[Oculo-mandibulo-facial syndrome. Hallermann-Streiff syndrome].
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Female↗
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[Seckel's syndrome and Hallermann-Streiff syndrome].
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Dwarfism↗
[Hallermann-Streiff syndrome].
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.
Craniofacial Dysostosis↗
Airway management in Hallermann-Streiff syndrome.
Hallermann-Streiff syndrome and other craniofacial anomalies predispose patients to airway emergencies. Anatomic abnormalities may render both intubation and tracheotomy unusually difficult. It is important for the otolaryngologist to be familiar with the special difficulties and hazards associated with these malformations in order to ensure optimal airway management.
Adolescent↗
Case report: orofacial characteristics of Hallermann-Streiff Syndrome.
BACKGROUND: Hallermann in 1948 and Streiff in 1950 described patients characterised by "bird face", congenital cataract, mandibular hypoplasia, and dental abnormalities. The new syndrome was later defined as Hallermann-Streiff Syndrome (HSS), underlining the differences with regard to Franceschetti's mandibulofacial dysostosis. CASE REPORT: Examination of a white male affected by Hallermann-Streiff Syndrome revealed facial characteristics typical of the "bird face" in HSS. The nose appeared thin, sharp and hooked; the prominence of the chin was absent in the lateral view; a marked microstomia was evident as well. Radiographic records showed aplasia of teeth 14, 13, 12, 22, 24, 35, 34, 33, 32, 31, 41, 42, and 43. As for orthodontic diagnosis, the following dentoskeletal features were assessed: skeletal Class II malocclusion, narrow upper arch, bilateral posterior crossbite, and anterior open bite. Lateral cephalogram showed hypoplasia of the mandible, a typical sign of HSS. The mandible revealed a clockwise rotation growth pattern with an opening of the gonial angle, skeletal anterior open bite, and an excessive vertical dimension of the lower third of the face. Radiographs at the age of 13 years showed an anomaly in shape of the lower right first bicuspid, which appeared with a double crown. TREATMENT: Orthodontic treatment started at 10 years of age with rapid maxillary expansion in the early stages of the mixed dentition, in order to correct the posterior crossbite due to the narrow maxillary arch. A subsequent phase of the orthodontic therapy consisted of a functional appliance with the goal of maintaining the transverse dimension of the dental arches and of controlling the tongue thrust in the anterior open bite. Surgical and prosthetic interventions were scheduled at completion of growth to solve the skeletal discrepancy and for occlusal rehabilitation. FOLLOW-UP: The therapeutic protocol used in the patient presented here was able to reach the orthodontic goals during the developmental ages. Further treatment is planned as there are multiple missing permanent teeth and prosthetic/restorative care is needed until the patient reaches full adulthood.
Child↗
Ocular manifestations in Kniest syndrome, Smith-Lemli-Opitz syndrome, Hallermann-Streiff-François syndrome, Rubinstein-Taybi syndrome and median cleft face syndrome.
In the pathology of malformations it is only in very few cases possible to identify a complete syndromal picture; the authors present some multimalformative syndromes, observed in the past few years at the Department of Pediatric Ophthalmology of the University of Siena. The ocular findings are reported and discussed in relation with the general features in order to make a correct diagnosis.
Abnormalities, Multiple↗
[Spontaneous bilateral lens resorption in a case of Hallermann-Streiff syndrome].
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.
Aphakia↗
What syndrome is this? Hallermann-Streiff syndrome.
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Child, Preschool↗
Roentgen differentiation of the oculodentodigital syndrome and the Hallermann-Streiff syndrome in infancy.
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Congenital Abnormalities↗
The Hallermann-Streiff syndrome.
The Hallermann-Streiff syndrome is characterised by systemic and ocular anomalies, including congenital cataract and microphthalmia. A case is presented and the differential diagnosis discussed.
Cataract↗
MCA/MR syndrome with features of Hallermann-Streiff syndrome and 4q deficiency/14q duplication.
In this report we present the clinical history and findings in a female newborn with 4q deficiency/14q duplication, the unbalanced product of a paternal t(4;14)(q33;q32). The clinical symptoms and signs observed in this child up to the age of 14 months were most compatible with the diagnosis of Hallermann-Streiff syndrome.
Abnormalities, Multiple↗
[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.
Child↗
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.
Adult↗
Evaluation and surgical correction of the facial skeletal deformity in Hallermann-Streiff syndrome.
A case of Hallermann-Streiff syndrome (HSS) is presented. The syndrome characteristics and differential diagnoses are reviewed. Diagnosis and correction of the maxillofacial deformities of this case is described. In light of this experience, surgical and anesthetic considerations are discussed.
Adult↗
Hallermann-Streiff syndrome: a review.
The Hallermann-Streiff syndrome is characterized by dyscephaly, hypotrichosis, microphthalmia, cataracts, beaked nose, micrognathia, and proportionate short stature. Cause is unknown; sporadic occurrence is the rule. Data presented in this review include the characteristics of pregnancy, growth and development, principal manifestations, radiographic and ophthalmological characteristics, and the results of cephalometric study. Potential complications in the syndrome are related to the narrow upper airway associated with the craniofacial configuration. Severe complications may include early pulmonary infection, respiratory embarrassment, obstructive sleep apnea, and anesthetic risk. Topics for future study are suggested.
Adolescent↗
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.
Bone and Bones↗
[Hallermann-Streiff-Francois syndrome].
Hallermann-Streiff-Francois Syndrome, or oculomandibulodyscephaly is a rare congenital anomaly, characterized by bilateral congenital cataracts and bird-face. As the disease always involves oral troubles, that sometimes precede or predominate over other symptoms, the patient need oral examinations and life-long rehabilitation.
Hallermann's Syndrome↗
[A case of Hallermann-Streiff syndrome with rapidly fatal course].
The Hallermann-Streiff syndrome is a rare affection characterized by beaked nose, dyscephaly, hypotrichosis, cataracts, micrognathia and proportionate short stature. The most severe complication in the syndrome is respiratory embarrassment. Narrow air passage with abnormal glottic closure, prenatal growth deficiency, immunodeficiency and sometimes associated cardiovascular anomalies, can predispose these patients to pulmonary infections. The Authors describe a new-born with Hallermann-Streiff syndrome, who, after a brief period of obstructive apnea successfully treated with CPAP, developed a severe pulmonary infection that caused his death at the age of 61 days.
Age Factors↗