Editing Plagiocephaly

{{Short description|Skull malformation such that one side is flattened}}
{{Redirect|Flat head syndrome|the condition of an unusually short skull|Brachycephaly}}
{{Infobox medical condition (new)
| name          = Plagiocephaly
| synonyms      = Flat head syndrome
| image         = Plagiocephalus.jpg
| caption       = Patient with plagiocephaly and [[wry neck]]
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'''Plagiocephaly''', also known as '''flat head syndrome''',<ref name="AJR 194">{{cite journal |doi=10.2214/AJR.07.7121 |title=Radiological Reasoning: A Child with Posterior Plagiocephaly |year=2010 |last1=Kadom |first1=Nadja |last2=Sze |first2=Raymond W. |journal=American Journal of Roentgenology |volume=194 |issue=3 Suppl |pages=WS5–9 |pmid=20173180}}</ref><ref>{{cite web |url=http://losangeles.cbslocal.com/2013/04/30/doctor-finds-success-in-treating-infants-with-flat-head-syndrome/ |title=Doctor Finds Success In Treating Infants With Flat-Head Syndrome |publisher=CBS Los Angeles |date=April 30, 2013 |access-date=November 11, 2013}}</ref> is a condition characterized by an asymmetrical distortion (flattening of one side) of the [[human skull|skull]]. A mild and widespread form 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.<ref name="Pediatrics 128">{{cite journal |doi=10.1542/peds.2011-2220 |title=Prevention and Management of Positional Skull Deformities in Infants |year=2011 |last1=Laughlin |first1=J. |last2=Luerssen |first2=T. G. |last3=Dias |first3=M. S. |journal=Pediatrics |volume=128 |issue=6 |pages=1236–41 |pmid=22123884 |author4=Committee On Practice Ambulatory Medicine|doi-access=free }}</ref>

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. Depending on whether [[synostosis]] is involved, plagiocephaly divides into two groups: synostotic, with one or more fused cranial sutures, and non-synostotic (deformational). Surgical treatment of these groups includes the deference method; however, the treatment of deformational plagiocephaly is controversial.<ref>{{cite journal |doi=10.1136/adc.86.3.144 |title=Plagiocephaly and head binding |journal=Archives of Disease in Childhood |volume=86 |issue=3 |pages=144–145 |year=2002 |last1=Bridges |first1=S J |pmid=11861226 |pmc=1719136 }}</ref> [[Brachycephaly]] describes a very wide head shape with a flattening across the whole back of the head.

==Causes==
Slight plagiocephaly is routinely diagnosed at birth and may be the result of a restrictive [[uterus|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]].<ref name=pmid22920774>{{cite journal |doi=10.1016/j.pedhc.2011.10.002 |pmid=22920774 |title=Evidence-Based Care of the Child with Deformational Plagiocephaly, Part II: Management |journal=Journal of Pediatric Health Care |volume=26 |issue=5 |pages=320–331 |year=2012 |last1=Flannery |first1=Amanda B. Kack |last2=Looman |first2=Wendy S |last3=Kemper |first3=Kristin |doi-access=free }}</ref>

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.<ref name="PedView">{{cite web |title=Plagiocephaly and related cranial deformities |website= childrenshospital.org |date=April 2010 |url= http://www.childrenshospital.org/views/april10/plagiocephaly_and_related_cranial_deformities.html |publisher=Pediatric Views, Children's Hospital Boston |archive-url= https://web.archive.org/web/20110622104213/http://www.childrenshospital.org/views/april10/plagiocephaly_and_related_cranial_deformities.html |archive-date=June 22, 2011 |access-date=November 11, 2013}}</ref>

Data also suggest that the rates of plagiocephaly are higher for twins and multiple births, premature babies, babies who were positioned in the breech position or back-to-back, as well as for babies born after a prolonged labour.<ref>{{Cite web|url=http://www.nhs.uk/conditions/plagiocephaly/Pages/Introduction.aspx|title=Plagiocephaly and brachycephaly (flat head syndrome) |work= NHS Choices|last= |first= |via= nhs.uk| publisher= National Health Service |access-date=2016-05-30}}</ref>
=== Conditions and syndromes ===
Plagiocephaly is seen in multiple conditions:<ref>{{Cite web |title=Plagiocephaly[Clinical Features] - MedGen - NCBI |url=https://www.ncbi.nlm.nih.gov/medgen?term=Plagiocephaly%5BClinical+Features%5D&cmd=DetailsSearch |access-date=2023-10-29 |website=www.ncbi.nlm.nih.gov}}</ref>
* [[Arthrogryposis]], [[Cleft lip and cleft palate|cleft palate]], craniosynostosis, and impaired [[Cognitive development|intellectual development]]
* [[Autism spectrum disorder]], susceptibility to, X-linked 2
* [[Blepharophimosis intellectual disability syndromes|Blepharophimosis-impaired intellectual development syndrome]]
* Cardiac anomalies - [[Global developmental delay|developmental delay]] - [[Dysmorphic feature|facial dysmorphism]] syndrome
* [[CHIME syndrome]]
* [[Coffin–Siris syndrome|Coffin-Siris syndrome]] 1 and 6
* [[Congenital myopathy|Congenital nonprogressive myopathy]] with [[Moebius syndrome|Moebius]] and [[Pierre Robin sequence|Robin sequences]]
* [[Cornelia de Lange syndrome]] 3
* [[Sensenbrenner syndrome|Cranioectodermal dysplasia]] 2
* Craniosynostosis (nonsyndromic) 6
* Developmental and epileptic [[encephalopathy]], 1, 65, 77, and 84
* Developmental delay with variable [[Developmental disability|intellectual impairment]] and [[Abnormality (behavior)|behavioral abnormalities]]
* [[Dihydropyrimidinase]] deficiency
* Early-onset progressive diffuse [[Cerebral atrophy|brain atrophy]]-[[microcephaly]]-[[muscle weakness]]-[[Optic neuropathy|optic atrophy]] syndrome
* [[Faciocardiorenal syndrome]]
* [[FG syndrome]]
* [[Galloway Mowat syndrome|Galloway-Mowat syndrome]] 4
* [[Conjugate gaze palsy|Gaze palsy]], familial horizontal, with progressive [[scoliosis]] 1
* [[Hyperphosphatasia with mental retardation syndrome|Hyperphosphatasia with intellectual disability syndrome]] 1 and 2
* [[Hypotonia]], infantile, with [[psychomotor retardation]] and [[Facies (medical)|characteristic facies]] 2
* Intellectual developmental disorder, autosomal dominant 64 and 66
* Intellectual disability, autosomal dominant 13, 40, 48, and 58
* [[Joubert syndrome]] 1
* [[9q34.3 deletion syndrome|Kleefstra syndrome]] 2
* [[Langer–Giedion syndrome|Langer-Giedion syndrome]]
* [[Microphthalmia]] with brain and digit anomalies
* [[Mitochondrial DNA depletion syndrome]] 13
* [[Muenke syndrome]]
* [[Noonan syndrome]] 13
* [[Osteogenesis imperfecta]], type 20
* [[3C syndrome|Ritscher-Schinzel syndrome]] 4
[[File:Shuttleworth Potts Plate X (1).jpg|thumb|Plagiocephaly with [[oxycephaly]] in Saethre-Chotzen syndrome]]
* [[Saethre–Chotzen syndrome|Saethre-Chotzen syndrome]]
* [[Hereditary spastic paraplegia|Spastic paraplegia]], intellectual disability, [[nystagmus]], and [[obesity]]
* Syndromic [[X-linked intellectual disability]] Najm type

==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.<ref name=pmid22920774/>

It is also common for an infant with positional plagiocephaly to have misaligned ears (the ear on the affected side may be pulled forward and down and be larger or protrude more than the unaffected ear).<ref>{{Cite web|date=2014-07-31|title=Plagiocephaly (Flat Head Syndrome) & Reposition Therapy|url=https://www.morningsidechiropractic.co.uk/plagiocephaly-reposition-therapy/| access-date=2021-04-28 |website= morningsidechiropractic.co.uk| publisher= Morningside Chiropractic Edinburgh - Chiropractor Edinburgh|language=en-GB}}</ref>
<gallery>
Plagiocephaly Greig.jpg|Left anterior plagiocephaly
Plagiocephaly or Asymmetrical Skull.jpg|Right anterior plagiocephaly
Plagiocéphalie.JPG|Left posterior plagiocephaly (positional case)
Плагиоцефалия.jpg|Right posterior plagiocephaly
</gallery>
==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.<ref name=pmid22920774/>

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.<ref>{{Cite web|last=Villaneda|first=Alene|date=2015-02-09|title=The Learning Risks when Babies Skip the Crawling Phase|url=https://ilslearningcorner.com/why-babies-should-never-skip-the-crawling-phase/|access-date=2020-07-04|website=Integrated Learning Strategies|language=en-US}}</ref>

In addition, specialized mattresses are available to prevent plagiocephaly. The design of these mattresses is characterized by an ergonomic design that reduces pressure on the baby's head. These mattresses must be certified to guarantee their effectiveness.

==Treatment==
The condition may improve to some extent as the baby grows, but in some cases, home treatment<ref>{{Cite journal|last1=Gee|first1=Edward|last2=Hill|first2=Christopher E.|last3=Saithna|first3=Adnan|last4=Modi|first4=Chetan S.|last5=van der Ploeg|first5=Irene D.|date=May 2013|title=Treatment of deformational plagiocephaly and torticollis using a weight distribution ring: a report of three cases|journal=Journal of Pediatric Orthopaedics B|volume=22|issue=3|pages=275–281|doi=10.1097/BPB.0b013e32835e38d6|pmid=23358241|s2cid=26416944|issn=1060-152X}}</ref> or physical therapy treatment can improve the shape of a baby's head.<ref name=pmid22920774/>

Early interventions (based on the severity) are important to reduce the severity of the degree of plagiocephaly.<ref name=pmid22920774/> Diagnosis is most commonly determined through clinical examination. To assess the severity of the condition and determine the best course of treatment, practitioners often use the Plagiocephaly Severity Scale.<ref>{{Cite journal|last1=Holowka|first1=Mark A.|last2=Reisner|first2=Andrew|last3=Giavedoni|first3=Brian|last4=Lombardo|first4=Janet R.|last5=Coulter|first5=Colleen|date=May 2017|title=Plagiocephaly Severity Scale to Aid in Clinical Treatment Recommendations|journal=Journal of Craniofacial Surgery|language=en|volume=28|issue=3|pages=717–722|doi=10.1097/SCS.0000000000003520|pmid=28468155|s2cid=25549515|issn=1049-2275}}</ref> This is a scale that can help practitioners evaluate the condition in a standardized way.{{cn|date=October 2020}}

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 and six months of age.<ref>{{Cite journal|last1=Ditthakasem|first1=Kanlaya|last2=Kolar|first2=John C.|date=March 2017|title=Deformational Plagiocephaly: A Review|journal=Pediatric Nursing|volume=43|issue=2|pages=59–64|issn=0097-9805|pmid=29394478}}</ref>

===Repositioning===
Initially, treatment usually takes the form of reducing the pressure on the affected area by repositioning the baby onto the abdomen for extended periods throughout the day.<ref name=Rob2009/>

This may include repositioning the child's head throughout the day so that the rounded side of the head is placed against the mattress, repositioning 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, repositioning 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 position|supine]] seating which is thought to exacerbate the problem.{{Citation needed|date=February 2018}}  If the child appears to have discomfort or cries when they are repositioned, a neck problem should be ruled out.<ref name="Pediatrics 112">{{cite journal |doi=10.1542/peds.112.1.199 |title=Prevention and Management of Positional Skull Deformities in Infants |year=2003 |last1=Persing |first1=J. |last2=James |first2=H. |last3=Swanson |first3=J. |last4=Kattwinkel |first4=J. |journal=Pediatrics |volume=112 |pages=199–202 |pmid=12837890 |author5=American Academy Of Pediatrics Committee On Practice Ambulatory Medicine |issue=1 Pt 1|doi-access=free }}</ref>

===Helmets===
High-quality evidence is lacking for cranial remolding orthosis (baby helmet) for the positional condition and use for this purpose is controversial.<ref>{{cite journal|last1=Goh|first1=JL|last2=Bauer|first2=DF|last3=Durham|first3=SR|last4=Stotland|first4=MA|title=Orthotic (helmet) therapy in the treatment of plagiocephaly|journal=Neurosurgical Focus|date=October 2013|volume=35|issue=4|pages=E2|pmid=24079781|doi=10.3171/2013.7.focus13260|doi-access=free}}</ref> 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.<ref name=Rob2009>{{cite journal|last1=Robinson|first1=S |last2=Proctor |first2=M |title=Diagnosis and management of deformational plagiocephaly |journal=J Neurosurg Pediatr |date=April 2009 |volume=3|issue=4|pages= 284–95 |pmid=19338406 |doi=10.3171/2009.1.PEDS08330|doi-access= }}</ref>

==Prognosis==
Preliminary research indicates that some babies with plagiocephaly may comprise a high-risk group for developmental difficulties.<ref name="Speltz">{{cite web|url=https://www.seattlechildrens.org/about/stories/unraveling-how-craniofacial-conditions-affect-development/|website=Seattle Children's Hospital|title=Unraveling How Craniofacial Conditions Affect Development|date=September 29, 2014|access-date=September 29, 2014|archive-date=August 10, 2016|archive-url=https://web.archive.org/web/20160810182459/http://www.seattlechildrens.org/about/stories/unraveling-how-craniofacial-conditions-affect-development/|url-status=dead}}</ref><ref name="Pediatrics">{{cite journal |last1=Miller |first1=RI |last2=Clarren |first2=SK  |date=February 2000 |title=Long-term developmental outcomes in patients with deformational plagiocephaly  |journal=Pediatrics |volume=105 |issue=2 |pages=E26 |pmid=10654986 |doi=10.1542/peds.105.2.e26|doi-access= }}</ref><ref>{{cite news |url=http://www.cbc.ca/news/technology/flat-headed-babies-may-face-learning-problems-1.944026|work=CBC News |title=Flat-headed babies may face learning problems |date=September 29, 2014}}</ref> Plagiocephaly is associated with motor and language developmental delays.<ref>{{cite journal |doi=10.1097/DBP.0000000000000376 |pmid=28009719 |title=Plagiocephaly and Developmental Delay |journal=Journal of Developmental & Behavioral Pediatrics |volume=38 |issue=1 |pages=67–78 |year=2017 |last1=Martiniuk |first1=Alexandra L. C |last2=Vujovich-Dunn |first2=Cassandra |last3=Park |first3=Miles |last4=Yu |first4=William |last5=Lucas |first5=Barbara R |s2cid=39477708 |hdl=1959.4/unsworks_43153 |hdl-access=free }}</ref> While developmental delay is more commonplace among babies with plagiocephaly, it cannot be inferred that plagiocephaly is the cause of the delay.<ref>{{Cite web|url=http://pulse.seattlechildrens.org/developmental-delays-found-in-children-with-deformational-plagiocephaly/|title=Developmental Delays Found in Children with Deformational Plagiocephaly|date=2012-12-24|website=On the Pulse|access-date=2016-05-30|archive-date=2016-06-10|archive-url=https://web.archive.org/web/20160610162207/http://pulse.seattlechildrens.org/developmental-delays-found-in-children-with-deformational-plagiocephaly/|url-status=dead}}</ref>

==Etymology==
[[Ancient Greek]] πλάγιος (''plagios'') 'oblique, slanting', from [[PIE]] ''plag-'' 'flat, spread', from *''plak-'',<ref>{{Cite web | url=http://www.etymonline.com/index.php?allowed_in_frame=0&search=Plagio+&searchmode=none | title=Plagio &#124; Search Online Etymology Dictionary}}</ref> and [[Neo-Latin|Modern Latin]] ''cephal-'' 'head, skull, brain' (from Greek κεφαλή),<ref>{{Cite web | url=http://www.etymonline.com/index.php?term=cephalo-&allowed_in_frame=0 |title = Cephalo- &#124; Origin and meaning of prefix cephalo- by Online Etymology Dictionary}}</ref> together means 'flat head'.

==See also==
* [[Artificial cranial deformation]]
* [[Yakovlevian torque]]

==References==
{{Reflist}}

== External links ==
{{Medical resources
|  ICDO           =
|  DiseasesDB     = 29858
|  ICD11          = {{ICD11|LB70.00}}
|  ICD10          = {{ICD10|Q|67|3|q|65}}
|  ICD9           = {{ICD9|754.0}}
|  OMIM           =
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{{Congenital malformations and deformations of musculoskeletal system}}

[[Category:Congenital disorders of musculoskeletal system]]