Orodental findings in Hallermann-Streiff syndrome.

Hallermann-Streiff syndrome-also called occulomandibulofacial syndrome, Francois syndrome, oculomandibulodyscephaly with hypotrichosis, Aubry syndrome I, and Ullrich-Fremery-Dohna syndrome-is a rare genetic disorder, which comprisesmultiple congenital abnormalities affecting chiefly the head and face. It is characterized by bird-like facies, dental abnormalities, hypotrichosis, atrophy of skin, congenital cataracts, bilateral microphthalmia, and proportionate nanism. An interesting case of Hallermann-Streiff syndrome in a 23-year-old female patient is reported here, with the emphasis on the orodental findings.

Hallermann-Streiff syndrome: a case report from Georgia.

We report a 9 years and 6 month old boy with the Hallermann-Streiff syndrome (HSS). The patient was referred by a pediatrician. The diagnosis was established by endocrinologist based on the presence of specific facial gestalt (bird-like face) and bilateral congenital cataracts. The patient was not short, but had mild mental retardation; hypotrichosis was represented by Sign of Hertoghe (Queen Anne's sign) only. Thyroid function was normal. X-ray study yielded valuable data. The night apnoea (secondary to the dyscephalic narrowing of the upper airways) constituted the major concern. We also discuss diagnostic criteria for the HSS along with significance of various clinical signs. Combination of specific facial gestalt and ocular abnormalities should be particularly alarming. Endocrine aspects of the HSS are reviewed. X-ray study is recommended as an inexpensive and readily available but informative tool.

Hallerman-Streiff-like syndrome presenting with laterality and cardiac defects.

We report two patients considered to have an atypical presentation of Hallerman-Streiff syndrome (HSS) associated with laterality and cardiac defects. Clinical features include typical facial gestalt, atrophy of the skin, and hypotrichosis. Ophthalmologic abnormalities, normally present in HSS, are only found in one of the two patients. Both of them have respiratory problems secondary to the classical narrow upper airway described in this syndrome. Both these patients have laterality defects and one has additional structural cardiac malformations. Cardiac defects have occasionally been reported in the HSS literature, but are not considered as a classical feature of the syndrome. Situs inversus has never been reported in this syndrome. Almost all HSS cases have been sporadic and their origin and inheritance pattern remain unknown.

Osteocraniostenosis-hypomineralized skull with gracile long bones and splenic hypoplasia. Four new cases with distinctive chondro-osseous morphology.

Osteocraniostenosis is a severe skeletal dysplasia characterized by a hypomineralized skull that has been previously described as kleeblattschädel (cloverleaf skull) and overtubulated long bones. Dysmorphic facial features include a short nose, short philtrum, and a small, inverted V-shaped mouth. Splenic a/hypoplasia is a constant finding. We report four infants (two unrelated and two siblings) with osteocraniostenosis and describe the clinical, radiographic and chondro-osseous morphology findings. The two siblings lack the moderate long-bone shortening that is typically seen. The skull configuration is likely caused by severely hypoplastic cranial bones (parietal) rather than true craniosynostosis, making the term "osteocraniostenosis" misleading. Histological examination of bone in all cases showed an abnormal growth plate with short irregular columns. The resting cartilage showed pleomorphic chondrocytes with increased cellularity and unique pseudocolumn formation. There are some radiographic and chondro-osseous morphologic similarities between osteocraniostenosis and severe Hallermann-Streiff syndrome (HSS), suggesting the two disorders may be pathogenetically related.

Hallermann-Streiff syndrome: a case review.

Hallermann-Streiff Syndrome is a rare genetic disorder that is characterized primarily by head and face abnormalities. Patients show bird-like facies, dental abnormalities, and hypotrichosis with various ophthalmic abnormalities. We report here a 26-year-old woman with Hallermann-Streiff Syndrome and review the literature.

Lethal syndrome of slender bones, intrauterine fractures, characteristics facial appearance, and cataracts, resembling Hallermann-Streiff syndrome in two sibs.

We report on a family in which 1 males infant who died neonatally and 1 female fetus at 29 weeks of gestation had an identical condition resembling Hallermann-Streiff syndrome. The long bones were slender with a few fractures, the skull was underossified, and the face was characteristic of Hallermann-Streiff syndrome. Bilateral cataracts were identified in the male. We regard the condition in this family as a severe form of Hallermann-Streiff syndrome, which appears to have been lethal, at least in the liveborn male. This syndrome is usually sporadic. Recurrence in sibs suggests the possibility of autosomal-recessive inheritance, or of a dominant mutation with parental mosaicism.

Hallerman-Streiff syndrome.

A 3 day old female neonate with Hallerman Streiff Syndrome presented with white spots in both the eyes. Both eyebrows and eyelashes were found to be sparse. Anterior chamber was found to be shallow. Total cataract was detected with posterior synechiae. Fundus could not be viewed. General examination revealed other features of Hallerman-Streiff Syndrome--short stature, bird like face, atrophy of skin and natal teeth. Lensectomy was carried out for left eye at the age of 10 weeks. However, the child had repeated respiratory tract infections and died at the age of 22 weeks.

A typical Hallermann-Streiff syndrome in a 3 year old child.

Hallermann Streiff syndrome is a rare congenital disorder characterized by dyscephaly, dental anomalies, proportionate nanism, hypotrichosis, cutaneous atrophy limited to the head, bilateral congenital cataracts and bilateral microphthalmia. Despite the marked craniofacial characteristics and oral findings, a relative lack of reports in the dental literature has been noted. In this article, a review of the literature regarding the general clinical features and differential diagnosis is presented. A case of a 3-year-old female is reported with special consideration on the management of her dental problems under general anesthesia. The anesthetic risks of such a procedure as well as the prognosis of her dental development are being discussed.

Hallerman-Streiff syndrome: case report and recommendations for dental care.

Hallerman-Streiff syndrome is a rare congenital anomaly characterized by a peculiar bird facies, mandibular and maxillary hypoplasia, dyscephaly, cataracts, microphtalmia, hypotrichosis, skin atrophy, and short stature. Dental abnormalities are present in 80 percent of the cases and include malocclusion, crowding, severe caries, supernumerary and neonatal teeth, enamel hypoplasia, hypodontia, premature eruption of primary dentition, agenesis of permanent teeth, and anterior displacement or absence of condyles. Very few cases have been described in the dental literature. The predisposition to severe caries, together with other problems, makes it imperative that young patients be started in a strong prevention program as early as possible. This is a case report of a five-year-eleven-month-old white male who presented for a dental examination at The Children's Hospital in Denver, CO. The findings and recommendations for treatment are discussed.

[Spontaneous bilateral lens resorption in a case of Hallermann-Streiff syndrome]. / Spontane beidseitige Linsenresorption bei einem Fall von Hallermann-Streiff-Syndrom.

The Hallermann-Streiff-syndrome was first described in 1948 by Hallermann and in 1950 by Streiff. The most common features are dyscephalia, cataract, microphthalmia, dental anomalies, hypotrichosis, cutaneous atrophy, and nanism. Anomalies of the eye include cataract, microphthalmia, nystagm, strabism, blue sclera, fundus anomalies and combined anomalies of all segments of the eye. The frequency of cataract is about 90%. Spontaneous resorption of the lens is described in about 8%. We present a five year old girl showing the typical dyscephalia of the Hallermann-Streiff-syndrome (bird face). Her lenses were replaced by opaque membranes. These membranes presumably were remnants of the posterior lens capsule.

[A case of the Hallermann-Streiff syndrome].

The Hallermann-Streiff Syndrome has been characterized and established according to 7 positive and 5 negative signs, which were described by François. We encountered an 11 year, 5 months old boy who had 7 positive symptoms of this syndrome in our clinic. In this study, we reported on this typical patient focusing on his dental view. 1) Prolonged retention of the primary teeth which involved microdontia were noted. Congenitally missing teeth were also seen. 2) The occlusal relationship indicated open bite, and also the mandibular function was impaired. 3) The measurements of the length and width of the dental arch were smaller than that of a normal subject, and the dental arch of the maxilla was V-shaped. 4) According to X-ray cephalometric analysis, (a) the dental calculus and the alveolar bone absorption were very evident. (b) abnormal morphologic of the glenoid fossa, mandibulars condyle and the neck of mandibula were seen. These conditions were very evident on the left side. 5) The growth obstade of the maxilla and mandibla and the left shift of the mandible were found. 6) According to histological study, enamel hypoplasia was noted.

[The Hallermann-Streiff syndrome in 2 generations]. / Hallermann-Streiffov syndróm vo dvoch generáciach.

The authors discuss a rare case, unique in our literature, of the familial incidence of Hallermann-Streiff's syndrome and the treatment of some of its complications. Special emphasis is laid on genetic analysis which provides evidence of autosomal dominant heredity with a variable expressivity of the pathological gene, and which must be applied in genetic consultations.

[Hallermann-Streiff syndrome]. / Sindrome di Hallermann-Streiff.

Hallermann-Streiff syndrome is a second branchial arch defect with significant ophthalmologic, dental and craniofacial findings. These anomalies provide a difficult management and treatment situation for both the restorative dentist and orthodontist and maxillo-facial surgery.

Hallermann-Streiff syndrome and its oral implications.

A patient with Hallermann-Streiff syndrome was followed from birth to nine years of age. The oral manifestations during the course of the nine years were documented.

Dento-alveolar abnormalities in oculomandibulodyscephaly (Hallermann-Streiff syndrome).

Oculomandibulodyscephaly (OMD) is characterized by anomalies of skull, mandible, skin and eyes and also by a proportionate nanism. Dental abnormalities are often observed but poorly defined. Examination of a 2-month-old infant that suffered from OMD, having died from an unrelated cause revealed premature eruption of several deciduous teeth, crowding of unerupted deciduous teeth and agenesis of all permanent teeth excepting the first permanent molars.