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PubMed1998

[Nager syndrome].

In this publication, Nager syndrome was analyzed in the literature and six patients from our clinic were evaluated in relation to symptoms, etiology and pathogenesis. The diseases to be considered when making a differential diagnosis are pointed out. Clarification of the etiology is still pending. Molecular genetic research in these patients is possibly the key for new findings. A case report illustrates the results of interdisciplinary treatment by the surgeon and orthodontist. Possibilities and problems in relation to therapy are demonstrated.

Abnormalities, Multiple↗
PubMed1978

Noonan syndrome in an adult family presenting with chronic lymphedema.

A 27 year old man with multiple findings of the Noonan syndrome ("male Turner" phenotype) presented with chronic lymphedema which was also present in his mother. Noonan syndrome should be considered in the differential diagnosis of chronic or familial lymphedema.

Abnormalities, Multiple↗
PubMed1986

The prenatal diagnosis of Robin anomalad.

The Robin anomalad was diagnosed by the sonographic detection of polyhydramnios and fetal micrognathia in a patient at risk because of a previously affected child. Ultrasound in the second trimester failed to demonstrate any facial anomaly, but mandibular hypoplasia was clearly documented in the third trimester. The antenatal diagnosis allowed immediate neonatal assistance to prevent glossoptosis-induced respiratory failure.

Adult↗
PubMed1980

The cerebro-oculo-facio-skeletal syndrome.

A 3 1/2-month-old boy with the cerebro-oculo-facio-skeletal syndrome had low birth weight, microcephaly, microphthalmia, cataracts, blepharophimosis, high nasal bridge, micrognathia, kyphosis, rocker-bottom feet, and a longitudinal foot groove. The product of a consanguineous parentage, he showed marked developmental retardation, suggesting abnormal recessive inheritance.

Adult↗
PubMed1975

Diagnosis and treatment of maxillomandibular dysplasias.

Skeletal dysplasia of the maxilla and/or mandible is responsible for prognathism or retrognathism. The severity of either condition is in direct proportion to the degree of dysplasia noted within each jaw. Mandibular prognathism is real or apparent. Some of the patients described in this article demonstrate this quite clearly when the jaws are analyzed individually and related to each other. A differential diagnosis is essential to determine whether the abnormality is in the maxilla or the mandible, or both. When recognized early in the formative years, it permits the orthodontist to employ guidance and full therapy procedures in a treatment plan that is programmed to include surgical procedures specifically for the skeletal aberration to be corrected. The treatment skills of the orthodontist and his knowledge of growth of the skeletal and dental structures aid in diagnosis and treatment planning in the areas of both orthodontics and surgery. Close cooperation between the orthodontist and the surgeon permits a more definitive resolution of the existing and potential skeletal and dentoalveolar abnormalities.

Adolescent↗
PubMed1981

Hypoglossia: case report and discussion.

A case of hypoglossia was observed between the ages of 3 and 15 years in a patient with micrognathia, congenital absence of three lower incisors, and a telescopic occlusion accompanied by an extremely narrow lower arch and severe overbite. Orthodontic intervention was begun when the patient was 8 years old. Craniofacial growth changes that occurred before and after orthodontic treatment are discussed. Cephalometric study of this case suggests the distinctive features of the postnatal growth pattern similar to other cases with the syndrome.

Adolescent↗
PubMed1993

Sternal cleft associated with vascular anomalies and micrognathia.

Sternal defects combined with craniofacial vascular defects are rare. We report on a 45-year-old woman with a sternal cleft associated with craniofacial and brain hemangiomata, an aneurysm of the aortic arch, anomalous origin of the coronary arteries, a left superior vena cava, micrognathia, supraumbilical midline raphe, and a cervical cyst. The surgical procedure consisted of the resection and replacement of the aortic arch and the innominate artery with reimplantation of the left carotid artery into the graft under circulatory arrest and deep hypothermia. The presence of sternal cleft is an indication for the search for other asymptomatic internal vascular anomalies.

Abnormalities, Multiple↗
PubMed1995

Hanhart's syndrome with facial asymmetry: case report.

An unusual variant of Hanhart's syndrome associated with facial asymmetry in a female patient is presented together with surgical treatment of her facial anomalies. Following reconstruction which included bimaxillary osteotomies and osteopericranial and pericranial flaps, a good aesthetic result was achieved which enabled the psychological and social rehabilitation of the patient.

Abnormalities, Multiple↗
PubMed1983

Micrognathia-prenatal ultrasonographic diagnosis.

The first case of prenatal real-time ultrasonographic diagnosis of micrognathia is presented. Diagnosis was made at 17 weeks' gestation in the fetus of a patient with previous infant affected with the syndrome of thrombocytopenia with absent radii (TAR). Recognition of micrognathia as a part of TAR syndrome and the easy visualization of fetal facial bones in the second trimester made diagnosis possible.

Adult↗
PubMed1990

Occlusion for the Class II jaw relations patient.

Each patient with a small mandible presents an occlusal pattern that differs from most of the criteria established for a class I occlusion. The components of a class II occlusion are unlike other occlusions. The anterior teeth do not provide the protection and guidance normally provided by horizontal and vertical overlap. The posterior teeth bear the full force of occlusion. Protrusive movement is the dominant and the most used movement. The posterior teeth must function from centric relation forward to an anterior separation. Most of the posterior teeth exhibit balancing contacts. This occlusion is a type of bilateral balanced occlusion. Greater interocclusal clearance must be provided and tested for adequacy of space during speech and function. A class II occlusion is a most exacting occlusion. The controlling factors are the posterior determinants of occlusion.

Dental Occlusion↗