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.

Brown-McLean Syndrome in a Patient with Hallermann-Streiff Syndrome

No abstract available.

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.

Surgical Correction of Hallermann-Streiff Syndrome: A Case Report of Esotropia, Entropion, and Blepharoptosis

We report a case of surgical treatment for Hallermann-Streiff syndrome in a patient with ocular manifestations of esotropia, entropion, and blepharoptosis. A 54-year-old man visited Yeouido St. Mary's Hospital complaining of ocular discomfort due to cilia touching the corneas of both eyes for several years. He had a bird-like face, pinched nose, hypotrichosis of the scalp, mandibular hypoplasia with forward displacement of the temporomandibular joints, a small mouth, and proportional short stature. His ophthalmic features included sparse eyelashes and eyebrows, microphthalmia, nystagmus, lower lid entropion in the right eye, and upper lid entropion with blepharoptosis in both eyes. There was esodeviation of the eyeball of more than 100 prism diopters at near and distance, and there were limitations in ocular movement on lateral gaze. The capsulopalpebral fascia was repaired to treat the right lower lid entropion, but an additional Quickert suture was required to prevent recurrence. Blepharoplasty and levator palpebrae repair were performed for blepharoptosis and dermatochalasis. Three months after lid surgery, the right medial rectus muscle was recessed 7.5 mm, the left medial rectus was recessed 7.25 mm, and the left lateral rectus muscle was resected 8.0 mm.

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.

Enfermedades genéticas que afectan la cavidad bucal: revisión de la literatura / Genetic diseases affecting the oral cavity: review of the literature

En el presente artículo se hace referencia a síndromes y enfermedades sistémicas involucradas en la cronología de la erupcion dentaria, afeccion de los maxilares y en general con manifestaciones a nivel de la cavidad bucal. La lista es excesiva, y por lo tanto no se pueden mencionar todos los isndromes y enfermedades sistémicas comprometidas, motivo por el cual nos limitaremos a algunas de las entidades más representativas del probelma como son: querubismo, displasia cleidocraneal, disostosis craneofacial, Síndrome de teacher Collins, Síndrome de Pierre Robin, Síndrome de Hollermann Streiff, osteopetrosis, osteogénesis imperfecta, Síndrome de Albright, Síndrome de Ellis - Van Creveld, Síndrome de Down, Síndrome de múltiples carcinomas basocelulares nevoides y quistes de los maxilares, Síndrome de Marfan. Para este estudio nos hemos basado en la clasificación de las enfermedades bucodentales de origen genético que hace la Clasificación Internacional de Enfermedades aplicadas a la odontología y estomatología (CIE -AO), Organización Panamericana de la Salud, 1985. El conocimiento de la existencia de estas patologías reviste gran importancia pues requieren de varios cuidados y remisión a especialistas adecuados. Con este artículo se pretende hacer un aporte en el reconocimiento de enfermedades que aunque no están relacionadas directamente con el odontólogo, éste debe conocer para su correcto manejo

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.

Tratamiento de las anormalidades dentofaciales que acompañan al síndrome de Hallermann-Streiff / Treatment of the dentofacial abnormalitys that accompany sindrome by Hallermann-Streiff

El síndrome de Hallermann-Streiff es una enfermedad genética rara caracterizada entre otros signos, por discefalia, hipotricosis, microftalmia, cataratas, nariz picuda y enanismo proporcionado así como alteraciones en el desarrollo de los maxilares y en el número, tamaño, forma y estructura de los dientes. El caso que reportamos presentaba hipoplasia de ambos maxilares, hipodoncia, microdoncia, hipoplasia del esmalte y dientes con raices cortas, que en su conjunto alterabn la estética facial y la función masticatoria. El tratamiento de las alteraciones dentofaciales se realizó utilizando sobredentaduras habiéndose obtenido resultados estéticos y funcionales aceptables.

Malformaciones genéticas y talla baja / Genetic malformations and low stature

Pra el médico pediatra es muy importante reconocer en aquellos pacientes con talla baja la posible asociación con un genotopatía. A continuación se revisan algunos de los síndromes malformativos que presentan características comunes y los hallazgos clínicos más frecuentes

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.

Hallermann-Streiff syndrome: airway problems during anaesthesia.

In the patients with Hallermann-Streiff Syndrome, presence of mandibular hypoplasia and microstomia results in difficult intubation. The anaesthetic management of a three month old child with this syndrome for lensectomy is presented in this report. There was difficulty in maintaining the airway patency during induction which was solved with the help of a modified, improvised airway. Intubation was done after induction with a combination of inhalational and intravenous anaesthetic agents and muscle relaxant. Recognition of this syndrome should alert the physician to the possibility of difficulty in airway maintenance.

Síndrome de Hallerman-Streiff y embarazo: reporte de un caso / Hallermann-Streiff's syndrome and pregnancy. a case report

El síndrome de Hallermann-Streiff se caracteriza por discefalia, microftalmia bilateral, catarata congenita, nariz pequeña y afilada, anomalías dentales, micrognatia, hipotricosis, atrofia cutánea y talla baja proporcionada. Las mujeres afectadas con el síndrome, raramente son vistas por el ginecoobstetra por lo que se desconocen sus repercusiones sobre la fertilidad. Su asociación con la gestación es muy rara. Se presenta el caso de una paciente con los signos clásicos del síndrome asociado al embarazo, este es el primer comunicado en la literatura nacional y el tercero en las publicaciones médicas internacionales. La evolución del embarazo fue satisfactoria, a edad gestacional de 39 semanas se practicó operación cesárea. Recién nacido de término eutrófico con peso de 2,525 gramos y talla de 47 cm; sin malformaciones congenitas aparentes