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"Plagiocephaly, also known as flat head syndrome, is a condition characterized by an asymmetrical distortion (flattening of one side) of the skull. It is characterized by a flat spot on the back or one side of the head caused by remaining in a supine position for prolonged periods. Plagiocephaly is a diagonal asymmetry across the head shape. Often it is a flattening which is to one side at the back of the head and there is often some facial asymmetry. Plagiocephaly divides into two groups: synostotic plagiocephaly, with one or more fused cranial sutures, and nonsynostotic (deformational) plagiocephaly. Surgical treatment of these groups includes the deference method; however, the treatment of deformational plagiocephaly is controversial. Brachycephaly describes a very wide head shape with a flattening across the whole back of the head. Causes Left posterior positional plagiocephaly in a baby Slight plagiocephaly is routinely diagnosed at birth and may be the result of a restrictive intrauterine environment giving a "diamond" shaped head when seen from above. If there is premature union of skull bones, this is more properly called craniosynostosis. The incidence of plagiocephaly has increased dramatically since the advent of anti-sudden infant death syndrome recommendations for parents to keep their babies on their backs. Data also suggest that the rates of plagiocephaly is higher among twins and multiple births, premature babies, babies who were positioned in the breech position or back-to-back, as well as babies born after a prolonged labour. Diagnosis A developmental and physical assessment performed by a physician or a pediatric specialist is recommended. Often imaging is obtained if the diagnosis is questionable to see if the baby's sutures are present or not. If the sutures are not present, craniosynostosis may be ruled into question. Prevention Prevention methods include carrying the infant and giving the infant time to play on their stomach (tummy time), which may prevent the baby from progressing into moderate or severe plagiocephaly. Letting babies crawl may also prove to be crucial in preventing plagiocephaly as it strengthens babies' spine and neck muscles. Crawling also boosts gross and fine motor skills (large and refined movements), balance, hand-eye-coordination and overall strength. Treatment The condition may improve to some extent as the baby grows, but in some cases, home treatment or physical therapy treatment can improve the shape of a baby’s head. Early interventions (based on the severity) are of importance to reduce the severity of the degree of the plagiocephaly. Diagnosis is most commonly determined through clinical examination. In order to assess the severity of the condition and determine the best course of treatment, practitioners often use the Plagiocephaly Severity Scale. This is a scale that can help practitioners evaluate the condition in a standardized way. The course of treatment is typically based on the age of the child when the diagnosis is made in conjunction with the severity of the diagnosis. If a diagnosis of mild to moderate plagiocephaly occurs before four months of age, repositioning therapy may be helpful. If the diagnosis is determined to be severe, practitioners will likely prescribe a cranial molding orthosis (helmet), which has the best results when prescribed between five to six months of age. =Repositioning= Initially, treatment usually takes the form of reducing the pressure on the affected area through repositioning of the baby onto their abdomen for extended periods of time throughout the day. This may include repositioning the child's head throughout the day so that the rounded side of the head is placed against the mattress, re-positioning cribs and other areas that infants spend time in so that they will have to look in a different direction to see their parents or others in the room, re-positioning mobiles and other toys for similar reasons, and avoiding extended time sleeping in car-seats (when not in a vehicle), bouncy seats, or other supine seating which is thought to exacerbate the problem. If the child appears to have discomfort or cries when they are re-positioned, a neck problem should be ruled out. =Helmets= High quality evidence is lacking for cranial remolding orthosis (baby helmet) for the positional condition and use for this purpose is controversial. If conservative treatment is unsuccessful, helmets may help to correct abnormal head shapes. These helmets are used to treat deformational plagiocephaly, brachycephaly, scaphocephaly and other head shape deformities in infants 3–18 months of age by gently allowing the head shape to grow back into a normal shape. This type of treatment has been used for severe deformations. Prognosis Preliminary research indicates that some babies with plagiocephaly may comprise a high- risk group for developmental difficulties. Plagiocephaly is associated with motor and language developmental delays. While developmental delay is more commonplace among babies with plagiocephaly, it cannot be inferred that plagiocephaly is the cause of the delay. Etymology Ancient Greek πλάγιος (plagios) "oblique, slanting," from PIE plag- "flat, spread," from *plak, and cephal- Modern Latin "head, skull, brain," (from Greek κεφαλή) together means “flat head”. See also * Artificial cranial deformation * Yakovlevian torque References External links Category:Congenital disorders of musculoskeletal system "
"Scaphocephaly is a type of cephalic disorder which occurs when there is a premature fusion of the sagittal suture. The sagittal suture joins together the two parietal bones of skull. Scaphocephaly is the most common of the craniosynostosis conditions and is characterized by a long, narrow head. Treatment This condition can be corrected by surgery if the child is young enough. The use of a cranial remolding orthosis can also benefit the child if the child begins wearing it at an early age. Terminology The term is from Greek skaphe meaning 'light boat or skiff' and kephale meaning 'head') describes a specific shape of a long narrow head that resembles an inverted boat. See also * Dolichocephaly References External links * NINDS Overview Category:Congenital disorders of musculoskeletal system "
"Trigonocephaly is a congenital condition of premature fusion of the metopic suture (from Greek metopon, "forehead"), leading to a triangular forehead. The merging of the two frontal bones leads to transverse growth restriction and parallel growth expansion. It may occur syndromic, involving other abnormalities, or isolated. The term is from Greek trigonon, "triangle", and kephale, "head". Cause Fused suture in trigonocephaly Trigonocephaly as a kind of craniosynostosis Trigonocephaly can either occur syndromatic or isolated. Trigonocephaly is associated with the following syndromes: 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. Trigonocephaly is probably a multifactorial congenital condition, but due to limited proof of these theories this cannot safely be concluded. =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 geneticFrydman M, Kauschansky A, Elian E. Trigonocephaly; a new familiail syndrome. Am J Med Genet 1984: 18: 55-9. (9p22–24, 11q23, 22q11, FGFR1 mutation), metabolic (TSH suppletion in hypothyroidism) or pharmaceutical (valproate in epilepsy). =Fetal-head constraint= The second theory says that synostosis begins when the fetal head gets hindered in the pelvic outlet during birth. =Intrinsic brain malformation= The third theory predominates disturbed brain formation of the two frontal lobes as the main issue behind synostosis. Limited growth of the frontal lobes leads to an absence of stimuli for cranial growth, therefore causing premature fusion of the metopic suture. Diagnosis Diagnosis can be characterized by typical facial and cranial deformities. Observatory signs of trigonocephaly are: *a triangular forehead seen from top view leading to a smaller anterior cranial fossa *a visible and palpable midline ridge *hypotelorism inducing ethmoidal hypoplasia 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: *problems in behaviour, speech and language *mental retardation *neurodevelopmental delays such as attention deficit hyperactivity disorder (ADHD), oppositional defiant disorder (ODD), autism spectrum disorder (ASD), and conduct disorder (CD). Many of these delays become evident at school age. 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. =Fronto-supraorbital advancement and remodelling= A form of surgery is the so-called fronto-supraorbital advancement and remodelling. 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' 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. 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. =Other= *Suturectomy *Distraction osteogenesis *Minimal invasive endoscopic surgery 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. Because of standardization of current surgical approaches there is no surgical mortality and complications are few to none.Galassi E, Giulioni M, Acciarri N, Cavina C, Pistorale T. Marchac procedure in the early treatment of metopic and coronal synostoses. Presented at the Consensus Conference on Craniosynostoses, Rome 1995. The simple suturectomy is presently insufficient to adjust the complicated growth restrictions caused by metopic synostosis. 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. Over the past few years distraction osteogenesis has been gradually acknowledged since it has a positive effect on hypotelorism. Expanding the distance between the orbits using springs seems to be successful. However, there are discussions whether hypotelorism really needs to be corrected. The minimal invasive endoscopic surgery has been gaining attention since the early '90s, however, it has technical limitations (only strip craniectomy is possible). Attempts have been made to reach beyond these limits. =Aesthetic= Aesthetic outcome of metopic synostosis surgery is persistently good with reoperation hazards below 20%. 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. 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. =Neurological= The highest rate of neurological problems of single suture synostosis are seen in patients with trigonocephaly. Surgery is performed generally before the age of one because of claims of better intellectual outcome. 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. Epidemiology The incidence of metopic synostosis is roughly between 1:700 and 1:15,000 newborns globally (differs per country). Trigonocephaly is seen more in males than females ranging from 2:1 to 6.5:1. Hereditary relations in metopic synostosis have been found of which 5.5% were well defined syndromic. Maternal age and a birth weight of less than 2500g may also play a role in trigonocephaly. 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. History In former times people born with malformed skulls were rejected based upon their appearance.Strickler M, van der Meulen J, Rahael B, Mazolla R. Craniofacial malformations. Edinburgh, London, Melbourne, New York: Churchill Livingston, 1990. This still persists today in various parts of the world even though the intellectual development is often normal. 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.Phrenology. http://www.phrenology.org/ 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.Hippocrates. On injuries of the head. Med classics 1938: 3: 145-60. Hermann Welcker coined the term trigonocephaly in 1862. He described a child with a V-shaped skull and a cleft lip.Welcker H. Untersugungen uber wachtsum und bau des menschlischen Schädels. Leipzig: Engelmann, 1862. Popular culture 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. References External links Category:Congenital disorders of musculoskeletal system "