Cases reported "Hallermann'S Syndrome"

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1/41. Obstructive sleep apnoea in a puerperal patient with Hallermann-Streiff syndrome.

    A 26 yr old puerperal female with Hallermann-Streiff syndrome developed serious obstructive sleep apnoea syndrome during pregnancy. She underwent an elective Caesarean section delivery, but ending the pregnancy did not improve her clinical symptoms. By treating her with nasal continuous positive airway pressure, a worsening of her headaches and glaucoma was prevented. The administration of acetazolamide controlled all of her symptoms. Treatment with nasal ventilation is the best initial approach. It is also important to assure normal oxygenation before pregnancy since the foetus may suffer from the severe deprivation that may occur in these patients. ( info)

2/41. 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. ( info)

3/41. Hallermann-Streiff syndrome and progeria.

    Reported is an atypical, severe case of Hallermann-Streiff syndrome combined with progeria, bilateral microphthalmus, cataracts, and normal chromosome count. A plea is made for study into the teratology of abnormal chemical, metabolic, and other forces that attack the early stages of the development of the human fetus and produce multiple anomalies. ( info)

4/41. Ultrasound biomicroscopic findings in hallerman-streiff syndrome.

    PURPOSE: To demonstrate the usefulness of ultrasound biomicroscopy in detecting the morphological changes in the lens caused by the spontaneous absorption of lens material and to detect fundus abnormalities in a patient with Hallermann-Streiff syndrome. methods: Case report of an infant diagnosed at the age of 2 months as having Hallermann-Streiff syndrome. RESULTS: Spontaneous lens absorption occurred during the course of follow-up and was detected only by ultrasound biomicroscopy after the patient was prepared for cataract surgery. The changes in the anterior chamber depth and lens shapes were documented by ultrasound biomicroscopy. Retinal folds that were barely observable by conventional ophthalmoscopy because of a dense cataract were clearly shown by ultrasound biomicroscopy. CONCLUSIONS: Ultrasound biomicroscopy can be used to examine the lenses of eyes that are not observable with conventional optical instruments. Ultrasound biomicroscopy can also be used to study the posterior segment of microphthalmic eyes. We recommend preoperative ultrasound biomicroscopy to prevent unnecessary anesthesia and surgical preparation. ( info)

5/41. Hallermann-Streiff syndrome associated with small cerebellum, endocrinopathy and increased chromosomal breakage.

    Hallermann-Streiff syndrome (HSS) is a rare clinic entity of unknown aetiology. Further clinical and metabolic-genetic evaluations are indicated. A 2-mo-old female baby presented with ocular abnormalities and severe failure to thrive since birth. The clinical features were compatible with the diagnosis of HSS. Further imaging, metabolic and cytogenetic examinations were performed. Features characteristic of HSS were dyscephaly with mandibular and nasal cartilage hypoplasia, microphthalmia, bilateral cataracts with congenital glaucoma, natal teeth and proportionate dwarfism. Rare anomalies such as choanal atresia and small cerebellum, very low insulin-like growth factor i level, hypothyroidism, generalized organic aciduria were also noticed. An increased chromosomal breakage rate is suggestive of the existence of some dna repair defects in HSS patients. CONCLUSION: The associated anomalies in this patient may broaden the clinical spectrum of HSS. Underlying conditions of organic aciduria, growth factor deficiency and impaired dna repair are likely to contribute to the progeria-like facies, congenital cataracts and growth failure. ( info)

6/41. 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. ( info)

7/41. An atypical Hallermann-Streiff syndrome. Focus on dental care and differential diagnosis.

    The Hallermann-Streiff syndrome (HSS) is a rare congenital disorder characterized by dyscephaly, birdlike facies, hypoplastic mandible, congenital cataracts, microphthalmia, hypotrichosis, skin atrophy, proportionate short stature, and dental anomalies. A case of a 29-year-old man with atypical HSS with neither cataracts, hair and skin alterations, nor short stature is reported, with special consideration to oral findings and dental management. Dental extractions, scaling, restorations, and endodontics were performed under local anesthesia. Later, orthodontic rehabilitation with fixed brackets was carried out. Finally, a removable partial denture for the maxillary arch was designed using transparent acrylic, and this also served as a retention splint. Young patients with HSS and other similar syndromes must be involved in personalized oral health prevention programs as early as possible. Despite numerous systemic anomalies, some of these patients may undergo conventional dental procedures under local anesthesia in the dental office. ( info)

8/41. 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. ( info)

9/41. Non-lethal Hallermann-Streiff syndrome with bone fracture: report of a case.

    The Hallermann-Streiff syndrome is characterized by bird-like face, micropthalmia, cataracts, micrognathia, beaked nose, abnormal dentition, hypotrichosis, cutaneous atrophy and proportional small stature. We present a 35-day-old patient with the classical signs except cutaneous atrophy, additionally he had a healing fracture at the proximal part of the left radius. ( info)

10/41. A new look at the management of the oculo-mandibulo-facial syndrome.

    The authors review the literature on the oculo-mandibulo-facial syndrome and present the case of a six-year-old boy with congenital cataracts, microphthalmos, nystagmus, failure to thrive, dysmorphic features with a tiny pinched nose, mandibular hypoplasia, microstomia, double chin, chronic snoring, recurrent respiratory infections and dental problems. Chronic obstructive sleep apnoea with decreased oxygen saturation was present. Optimal medical management of OMFS-patients is described. ( info)
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