Hallermann streiff syndrome nord national organization for. Hallermannstreiff syndrome genetic and rare diseases. Pdf a case of hallermannstreiff syndrome with aphakia. The syndrome is characterized by proportionate short stature, craniofacial dysostoses consisting of skeletal, ophthalmologic, and cutaneous defects. Hallermann streiff syndrome is a congenital disorder that affects growth, cranial development, hair growth and dental development. Hallermannstreiff syndrome is frequently characterized by dental abnormalities. Hallermann streiff syndrome is a congenital disorder involving glaucoma in both eyes, microphthalmia smallerthannormal eyes, dwarfism, lowerthannormal amount of body hair, a characteristic beakshaped nose, micrognathia, dental abnormalities, and increased upper airway resistance. Cardinal features include craniofacial dysmorphia and upper airway abnormalities. Here you can see if hallermann streiff syndrome can be hereditary. Hallerman streiff syndrome joseph junewick, md facr 09162010 history 2 year old with developmental delay.
Hallermannstreiff syndrome is a very rare disorder. Metyrapone test confirmed acth deficiency as a contributing factor to. It affects both male and female in all ethnic groups 1, 2. Background hallermann in 1948 and streiff in 1950 described patients characterised by bird face, congenital cataract, mandibular hypoplasia, and dental abnormalities. Hallermannstreiff syndrome medical condition youtube. Other articles where hallermanstreifffrancois syndrome is discussed.
Signs and symptoms or characteristics of this condition are a short stature. Hallermannstreiff syndrome associated with small cerebellum, endocrinopathy and increased chromosomal. May 17, 2015 hallermannastreiff syndrome is a congenital disorder that affects growth, cranial development, hair growth and dental development this video contains general medical information if in doubt. Hallermannstreiff syndrome patient treated with removable. Apr 10, 2020 hallermann streiff syndrome hss is a rare condition with characteristic features that are present at birth and become more apparent over time. Hallermann streiff syndrome is a rare congenital disorder characterized by birdlike facies and dental anomalies. These are genetic disorders that may be inherited in an autosomal. It is a rare genetic condition and is also known as francois dyscephaly syndrome 3. Hallermannstreiff is a rare syndrome characterized by multiple congenital anomalies, especially in the head and face. Hallermann streiff syndrome is characterized by a typical skull shape brachycephaly with frontal bossing, hypotrichosis, microphthalmia, cataracts, beaked nose.
Discussion hallermann streiff syndrome hss is a rare congenital anomaly that was first identified in 1893 and. The medical community discovered it in the late 1800s. A third condition, hallermanstreifffrancois syndrome, is characterized by the presence of progeria in combination with dwarfism and other features of abnormal growth. Learn indepth information on hallermann streiff syndrome, its causes, symptoms, diagnosis, complications, treatment, prevention, and prognosis. E2368 march 2009 with 432 reads how we measure reads. A case report from turkey article pdf available in medicina oral, patologia oral y cirugia bucal 145. Hallermannstreiff syndrome hss is a rare genetic disorder that is primarily characterized by distinctive malformations of the skull and facial craniofacial.
Hallermanstreifffrancois syndrome pathology britannica. It was first described by hallermann in 1948 and later by streiff in 1950. Hallermann streiff syndrome was independently described by hallermann in 1948 and streiff in 1950. The icd10cm alphabetical index is designed to allow medical coders to look up various medical terms and connect them with the appropriate icd codes. A case of hallermannstreiff syndrome with aphakia article pdf available in korean journal of pediatrics 516 january 2008 with 84 reads how we measure reads. Congenital cataract, hallermann streiff syndrome, microphthalmos, retinal fold, ultrasound biomicroscopy. Endocrine evaluation showed a low t4 and tsh levels, suggesting hypothalamic hypothyroidism. The hallermann streiff syndrome mandibulooculofacial dyscephaly omim 234100 is a branchial arch syndrome which combines a characteristic birdlike facies with ocular abnormalities and alopecia, which may have an unusual sutural distribution on the scalp. Absence agenesis or malformation of the tissue connecting the left and right halves of the brain corpus callosum also occurs in most people with this disorder. Hallermannstreiff syndrome definition of hallermann. Hallermannstreiff syndrome hss, or oculomandibulodyscephaly, is a rare congenital anomaly, characterized by dyscephaly with bird facies, frontal or parietal bossing, hypotrichosis of scalp, eyebrows and eyelashes, cutaneous atrophy of scalp and nose, mandibular hypoplasia, forward displacement of temporomandibular joints, high arched plate, small mouth, multiple dental anomalies and.
These signs are dyscephalia and bird face, dental abnormalities, proportionate short stature, hypotrichosis, atrophy of skin especially on nose, bilateral microphthalmos, and. Hallermannstreiff syndrome is a rare genetic disorder characterized by craniofacial malformations, sparse hair, eye. Hallermannstreifffrancois syndrome, familial cataract, dysmorphic features, rare, cameroon. Nonlethal hallermannstreiff syndrome with bone fracture.
Mim234100 a syndrome of bony anomalies of the calvaria, face, and jaw, with brachygnathia, narrow curved nose, and multiple ocular defects including microphthalmia, microcornea, and cataract, often with alopecia overlying cranial sutures, or alopecia areata, or absence of eyebrows. Hallermannstreiff syndrome is a congenital disorder that affects growth, cranial development, hair growth and dental development. Introduction the hallermann streiff syndrome is a rare congenital anomaly first described by hallermann 1 and streiff. A familial study of hallermannstreifffrancois syndrome e epee,1 d beleho,2 at. The new syndrome was later defined as hallermannstreiff syndrome hss.
Hallermann streiff syndrome occurs sporadically and is not associated with chromosomal anomalies. Hallermann streiff syndrome hss is a rare disorder that is primarily characterized by distinctive malformations of the skull and facial craniofacial region. Please remove adblock adverts are the main source of revenue for dovemed. Hallermannstreiff syndrome is a rare, congenital condition characterized mainly by abnormalities of the skull and facial bones. What is the abbreviation for hallermann streiff syndrome. Hallermann streiff syndromethe oral manifestations in a child. This means that hallermann streiff syndrome, or a subtype of hallermann streiff syndrome, affects less than 200,000 people in the us population. Signs and symptoms include an unusually shaped skull, distinctive facial features, thin skin and hair, and eye and dental abnormalities. These may include the eruption of teeth before or shortly after birth natal or neonatal teeth, which may be misdiagnosed as supernumerary teeth. Hallermannstreiff syndrome how is hallermannstreiff. There are fewer than 200 people with the syndrome worldwide. Diagnosis hallerman streiff syndrome additional clinical history of congenital cataract resection. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree.
Hallermann streiff syndrome an overview sciencedirect. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for hallermannstreiff. An oculomandibulofacial syndrome principally characterized by dyscephaly usually brachycephaly, parrot nose, mandibular hypoplasia, proportionate nanism, hypotrichosis, bilateral congenital cataracts, and microphthalmia. Dyscephaly can be defined as malformation of the cranium and bones of the face. A description of a 21yearold hispanic woman with hallermann streiff syndrome, whose anterior open occlusal relationship and smile were improved with overdentures is presented. What is the life expectancy of someone with hallermann. Clinical and experimenta dermatology, 29, 477479 17. Hallermanstrieff syndrome hss, occulomandibulodyscephaly was first described completely in 1948 by hallermann and then in 1950 by streiff. Hallermann streiff syndrome hss is a rare genetic condition which involves multiple congenital abnormalities chiefly affecting the head and the face. There are 0 terms under the parent term hallerman streiff syndrome in the icd10cm alphabetical index. Ultrasound biomicroscopic findings in hallermanstreiff syndrome. Hallermannstreiff syndrome hss is a rare disorder characterized primarily by head and face abnormalities, with dental abnormalities also present in 5080 percent of cases. Around 150 cases have been reported in literature world wide.
For language access assistance, contact the ncats public information officer. May 06, 2020 hallermann streiff syndrome gets its name from wilhelm hallermann and enrico streiff, the men who made the first reports about patients with the condition. Hallermannstreiff syndrome pediatric oncall journal. Sep 07, 2018 hallermann streiff syndrome is a rare congenital condition that also is referred to as hss or hallermann streiff fransois syndrome. Most relevant lists of abbreviations for hss hallermann streiff syndrome. Hallerman streiff syndrome icd10cm alphabetical index. Hallermannstreiff syndrome hss is a very rare genetic disorder that has a characteristic facial appearance, dental abnormalities.
Hss abbreviation stands for hallermann streiff syndrome. National organization of rare diseases webbased medical brief description of the clinical featurescharacteristics, medical complications and genetics of this condition. Jun 02, 2016 a familial study of hallermannstreifffrancois syndrome e epee,1 d beleho,2 at bitang,3 va njami,4 c bengondo,5 come ebana mvogo1 1ophthalmology department, yaounde university teaching hospital, yaounde, cameroon. Hallermann streiff syndrome nord national organization. Icd10 code of hallermann streiff syndrome and icd9 code. These signs are dyscephalia and bird face, dental abnormalities, proportionate short stature, hypotrichosis, atrophy of skin especially on nose, bilateral microphthalmos, and congenital cataract. If you have problems viewing pdf files, download the latest version of adobe reader. Hallermann streiff syndrome is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih. Hallermannstreiff syndrome hss is a rare disorder characterized by dyscephalia, with facial and dental abnormalities.
Full text a familial study of hallermannstreifffrancois syn imcrj. Hallermannstreiff syndrome a case report from egypt. Does any member of your family have hallermann streiff syndrome or may be more predisposed to developing t. Streiff syndrome with hypopituitarism contributing. Intellectual disability is present in some individuals. Brock the star hes literally 1 in 50 million hallermann streiff syndrome. Hallermannstreiff syndrome also known as the francois dyscephalic syndrome, hallermannstreifffrancois syndrome, oculomandibulodyscephaly with hypotrichosis and oculomandibulofacial syndrome is a congenital disorder that affects growth, cranial development, hair growth and dental development. Hallermann 1948 and streiff 1950 reported patients with dyscephaly, a birdlike face, congenital cataracts, and microphthalmia. Andermann syndrome is a disorder that damages the nerves used for muscle movement and sensation motor and sensory neuropathy. Hallermannstreiff syndrome genetic and rare diseases nih. Seven essential signs described by francois as diagnostic criteria for hss. Hallermannstreifffrancois hsf syndrome is marked by a characteristic facies with hypoplastic mandible and beaked nose, proportionate. Francois 1958 identified similar reported cases with the additional features of hypotrichosis, skin atrophy, dental anomalies, and short stature.
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