Dental Rehabilitation of a Rare Hallermann-Streiff Syndrome with Olygodontia: A Case Report / Rehabilitación dental de un caso raro de Síndrome de HallermannStreiff con Oligodoncia: Reporte de un caso

Abstract Hallermann-Streiff syndrome (HSS) is a rare oculomandibulofacial discephaly with hypotrichosis that occurs as a sporadic mutation. It is characterized by abnormal findings especially in head and face. Dental anomalies occur in 50-80% of the patients. In this case report, facial-oral findings and the existing dental anomalies of the syndrome in a 6-year-old male patient diagnosed with HSS were identified. Dental rehabilitation and a 12-month follow-up of the patient were reported. Each case presented with this rare syndrome may contribute to the literature to determine the prognosis of the disease and to take protective and preventive measures.

Resumen El síndrome de Hallermann-Streiff (HSS) es una discefalia oculomandibulofacial rara con hipotricosis que ocurre como una mutación esporádica. Se caracteriza por hallazgos anormales, especialmente en cabeza y cara. Las anomalías dentales ocurren en 50-80% de los pacientes. En este reporte de caso, se identificaron los hallazgos faciales-orales y las anomalías dentales existentes del síndrome en un paciente masculino de 6 años diagnosticado con HSS. Se informó la rehabilitación dental y un seguimiento de 12 meses del paciente. Cada caso presentado con este síndrome raro puede contribuir a la literatura para determinar el pronóstico de la enfermedad y tomar medidas de protección y prevención.

Síndrome de Hallermann-Streiff y embarazo: manejo de la vía aérea difícil en embarazadas / Hallermann-Streiff syndrome and pregnancy: management of difficult airway in pregnant women

El síndrome de Hallermann-Streiff es una rara entidad asociada a hipoplasia del tercio inferior de la cara, determinando así una vía aérea de difícil manejo. Se presenta el caso de una mujer de 21 años con ese síndrome, acondroplasia, escoliosis severa e infección respiratoria los días previos a la interrupción exitosa de su embarazo mediante cesárea. El manejo requirió una cuidadosa evaluación preoperatoria y disponibilidad inmediata de dispositivos alternativos para el manejo de la vía aérea en caso necesario. La gravidez, así como ciertas comorbilidades asociadas, aumentan la posibilidad de una intubación fallida con morbi-mortalidad secundaria importante, dado ello, es necesaria la preparación e implementación de algoritmos atingentes para el manejo de la vía aérea en casos como el presentado.

The Hallermann-Streiff syndrome is a rare entity associated to hypoplasia of the lower third of the face, determining a difficult airway management. We report the case of a 21 years female with this syndrome, achondroplasia, severe scoliosis and respiratory infection at the days prior to the interruption of her preg-nancy by cesarean section. Her management required a carefully preoperative evaluation and availability of alternative devices to secure her airway. Pregnancy and certain comorbidities increase the chance of a failed intubation with severe secondary morbidity and mortality, given this, the correct preparation and implementation of difficult airway algorithms in pregnancy if it’s necessary.

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.

Two Cases of Hallermann-Streiff Syndrome Diagnosed in Early Neonatal Period

Hallermann-Streiff syndrome is a very rare congenital disorder, which is primarily characterized by the head and face abnormalities. Approximately 180 cases have been reported worldwide, including 8 cases in Korea since it was first described by Hallermann in 1893. Patients exhibit a bird-like face, hypotrichosis, atrophy of skin, dental abnormalities, proportionate nanism, and various ophthalmic disorders, including congenital cataracts and bilateral micropthalmia. As a result of many life-threatening complications, such as respiratory and cardiac difficulties, many patients die during infancy. We report here two cases of HSS diagnosed immediately after birth with literature reviews. They showed two additional characteristics, including chubby cheeks and antenatal ultrasonographic findings, which have not been mentioned in previous reports.

A case of Hallermann-Streiff syndrome with aphakia / 소아과

Hallermann-Streiff syndrome is a rare disease. Approximately 150 cases have been reported, including 6 cases in Korea. The authors experienced a case of Hallermann-Streiff syndrome in a 6-year-old female with aphakia. The syndrome is characterized by a bird-like face, dental abnormalities, hypotrichosis, atrophy of the skin, bilateral microphthalmia, and proportionate dwarfism. A brief review of the literature was conducted.

A Case of Hallermann-Streiff Syndrome

PURPOSE: We report a case of Hallermann-Streiff syndrome with 360 degrees posterior synechiae, small pupils and aphakia. METHODS: A five-year-old female presented with decreasing visual acuity of both eyes. Visual acuity was not checkable due to mental retardation. Microcornea, microphthalmia, nystagmus and esotropia were found, and a fundus examination was not available due to 360 degrees posterior synechiae and small pupils. She had developmental delays, bird-like face and hypotrichosis. A pediatric physician was consulted who diagnosed her with Hallermann-Streiff syndrome. Refraction and fundus examinations were impossible due to her small pupils, so synechiolysis was done. RESULTS: After synechiolysis and pupil dilatation in right eye with iris retractors, continuous curvilinear capsulorhexis (CCC) was attempted. However, the anterior capsule was unusually fragile and fibrtic. Therefore, the CCC failed. In addition, the crystalline lens and the zonule were not found. The posterior capsule was fragile similar to the anterior capsule. Complete posterior CCC (PCCC) was impossible. We could not find any formed vitreous in the vitreous cavity during anterior vitrectomy. We diagnosed the condition as aphakia with only two layers of membranes. Two weeks later, synechiolysis in the left eye was done. The left eye was also diagnosed with aphakia, and only synechiolysis was performed. CONCLUSIONS: The possibility of aphakia must be always considered in cases of Hallermann-Streiff syndrome.

A Case of Hallermann-Streiff Syndrome

PURPOSE: We report a case of Hallermann-Streiff syndrome with 360 degrees posterior synechiae, small pupils and aphakia. METHODS: A five-year-old female presented with decreasing visual acuity of both eyes. Visual acuity was not checkable due to mental retardation. Microcornea, microphthalmia, nystagmus and esotropia were found, and a fundus examination was not available due to 360 degrees posterior synechiae and small pupils. She had developmental delays, bird-like face and hypotrichosis. A pediatric physician was consulted who diagnosed her with Hallermann-Streiff syndrome. Refraction and fundus examinations were impossible due to her small pupils, so synechiolysis was done. RESULTS: After synechiolysis and pupil dilatation in right eye with iris retractors, continuous curvilinear capsulorhexis (CCC) was attempted. However, the anterior capsule was unusually fragile and fibrtic. Therefore, the CCC failed. In addition, the crystalline lens and the zonule were not found. The posterior capsule was fragile similar to the anterior capsule. Complete posterior CCC (PCCC) was impossible. We could not find any formed vitreous in the vitreous cavity during anterior vitrectomy. We diagnosed the condition as aphakia with only two layers of membranes. Two weeks later, synechiolysis in the left eye was done. The left eye was also diagnosed with aphakia, and only synechiolysis was performed. CONCLUSIONS: The possibility of aphakia must be always considered in cases of Hallermann-Streiff syndrome.

A Case of Hallermann-Streiff Syndrome with Intra-Uterine Growth Retardation

Hallermann-Streiff syndrome is a rare disease; approximately 150 cases have been reported in the world literature. The syndrome consists of proportionate nanism; hypotrichosis; atrophy and extreme thinness of skin, particulary over the facial area; an usual "bird-like" face with mandibular hypoplasia; a prominent thin, pointed nose; congenital cataracts; and severe dental abnormalities. We report a case of Hallermann-Streiff syndrome in premature who showed intrauterine growth retardation with proportionate nanism, brachycephaly, a beaked nose, "bird like" face, hypoplasia of the mandible, microphthalmia, congenital cataract, neonatal teeth, and widening of sagittal suture were all found on our patient.

A Case of Hellermann-Streiff Syndrome

Hallermann-Streiff Syndrome is a rare congenital anomaly characterized by dyscephalia, dental anomalies, hypotrichosis, congenital cataract, bilateral microphthalmia and has characteristic feature of bird face due to marked mandibular hypoplasia and beaked nose. The authors have experienced a case of Hallermann-Streiff Syndrome combined with bilateral aphakia and glaucoma in a 34 years old woman and we report this case with the review of literatures.

HALLERMANN-STREIFF SYNDROME / 西安交通大学学报(医学版)

8 cases of Hallermann-streiff syndrome were found trom 1980 to 1988. Two of them had been followed up for 7 years. The patients consisted of 6 males and 2 females at 8-32 years of age. The clinical manifestations were as follows : ① dsycephaly with bird face and ; ② congenital cataract or aphakic in all cases; ③ dental anomaly in 7 cases; ④nanism in 4 cases and the fontanel frontalis (maior) of 1 casc (case 7, males 32 years of age, height 1. 23 m, weight 29. 5 kg) was not closed, ⑤ atrophy of skin in 7 cases whosc skin appeared shine and thin, particularly on the nose top, and one or two areas of alopecia appeared in 3 of 6 cases, ⑥ hypotrichosis in 8 cases and ⑦ bilateral microphthalmia in 6 cases. ① and ② were significant for the diagnosis of Hallemann-Streiff syndrome.