girl with a rare combination of congenital red cell aplasia or Diamond-Blackfan anemia (DBA) and Treacher-Collins syndrome (TCS). The anemia is only marginally responsive to high-dose corticosteroid, and the child is transfusion dependent. There is no one in the family affected with either DBA or TCS. A hypothesis is advanced that the simultaneous occurrence of the dysmorphism and erythroid agenesis in this case may have been the consequences of an insult to the fetus at the critical stage of development of maxillomandibular structure and the stage of primitive erythroid cell migration from the yolk sac to the fetal liver and bone marrow.

Adrenal Cortex Hormones↗
PubMed1980

Intracranial dermoid cyst in Goldenhar's syndrome. Case report.

A patient with Goldenhar's syndrome and an ipsilateral intracranial dermoid cyst is reported. It is suggested that the combination of both entities may not be coincidental but due to their related embryological origin. This report also reemphasizes the value of computerized tomography in the preoperative diagnosis of intracranial dermoid cysts.

Adult↗