Rare Facial Clefts Research Articles

Measuring Surgical Outcomes in Congenital Craniofacial Surgery

Assessment of surgical outcome in congenital craniofacial malformations is necessary to evaluate treatment efficiency since the results of early surgery are influenced by surgical technique and timing. Available outcome measurements all have their limitations, especially for application in congenital craniofacial malformations. Therefore a new outcome measurement was developed, in which each facial unit is scored in a standardized way. For each facial unit, deformities of shape or contour, malposition and soft tissue involvement were evaluated, besides scoring for specific congenital malformations of that area. The final result was tested on pre- and postoperative photographs of patients with rare facial clefts and reliability and validity were demonstrated. The new developed instrument showed the ability to provide quantification of outcome. Because of its properties it can serve as an instrument to compare outcome between techniques, surgeons and centers in a more objective and standardized way.

Rare Craniofacial Clefts and Encephalomeningoceles—Clinical Findings and Surgical Management under Conditions of Charity Missions

Purpose: The wide variation of craniofacial clefts and their complexity as well as the rarity of these conditions make classification difficult and require adjusted surgical strategies. For surgical treatment among the different structures involved in the cleft formation, the orbit and the cranial base require special consideration. In general, surgical treatment of rare facial clefts is a multistep procedure in almost all cases. This session reviews our experience in surgical management of craniofacial clefts under Third-World conditions applied during 18 charity missions in Africa, Asia, and Central America.

Craniofacial cleft: a case of Tessier no. 3, 7 and 11 cleft

The incidence of the rare facial clefts is between 1.43 and 4.85 per 100.000 births. We report a case of right associated Tessier no. 3, 7 and 11 craniofacial clefts with cardiac malformation. The epidemiology, classification, embryology and pathogenesis of each craniofacial malformation are briefly reviewed. After an extensive review of the literature, we conclude that this association has not previously been reported.

Étude épidémiologique et clinique des macrostomies. À propos d'une série de dix observations

Introduction. – Macrostomia or lateral cleft or commissural cleft or transverse cleft is a rare facial cleft in comparaison with standard cleft lip and palate. Method. – A retrospective study of 10 cases of congenital macrostomia is realised. For each case a retrospective study with epidemiologic and clinic features was realised. Results and Discussion. – From these clinical considerations, a new classification with direct surgical consequences is proposed.

A case of bilateral temporal muscle myositis accompanied by rare facial cleft

A case of myositis of both temporalis muscles in a craniofacial cleft is described. The patient was a 19-year-old male who underwent orbital translocation for hypertelorism thirteen months previously. He complained of bilateral painful temporal swellings and trismus with a high fever. Initially, antibiotic therapy seemed to be effective. At operation both temporalis muscles were slightly edematous but normal in color. No pus nor necrotic tissue was seen. A titanium plate and screws covered by the muscle on each side were removed. The postoperative course was uneventful. The histopathological diagnosis of the biopsied muscle was myositis.

Amniotic band syndrome: the association between rare facial clefts and limb ring constrictions.

The pathologic cause of both rare craniofacial clefts and congenital limb ring constrictions is the subject of some debate. Uncommon though these two conditions are, they have often been anecdotally reported in association. This study sought to determine the frequency of congenital limb anomalies in general and limb ring constrictions in particular amongst a population of patients with rare craniofacial clefts. Eighty-five cases of nonsyndromal, rare, craniofacial cleft were identified from a population of 280 patients assessed at the Women's and Children's Hospital, North Adelaide, with a diagnosis of rare craniofacial cleft classifiable by the Tessier system. Twenty-two patients (25.9 percent) displayed congenital limb anomalies. Eleven of these (12.9 percent) showed evidence of limb ring constrictions, a frequency much greater than in the general population. The group with limb ring constrictions demonstrated a significantly greater complexity of involvement with craniofacial clefting than the non-limb ring constriction group (4.27 clefts/patient versus 2.3 clefts/patient, p < 0.01). The distribution of craniofacial cleft locations in patients with evidence of limb ring constrictions was found to differ significantly from those with other or no limb anomalies (p < 0.01). The clefts in cases in which limb ring constrictions coexist are largely restricted to the paramedian 2-12, 3-11, 412 axes. This study confirms an association between rare craniofacial clefts and limb ring constrictions. The two conditions may therefore possess common etiology.

Rare facial and craniofacial clefts: Therapeutic guidelines and late results

Rare facial and craniofacial clefts: Therapeutic guidelines and late results

Craniofacial Anomalies in Twins

Studies of twins provide insight into the relative contribution of genetic and environmental factors in the causality of structural anomalies. Thirty-five affected twin pairs were identified from a group of 1114 patients with congenital craniofacial deformities evaluated from 1972 to 1989. Forty-three of these 70 twins exhibited one or more craniofacial anomalies; these were analyzed for dysmorphic characteristics, zygosity, concordance, and family history. The anomalies were categorized into two groups: malformations and deformations. The malformations (n = 36) included hemifacial microsomia (n = 10), cleft lip and palate (n = 8), cleft palate (n = 4), rare facial cleft (n = 2), craniosynostosis (n = 2), Binder syndrome (n = 2), Treacher Collins syndrome (n = 2), craniopagus (n = 2), CHARGE association (n = 1), frontonasal dysplasia (n = 2), and constricted ears (n = 1). The deformations (n = 7) included plagiocephaly (n = 5), hemifacial hypoplasia (n = 1), and micrognathia (n = 1). Twenty-one monozygotic and 14 dizygotic twin pairs were identified. The concordance rate was 33 percent for monozygotic twins and 7 percent for dizygotic twins.(ABSTRACT TRUNCATED AT 250 WORDS)