Biomaterial Database

Hanhart's syndrome with special reference to temporal bone findings. 1987

H K Sekhar, and M Sachs, and V C Siverls

We report two cases of Hanhart's syndrome. The first patient was a male who died in the neonatal period, and whose temporal bones were removed, processed, and histologically examined; the findings are presented in this article. The second case concerns a living patient with the typical characteristics of the syndrome. An attempt is made to show that a gross first and second branchial arch anomaly exists in this syndrome and not just the micrognathia that is obvious to the onlooker. Possible causes and classifications of facial dysmorphia are discussed.

UI	MeSH Term	Description	Entries
D007231	Infant, Newborn	An infant during the first 28 days after birth.	Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008297	Male		Males
D008342	Mandibulofacial Dysostosis	A hereditary disorder occurring in two forms: the complete form (Franceschetti's syndrome) is characterized by a slant of the palpebral fissures, COLOBOMA of the lower lid, MICROGNATHIA and hypoplasia of the ZYGOMATIC ARCHES, and CONGENITAL MICROTIA. It is transmitted as an autosomal trait. The incomplete form (Treacher Collins syndrome) is characterized by the same anomalies in less pronounced degree. It occurs sporadically, but an autosomal dominant mode of transmission is suspected. (Dorland, 27th ed)	MFD1 Mandibulofacial Dysostosis,Treacher Collins Syndrome,Franceschetti-Zwahlen-Klein Syndrome,Mandibulofacial Dysostosis (MFD1),Treacher Collins-Franceschetti Syndrome,Dysostoses, MFD1 Mandibulofacial,Dysostoses, Mandibulofacial,Dysostoses, Mandibulofacial (MFD1),Dysostosis, MFD1 Mandibulofacial,Dysostosis, Mandibulofacial,Dysostosis, Mandibulofacial (MFD1),Franceschetti Zwahlen Klein Syndrome,Franceschetti-Zwahlen-Klein Syndromes,MFD1 Mandibulofacial Dysostoses,Mandibulofacial Dysostoses,Mandibulofacial Dysostoses (MFD1),Mandibulofacial Dysostoses, MFD1,Mandibulofacial Dysostosis, MFD1,Syndrome, Franceschetti-Zwahlen-Klein,Syndrome, Treacher Collins,Syndrome, Treacher Collins-Franceschetti,Syndromes, Franceschetti-Zwahlen-Klein,Syndromes, Treacher Collins-Franceschetti,Treacher Collins Franceschetti Syndrome,Treacher Collins-Franceschetti Syndromes
D008844	Micrognathism	Abnormally small jaw.	Congenital Micrognathia,Congenital Micrognathism,Mandibular Micrognathia,Mandibular Micrognathism,Micrognathia,Congenital Micrognathias,Congenital Micrognathisms,Mandibular Micrognathias,Mandibular Micrognathisms,Micrognathia, Congenital,Micrognathia, Mandibular,Micrognathias,Micrognathias, Congenital,Micrognathias, Mandibular,Micrognathism, Congenital,Micrognathism, Mandibular,Micrognathisms,Micrognathisms, Congenital,Micrognathisms, Mandibular
D001934	Branchial Region	A region, of SOMITE development period, that contains a number of paired arches, each with a mesodermal core lined by ectoderm and endoderm on the two sides. In lower aquatic vertebrates, branchial arches develop into GILLS. In higher vertebrates, the arches forms outpouchings and develop into structures of the head and neck. Separating the arches are the branchial clefts or grooves.	Branchial Arches,Branchial Clefts,Pharyngeal Arches,Visceral Arches,Branchial Arch,Branchial Grooves,Pharyngeal Arch,Pharyngeal Clefts,Pharyngeal Grooves,Visceral Arch,Arch, Branchial,Arch, Pharyngeal,Arch, Visceral,Arches, Branchial,Arches, Pharyngeal,Arches, Visceral,Archs, Pharyngeal,Branchial Cleft,Branchial Groove,Branchial Regions,Cleft, Branchial,Cleft, Pharyngeal,Clefts, Branchial,Clefts, Pharyngeal,Groove, Branchial,Groove, Pharyngeal,Grooves, Branchial,Grooves, Pharyngeal,Pharyngeal Archs,Pharyngeal Cleft,Pharyngeal Groove,Region, Branchial,Regions, Branchial
D004432	Ear, Middle	The space and structures directly internal to the TYMPANIC MEMBRANE and external to the inner ear (LABYRINTH). Its major components include the AUDITORY OSSICLES and the EUSTACHIAN TUBE that connects the cavity of middle ear (tympanic cavity) to the upper part of the throat.	Tympanic Cavity,Tympanum,Middle Ear,Cavities, Tympanic,Cavity, Tympanic,Ears, Middle,Middle Ears,Tympanic Cavities,Tympanums
D004480	Ectromelia	Gross hypo- or aplasia of one or more long bones of one or more limbs. The concept includes amelia, hemimelia, phocomelia, and sirenomelia.	Amelia,Congenital Limb Reduction Deformities,Congenital Limb Reduction Deformity,Hemimelia,Phocomelia,Sirenomelia,Fused Legs and Feet,Mermaid Malformation,Mermaid Syndrome,Sirenomelia Sequence,Sirenomelus,Malformation, Mermaid,Sequence, Sirenomelia,Sirenomelia Sequences,Syndrome, Mermaid
D005154	Facial Nerve	The 7th cranial nerve. The facial nerve has two parts, the larger motor root which may be called the facial nerve proper, and the smaller intermediate or sensory root. Together they provide efferent innervation to the muscles of facial expression and to the lacrimal and SALIVARY GLANDS, and convey afferent information for TASTE from the anterior two-thirds of the TONGUE and for TOUCH from the EXTERNAL EAR.	Cranial Nerve VII,Marginal Mandibular Branch,Marginal Mandibular Nerve,Seventh Cranial Nerve,Nerve VII,Nerve of Wrisberg,Nervus Facialis,Nervus Intermedius,Nervus Intermedius of Wrisberg,Cranial Nerve VIIs,Cranial Nerve, Seventh,Facial Nerves,Mandibular Nerve, Marginal,Mandibular Nerves, Marginal,Marginal Mandibular Nerves,Nerve VIIs,Nerve, Facial,Nerve, Marginal Mandibular,Nerve, Seventh Cranial,Nerves, Marginal Mandibular,Nervus Faciali,Seventh Cranial Nerves,Wrisberg Nerve,Wrisberg Nervus Intermedius
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths