Trigonocephaly

Trigonocephaly
Trigonocephaly in a boy with 1q22–1q23.1 duplication
SpecialtyMedical genetics

Trigonocephaly is a congenital condition due to premature fusion of the metopic suture (from Ancient Greek metopon 'forehead'), leading to a triangular forehead. The premature merging of the two frontal bones leads to transverse growth restriction and parallel growth expansion. It may occur as one component of a syndrome together with other abnormalities, or in isolated form. The term is from Ancient Greek trigonon 'triangle' and kephale 'head'.

Cause

Fused suture in trigonocephaly
Trigonocephaly as a kind of craniosynostosis

Trigonocephaly can either occur in a syndrome or isolated, all by itself. Trigonocephaly is associated with the following syndromes: Bohring-Opitz syndrome, Muenke syndrome, Jacobsen syndrome, Baller–Gerold syndrome and Say–Meyer syndrome. The etiology of trigonocephaly is mostly unknown although there are three main theories.[1] Trigonocephaly is probably a multifactorial congenital condition, but due to limited proof of these theories this cannot safely be concluded.[2]

Intrinsic bone malformation

The first theory assumes that the origin of pathological synostosis lies within disturbed bone formation early on in the pregnancy. Causes can either be genetic[3][4][5][6] (9p22–24, 11q23, 22q11, FGFR1 mutation),[7] metabolic[8] (TSH suppletion in hypothyroidism)[9][10] or pharmaceutical[11] (valproate in epilepsy).[12][13]

Fetal-head constraint

The second theory says that synostosis begins when the fetal head gets hindered in the pelvic outlet during birth.[14][15]

Intrinsic brain malformation

The third theory predominates disturbed brain formation of the two frontal lobes as the main issue behind synostosis.[16][17][18] Limited growth of the frontal lobes leads to an absence of stimuli for cranial growth, therefore causing premature fusion of the metopic suture.[citation needed]

Other conditions and syndromes

male
female
Trigonocephaly in two cases of microcephaly

Other conditions and syndromes with trigonocephaly include:

Diagnosis

Diagnosis can be characterized by typical facial and cranial deformities.[2][19]

Observatory signs of trigonocephaly are:

Imaging techniques (3D-CT, Röntgenography, MRI) show:

  • epicanthal folds in limited cases
  • teardrop-shaped orbits angulated towards the midline of the forehead ('surprised coon' sign) in severe cases
  • a contrast difference between a röntgenograph of a normal and a trigonocephalic skull
  • anterior curving of the metopic suture seen from lateral view of the cranium on a röntgenograph
  • a normal cephalic index (maximum cranium width / maximum cranium length) however, there is bitemporal shortening and biparietal broadening

The neuropsychological development is not always affected. These effects are only visible in a small percentage of children with trigonocephaly or other suture synostoses.

Neuropsychological signs are:

Treatment

Treatment is surgical with attention to form and volume. Surgery usually takes place before the age of one since it has been reported that the intellectual outcome is better.[27][28][20][21][22][29][30][31][32][excessive citations]

A. Fronto-supraorbital advancement and remodelling – before remodelling
B. Fronto-supraorbital advancement and remodelling – after remodelling

Fronto-supraorbital advancement and remodelling

A form of surgery is the so-called fronto-supraorbital advancement and remodelling.[33] Firstly, the supraorbital bar is remodelled by a wired greenstick fracture to straighten it. Secondly, the supraorbital bar is moved 2 cm. forward and fixed only to the frontal process of the zygoma without fixation to the cranium. Lastly, the frontal bone is divided into two, rotated and attached to the supraorbital bar causing a nude area (craniectomy) between the parietal bone and frontal bone. Bone will eventually regenerate since the dura mater lies underneath (the dura mater has osteogenic capabilities). This results in an advancement and straightening of the forehead.

'Floating forehead technique'

The so-called 'floating forehead technique'[2] combined with the remodelling of the supraorbital bar is derived from the fronto-supraorbital advancement and remodelling. The supraorbital bar is remodelled as described above.[34][35] The frontal bone is split in two pieces. Instead of using both pieces as in fronto-supraorbital advancement and remodelling, only one piece is rotated and attached to the supraorbital bar. This technique also leaves a craniectomy behind.[36]

Other

The simplest form of surgery for trigonocephaly was suturectomy.[37][21][34][38][39] However, as this technique was insufficient to correct the deformities, it is not used anymore.

Distraction osteogenesis is based on creating more cranial space for the brain by gradually moving the bones apart. This can be achieved by using springs.[40]

These approaches are 2D solutions for a 3D problem, therefore the results are not optimal. Distraction osteogenesis and minimal invasive endoscopic surgery are yet in experimental phase.

Outcomes

Surgical

Trigonocephaly seems to be the most compliant form of craniosynostosis for surgery.[22] Because of standardization of current surgical approaches there is no surgical mortality and complications are few to none.[2][41][42] The simple suturectomy is presently insufficient to adjust the complicated growth restrictions caused by metopic synostosis.[2] On the other hand, the fronto-supraorbital advancement and remodelling and the 'floating forehead technique' create sufficient space for brain growth and result in a normal horizontal axis of the orbits and supraorbital bar. The fronto-supraorbital advancement and remodelling is the most used method nowadays.[41] Over the past few years[when?] distraction osteogenesis has been gradually acknowledged since it has a positive effect on hypotelorism.[citation needed] Expanding the distance between the orbits using springs seems to be successful.[2][43][44][45] However, there are discussions whether hypotelorism really needs to be corrected.[46] The minimal invasive endoscopic surgery has been gaining attention since the early '90s, however, it has technical limitations (only strip craniectomy is possible).[2] Attempts have been made to reach beyond these limits.[47][48][49][50][51]

Aesthetic

Aesthetic outcome of metopic synostosis surgery is persistently good with reoperation hazards below 20%.[52][53] In 1981 Anderson advised that craniofacial operations for synostosis should be as extensive as necessary after a study of 107 cases of metopic and coronal synostosis.[21] Surgery does not provide a 100% natural outcome, mostly there will be minor irregularities. Reoperations are usually performed on more severe cases (including syndromic metopic synostosis). The hypotelorism and temporal hollowing are the most difficult to correct: the hypotelorism usually remains under corrected and a second operation is often needed for correction of temporal hollowing.[52][54]

Neurological

The highest rate of neurological problems of single suture synostosis are seen in patients with trigonocephaly.[2] Surgery is performed generally before the age of one because of claims of better intellectual outcome.[27][28][20][21][22][29][30][31][32] Seemingly surgery does not influence the high incidence of neurodevelopment problems in patients with metopic synostosis. Neurological disorders such as ADHD, ASD, ODD and CD are seen in patients with trigonocephaly. These disorders are usually also associated with decreased IQ. The presence of ADHD, ASD and ODD is higher in cases with an IQ below 85. This is not the case with CD which showed an insignificant increase at an IQ below 85.[2]

Epidemiology

The incidence of metopic synostosis is roughly between 1:700 and 1:15,000 newborns globally (differs per country).[11][55] Trigonocephaly is seen more in males than females ranging from 2:1 to 6.5:1.[37][56][27][28] Hereditary relations in metopic synostosis have been found of which 5.5% were well defined syndromic.[11] Maternal age and a birth weight of less than 2500g may also play a role in trigonocephaly.[57] These data are based on estimations and do not give factual information.

Only one article gives valuable and reliable information regarding the incidence of metopic synostosis in the Netherlands. The incidence in the Netherlands showed an increase from 0.6 (1997) to 1.9 (2007) for every 10,000 live births.[58]

History

In former times people born with malformed skulls were rejected based upon their appearance.[1][59] This still persists today in various parts of the world even though the intellectual development is often normal.[2] The Austrian physician Franz Joseph Gall presented the science of phrenology in the early 19th century through his work The Anatomy and Physiology of the Nervous System in General, and of the Brain in Particular.[60]

Hippocrates described trigonocephaly as follows: Men's heads are by no means all like to one another, nor are the sutures of the head of all men constructed in the same form. Thus, whoever has a prominence in the anterior part of the head (by prominence is meant the round protuberant part of the bone which projects beyond the rest of it), in him the sutures of the head take the form of the Greek letter 'tau', τ.[61][62]

Hermann Welcker coined the term trigonocephaly in 1862. He described a child with a V-shaped skull and a cleft lip.[63]

Via a photo shown on a Facebook page, the mother of a child previously diagnosed with this condition recognised the symptoms and reported them to the family involved, resulting in an immediate diagnosis that medical professionals had overlooked in all earlier consultations.[64]

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ステュムパーロス

ステュムパーロス（古希: Στύμφαλος, Stymphālos）は、ギリシア神話の人物。ステュンパーロス、長母音を省略してステュムパロス、ステュンパロスとも表記される。アルカディアー地方の人物で、主に、 リュカーオーンの子 エラトスの子 が知られている。以下に説明する。 リュカーオーンの子 ステュムパーロスが創建したとされる古代都市ステュムパーロスの遺

 

Файл:Geiyo-shoto-ukr.png

Опис файлу Опис Острови Ґеййо. Японія Джерело http://www.maps-for-free.com/ Час створення 2010.8 Автор зображення я Ліцензія див. нижче Ліцензування: Я, власник авторських прав на цей твір, надаю дозвіл на копіювання, розповсюдження та/або модифікацію даного документу на умовах ліцензії ...

 

حفل توزيع جوائز الأوسكار الأربعون

حفل توزيع جوائز الأوسكار الأربعون الجائزة جوائز الأوسكار التاريخ 10 أبريل، 1968 المكان قاعة عرض سانتا مونيكا المدنية في سانتا مونيكا، كاليفورنيا البلد الولايات المتحدة  المنتج آرثر فريد المضيف بوب هوب الجوائز أفضل فيلم في دفء الليل أفضل مخرج مايك نيكولز أفضل ممثل رود ستا...

Варагаш Ірина Юріївна

Варагаш Ірина Юріївна Народилася 3 квітня 1970(1970-04-03) (53 роки)Трускавець, Львівська область, Українська РСР, СРСР Ірина Юріївна Варагаш (укр. Ірина Юріївна Варагаш, народилася 3 квітня 1970 року, м. Трускавець (УСРР)) — українська підприємниця, засновниця та керівниця низки компа

 

Стура-Лулеваттен

Стура-Лулеваттеншвед. Stora Lulevatten 67°08′39″ пн. ш. 19°35′07″ сх. д. / 67.1443639° пн. ш. 19.5854194° сх. д. / 67.1443639; 19.5854194Розташування Країна  Швеція[5]Регіон ЄллівареЙоккмоккПрибережні країни  ШвеціяГеологічні дані Тип водосховище і lake water bodyd&#...

 

Rowing at the 1988 Summer Olympics

Main article: Rowing at the Summer Olympics Rowingat the Games of the XXIV OlympiadVenueHan River Regatta CourseDate19–25 September 1988No. of events14Competitors593 (399 men, 194 women) from 38 nations← 19841992 → Rowing at the1988 Summer OlympicsSingle scullsmenwomenDouble scullsmenwomenQuadruple scullsmenwomenCoxless pairmenwomenCoxed pairmenCoxless fourmenCoxed fourmenwomenEightmenwomenvte Rowing at the 1988 Summer Olympics in Seoul featured 14 events in ...

Natural Capitalism

1999 book by Hawken, Lovins, & Lovins Natural Capitalism : Creating the Next Industrial Revolution AuthorPaul HawkenAmory B LovinsL Hunter LovinsSubjectEconomic forecasting, capitalism, environmental protectionPublisherLittle, Brown & Company[1]Publication date1999Pagesxix, 396 p.ISBN978-0-316-35316-8Dewey Decimal338.064LC ClassHC106.82 .H39 1999 Natural Capitalism: Creating the Next Industrial Revolution is a 1999 book on environmental economics co-authored by Paul ...

 

Nightmare Alley (novel)

1946 novel by William Lindsay Gresham Nightmare Alley First edition coverAuthorWilliam Lindsay GreshamCountryUnited StatesLanguageEnglishPublisherRinehart & CompanyPublication date1946Media typePrint (hardback & paperback) Nightmare Alley is a novel by William Lindsay Gresham published in 1946. It is a study of the lowest depths of showbiz and its sleazy inhabitants—the dark, shadowy world of a second-rate carnival filled with hustlers, scheming grifters, and Machiavellian femm...

 

Дуриба Ярослав Васильович

Дуриба Ярослав Васильович  Старший солдат Загальна інформаціяНародження 21 вересня 1973(1973-09-21)ДогмарівкаСмерть 15 травня 2015(2015-05-15) (41 рік)Станиця ЛуганськаПсевдо «Ярік»Військова службаПриналежність  УкраїнаВид ЗС Сухопутні військаРід військ  Танкові військаФорм...

Sunset Hospital

Former hospital in Dalkeith, Western Australia This article needs to be updated. Please help update this article to reflect recent events or newly available information. (November 2016)Hospital in Western Australia, AustraliaSunset HospitalSunset Hospital in May 2020GeographyLocationDalkeith, City of Nedlands, Western Australia, AustraliaCoordinates32°00′05″S 115°48′13″E / 32.001505°S 115.803719°E / -32.001505; 115.803719 (Sunset Hospital)HistoryFor...

 

The Pioneer Woman (TV series)

American cooking television series This article needs to be updated. Please help update this article to reflect recent events or newly available information. (November 2019) The Pioneer WomanGenreFood reality televisionDirected byOlivia BallStarringRee DrummondCountry of originUnited StatesOriginal languageEnglishNo. of seasons29No. of episodes400+ (as of October 2021)[1]ProductionExecutive producerRachel PurnellProducerPacific ProductionsProduction locationsPawhuska, Oklahoma, United...

 

Kongres Filipina

Kongres Filipina Kongreso ng PilipinasKongres Filipina ke-17 JenisJenisBikameral MajelisSenatDewan Perwakilan RakyatPimpinanPresiden SenatAquilino Pimentel III sejak 25 Juli 2016 Ketua Dewan Perwakilan RakyatGloria Macapagal Arroyo sejak 23 Juli 2018 KomposisiAnggota321 24 senator 297 anggota dewan perwakilanPartai & kursi Senat Daftar   Liberal (6)   PDP-Laban (3)   NPC (3)   UNA (2)   Nacionalista (2)   Akbayan (1)   LDP (1)   PMP (1)   ...

Makanan Tabu bagi Masyarakat Jeneponto

Artikel ini sebatang kara, artinya tidak ada artikel lain yang memiliki pranala balik ke halaman ini.Bantulah menambah pranala ke artikel ini dari artikel yang berhubungan atau coba peralatan pencari pranala.Tag ini diberikan pada Oktober 2022. Makanan tabu bagi masyarakat Jeneponto (salah satu daerah di Sulawesi Selatan) adalah suatu larangan dalam mengonsumsi makanan tertentu karena ada beberapa ancaman atau hukuman kepada masyarakat yang mengonsumsinya. Dalam ancaman ini, ada kekuatan supr...

 

Ishtar TV

Ishtar TVTypeSatellite television networkCountryIraqAvailabilityMiddle EastOwnerChaldean Syriac Assyrian Popular CouncilKey peopleSarkis Aghajan MamendoLaunch date2005 (2005)Official websitewww.ishtartv.com Ishtar TV (Syriac: ܥܫܬܪ, after the Assyro-Babylonian goddess, Ishtar) is an Assyrian broadcasting channel which has its headquarters in Ankawa, Iraq. It was established by Sarkis Aghajan and was led by George Mansour, who was Ishtar TV's first General Manager, in 2005. The network...

 

Nzema people

Ethnic group in Ghana and Ivory Coast NzemaTotal population~500,000Regions with significant populationsWestern Region of Ghana, Ivory CoastLanguagesNzema, English, FrenchReligionChristianity, African Traditional Religion, IslamRelated ethnic groupsAkan The Nzema are an Akan people numbering about 328,700, of whom 262,000 live in southwestern Ghana and 66,700 live in the southeast of Côte d'Ivoire. In Ghana the Nzema area is divided into three electoral districts: Nzema East Municipal, also k...

RealSports Football

1982 video game 1982 video gameRealSports FootballDeveloper(s)Atari, Inc.Publisher(s)Atari, Inc.Designer(s)Robert ZdybelProgrammer(s)Atari 2600Robert ZdybelAtari 8-bit / 5200Jim HeutherSeriesRealSportsPlatform(s)Atari 2600, Atari 5200, Atari 8-bitReleaseNA: December 1982[1] 1983: Atari 8-bit, 5200Genre(s)SportsMode(s)Single-player, multiplayer RealSports Football is a 1982 American football video game made by Atari, Inc. for the Atari 2600. Versions for the Atari 5200 and Atari 8-bit ...

 

Waktu Standar Pakistan

Waktu Standar Pakistan (PKT) adalah zona waktu untuk Pakistan. Zona waktu itu 5 jam lebih dulu dari GMT/UTC (UTC+5). Zona waktu standar di dunia. lbsWaktu di AsiaNegaraberdaulat Afganistan Arab Saudi Armenia1 Azerbaijan1 Bahrain Bangladesh Bhutan Brunei Filipina Georgia1 India Indonesia Irak Iran Israel Jepang Kamboja Kazakhstan3 Kirgizstan Korea Selatan Korea Utara Kuwait Laos Lebanon Maladewa Malaysia Mesir3 Mongolia Myanmar Nepal Oman Pakistan Qatar Rusia3 Singapura Siprus1 Sri Lanka Suria...

 

The Winning of Barbara Worth

1926 American silent Western film by Henry King The Winning of Barbara WorthTheatrical release posterDirected byHenry KingWritten byFrances MarionBased onThe Winning of Barbara Worthby Harold Bell WrightProduced bySamuel GoldwynStarring Ronald Colman Vilma Bánky Gary Cooper Cinematography George Barnes Thomas BraniganGregg Toland Edited byViola LawrenceMusic byTed HenkelProductioncompanyThe Samuel Goldwyn CompanyDistributed byUnited ArtistsRelease date October 14, 1926 (1926-1...

Mikhail Kalashnikov

Netralitas artikel ini dipertanyakan. Diskusi terkait dapat dibaca pada the halaman pembicaraan. Jangan hapus pesan ini sampai kondisi untuk melakukannya terpenuhi. (Pelajari cara dan kapan saatnya untuk menghapus pesan templat ini) Mikhail KalashnikovPFR PBSKalashnikov di Kremlin,Desember 2009Nama asalМихаил Тимофеевич КалашниковLahirMikhail Timofeyevich Kalashnikov(1919-11-10)10 November 1919Kurya, Krai Altai, RSFS RusiaMeninggal23 Desember 2013(2013-12-23) (u...

 

S.C. Damash Gilan

Iranian football club This article uses bare URLs, which are uninformative and vulnerable to link rot. Please consider converting them to full citations to ensure the article remains verifiable and maintains a consistent citation style. Several templates and tools are available to assist in formatting, such as reFill (documentation) and Citation bot (documentation). (September 2022) (Learn how and when to remove this template message) Football clubDamash Gilanداماش گیلانFull nameSpo...

 

Strategi Solo vs Squad di Free Fire: Cara Menang Mudah!