Isolated synostotic plagiocephaly (ISP) presents as an oblique head as a consequence of an anomalous unilateral fusion of the coronal or the lambdoid suture [1]. In both cases, a palpable ridge will develop over the site of premature suture fusion [2]. Unilateral coronal synostosis is associated with anterior ISP while posterior ISP is caused by an early fusion of the lambdoid suture [3]. ISP affects approximately 1/100000 infants [4].

The genetic role in ISP is still in the focus of medical research. Rare mutations in the FGFRs, TWIST1, LRIT3, ALX4, IGFR1, EFNA4, RUNX2, and FREM1 genes may play a role for isolated ISP [3] [5] [6].

Anterior ISP-associated deformities include a flattening of the frontal bone and of the ipsilateral orbital rim in combination with a contralateral frontal bossing. In 50-60% of cases, strabismus is found as a consequence of anatomical changes in the orbital roof. Anterior ISP can also lead to a deviation of the skull base, a positional change of orbits, eyebrow and ear asymmetries, as well as mandibular deviations with malocclusion [3] [7] [8].

Posterior ISP leads to a trapezoid head shape with occiput flattening and malpositioned ears. Moreover, early lambdoid suture synostosis is linked to debilitating changes in the posterior fossa. Most notable findings in this context include an Arnold-Chiari malformation type 1 and a fusion of the jugular foramen. Thus, posterior ISP predisposes the patient for intracranial hypertension [9] [10].

• Physician

  This book will be an ideal aid for pediatricians, neurosurgeons, maxillo-facial surgeons, orthopedic physicians, physiotherapists and for all involved in preventing and managing the condition. [books.google.com]

  West Texas Craniofacial Center of Excellence Lubbock About Us Texas Tech Physicians and Covenant Women’s and Children’s Hospital have joined forces to create the West Texas Craniofacial Center of Excellence, serving infants, children and adults with abnormal [texastechphysicians.com]

  The craniofacial team may also contact referring physicians if more information is required. The new referral pathway will enhance communication between the NPCC and referring speciality and the family of children referred. [imj.ie]

  A thorough physical examination by the primary care physician is necessary. [aafp.org]

  Am Fam Physician. 2004;69:2863-2870. INTERNET Sheth RD Aldana PR, Iskandar BJ. Craniosynostosis. Medscape. Last Update October 27, 2016. Available at: Accessed February 21, 2017. National Institute of Neurological Disorders and Stoke. [rarediseases.org]

• Developmental Delay

  craniosynostosis Extraskeletal myxoid chondrosarcoma Giant cell glioblastoma Gliosarcoma Hypochondroplasia Isolated cloverleaf skull syndrome Lacrimo-auriculo-dento-digital syndrome Muenke syndrome Saethre-Chotzen syndrome Severe achondroplasia - developmental [csbg.cnb.csic.es]

  If this condition is not treated, it causes pressure inside the head along with head deformity, developmental delay and, in some cases, seizures. Craniosynostosis can be classified as non-syndromic and syndromic. [rileychildrens.org]

  Developmental delay is common, and intellectual disabilities are seen in 50-85 percent of cases. [cincinnatichildrens.org]

  delay and low IQ -Craniosynostosis: bicoronal synostosis significant turribrachycephaly, enlarged anterior fontanel, bitemporal widening, occipital flattening Features of Pfeiffer syndrome 1 in 100,000 AD inheritance Mutations FGFR1 - 3 Features -Turribrachycephaly [quizlet.com]

  If left untreated, this can cause increased pressure within the skull (intracranial pressure) and can potentially result in cognitive impairment or developmental delays. [rarediseases.org]

• Surgical Procedure

  procedures safely and efficiently. [books.google.com]

  Even though there are many surgical options available, depending on the type of synostosis, the most common surgical procedure is generally performed by a plastic surgeon in conjunction with a neurosurgeon. [craniokids.co.za]

  Anyone who has had multiple surgical procedures might have such an allergy. [cincinnatichildrens.org]

  Specific sutures [ edit ] Sagittal craniosynostosis/scaphocephaly [ edit ] There are two surgical procedures which are commonly used to treat sagittal synostosis. [40] The matter of which procedure is superior is still heavily debated amongst the surgeons [en.wikipedia.org]

• Pain

  Pain is generally not a problem, unless radial head dislocation should occur. [1] [2] Most examples of radioulnar synostosis are isolated (non-syndromic). [ipfs.io]

  Pain If your child is under four years of age, administer regular children's Tylenol as directed for his or her pain and irritability. Children older than four years may be sent home with a prescription for Tylenol with codeine. [cincinnatichildrens.org]

  *pulsatile pain *pulsatory pain *throbbing pain (剥) 【剥離骨折】*avulsion fracture ( 裂離骨折) 【剥離性間質性肺炎】*desquamative interstitial pneumonia 【剥離性歯肉炎】*[[歯]]desquamative gingivitis 【剥離性皮膚炎】*exfoliative dermatitis (発) 【発育性骨空洞】*developmental bone cavity 【発育不全症】*hypogenesis [medo.jp]

  In three of these cases (23%), mothers had premature rupture of membranes with subsequent onset of labor pains; they were admitted to the hospital and their babies delivered at a mean estimated gestational age by last menstrual period of 35.8 weeks. [skullbaseinstitute.com]

  When craniosynostosis corrections are performed on older children they tell us that they never really feel any pain. They do say that their heads feels “tight,” especially during the peak of swelling, 24-48 hours after surgery. [thecraniofacialcenter.com]

• Falling

  These children continue to "fall through the cracks" of our medical education system. [books.google.com]

  The guideline also noted that termination of the orthotic treatment program is recommended, without weaning, when head shape falls within normal limits. [aetna.com]

• Hypertension

  Thus, posterior ISP predisposes the patient for intracranial hypertension. ISP diagnosis relies on clinical findings and a thorough analysis excluding other potential causes of plagiocephaly. [symptoma.com]

  agenesis of the corpus callosum, Chiari malformation type 1, or conditions of intracranial hypertension) [ 18 ]. [musculoskeletalkey.com]

  It never improves spontaneously and confers the risk of intracranial hypertension, although this happens in 10% of cases when only a single suture is involved. 25 In contrast, PPP is often subtle and goes unnoticed or is absent at birth. [neupsykey.com]

  【高血圧】*hypertension 【高血圧眼底】*fundus hypertonicus[L] 【高血圧性心疾患】*hypertensive heart disease (略 HHD) 【高血圧性脳症】*hypertensive encephalopathy 【高血圧性網膜症】*hypertensive retinopathy 【高血圧発作】*hypertensive episode 【高血糖症】*hyperglycemia 過血糖症 【高コレステロール血症】*cholesterolemia [medo.jp]

• Malocclusion

  Anterior ISP can also lead to a deviation of the skull base, a positional change of orbits, eyebrow and ear asymmetries, as well as mandibular deviations with malocclusion. [symptoma.com]

  Marked malocclusion of the alveolar process may occur with severe jaw deformities. Treatment is usually not needed except in most severe cases. Figure 171.1. — Facial molding. The right jaw appears sharper while the left is fuller. [pediatricneuro.com]

  […] widening, occipital flattening Features of Pfeiffer syndrome 1 in 100,000 AD inheritance Mutations FGFR1 - 3 Features -Turribrachycephaly/ Kleeblattschadel -BROAD thumbs or great toes -Midface hypoplasia - BEAK nose -Mild cutaneous syndactyly -Class III malocclusion [quizlet.com]

  B 24, 25 Complications The major complications associated with uncorrected craniosynostosis include increased intracranial pressure, asymmetry of the face, and malocclusion. [aafp.org]

  Infants with Crouzon or Apert syndromes face many similar potential clinical problems such as proptosis, shallow orbits, hypertelorism, strabismus, malocclusion, and hearing loss; many of these features are directly associated with bilateral coronal synostosis [eyewiki.aao.org]

• Exanthema

  【急性大動脈解離】*acute aortic dissection (略 AD) 【急性胆嚢炎】*acute cholecystitis 【急性虫垂炎】*acute appendicitis 【急性中毒性表皮剥離(剥脱)】*epidermolysis acuta toxica[L] (略 EAT) 【急性洞炎】*acute sinusitis 【急性脳症】*acute encephalopathy 【急性副鼻腔炎】*acute paranasal sinusitis 【急性発疹】*acute exanthema [medo.jp]

• Torticollis

  Positional plagiocephaly (PP) often occurs concomitantly with torticollis and produces stronger facial asymmetries; ISP is most easily identified by the posterior malpositioning of the ear on the affected side. [symptoma.com]

  Conversely, early developmental disturbances of vision often disrupt ocular motor control systems, giving rise to complex disorders such as nystagmus, strabismus, and torticollis. [books.google.com]

  Other factors which may increase the risk of deformational plagiocephaly include the following: Muscular torticollis. One cause of deformational plagiocephaly may be muscular torticollis. [stanfordchildrens.org]

  Infants with torticollis require physical therapy to stretch and straighten the neck. In such cases, head flattening typically improves through successful treatment of the torticollis. [rileychildrens.org]

  The physical examination should be completed with an assessment of neck movements to confirm or rule out the presence of torticollis. Normally, there is no need for complementary radiologic investigations. [neupsykey.com]

• Cranial Asymmetry

  The use of computed tomography (CT) imaging is only indicated in case of an ISP suspicion, particularly if infant repositioning does not improve cranial asymmetry over weeks. [symptoma.com]

  Neurosurgery 1980;6:39-44 Lohse DC, et al: Cranial asymmetry and lambdoid synostosis in a sibship. Neurosurgery 1985;16:836-838 Moss ML: Functional anatomy of cranial synostosis. [skullbaseinstitute.com]

• Strabismus

  In 50-60% of cases, strabismus is found as a consequence of anatomical changes in the orbital roof. [symptoma.com]

  Conversely, early developmental disturbances of vision often disrupt ocular motor control systems, giving rise to complex disorders such as nystagmus, strabismus, and torticollis. [books.google.com]

  […] asymmetry / facial hemiatrophy / facial hemihypertrophy - Frontal bossing / prominent forehead - Plagiocephaly Frequent - Abnormal eye movements / oculomotor disorder - Abnormal visual field / hemianopsia / hemianopia / scotoma / visual peripheral rim - Strabismus [csbg.cnb.csic.es]

  Further, the observed facial features were not suggestive of Crouzon dis- ease; in particular, none of the affected fam- ily members showed the characteristic ex- ophthalmus and external strabismus, nor was hypoplastic maxilla and relative man- dibular [docslide.us]

  Infants with Crouzon or Apert syndromes face many similar potential clinical problems such as proptosis, shallow orbits, hypertelorism, strabismus, malocclusion, and hearing loss; many of these features are directly associated with bilateral coronal synostosis [eyewiki.aao.org]

• Visual Impairment

  impairment, sleeping impairment, eating difficulties, or an impairment of mental development combined with a significant reduction in IQ. [4] Craniosynostosis occurs in one in 2000 births. [en.wikipedia.org]

• Hydrops Fetalis

  Stay at the forefront of your field thanks to new and completely revised chapters covering topics such as: Principles and Practice l Immune and Non-immune Hydrops Fetalis l Amniotic Fluid Volume l Enhancing Safe Prescribing in the Neonatal Intensive Care [books.google.com]

• Distractibility

  […] include Facial nerve rehabilitation; Septorhinoplasty; Vascular lesions; Craniofacial anomalies; Free tissue transfer; Craniomaxillofacial trauma; Cleft lip and palate; Surgical speech disorders; Otoplasty; Microtia; Soft tissu trauma and scar revision; Distraction [books.google.com]

  These researchers summarized operative characteristics and outcomes of distraction osteogenesis and presented data comparing distraction osteogenesis to cranial remodeling surgery. [aetna.com]

  […] isolated single suture synostosis including sagittal, metopic, coronal and lambdoid deformities to more complex syndromic cases with bicoronal synostosis, including Aperts, Crouzons, Muenkes, Pfeiffers and Carpenter syndromes Conventional orthognathic and distraction [texastechphysicians.com]

  Rarely, surgeons use cranial vault distraction. [childrens.com]

  Tellado MG, Lema A: Coronal suturectomy through minimal incisions and distraction osteogenesis are enough without other craniotomies for the treatment of plagiocephaly due to coronal synostosis. J Craniofac Surg 2009;20:1975-1977. [karger.com]

• Frontal Bossing

  Anterior ISP-associated deformities include a flattening of the frontal bone and of the ipsilateral orbital rim in combination with a contralateral frontal bossing. [symptoma.com]

  However, in this situation, the vertex view reveals a trapezium-shaped head: there is a unilateral occipitoparietal flattening associated with contralateral frontal bossing. [neupsykey.com]

  bossing / prominent forehead - Plagiocephaly Frequent - Abnormal eye movements / oculomotor disorder - Abnormal visual field / hemianopsia / hemianopia / scotoma / visual peripheral rim - Strabismus / squint Occasional - Autosomal dominant inheritance [csbg.cnb.csic.es]

  Ear shearing and frontal bossing were noted for side of involvement and degree of severity. A total of 6 children (46%) exhibited some degree of ear shearing on the right side. 9 children (70%) demonstrated some degree of frontal bossing. [skullbaseinstitute.com]

• Facial Hemihypertrophy

  hemihypertrophy - Frontal bossing / prominent forehead - Plagiocephaly Frequent - Abnormal eye movements / oculomotor disorder - Abnormal visual field / hemianopsia / hemianopia / scotoma / visual peripheral rim - Strabismus / squint Occasional - Autosomal [csbg.cnb.csic.es]

• Nystagmus

  Conversely, early developmental disturbances of vision often disrupt ocular motor control systems, giving rise to complex disorders such as nystagmus, strabismus, and torticollis. [books.google.com]

  Other ophthalmic manifestations may include globe subluxation, ametropia, amblyopia and nystagmus [10]. Congenital glaucoma secondary to closed angles and FGFR2-related anterior segment dysgenesis has been reported [11]. [eyewiki.aao.org]

  *nystagmus of retraction (太) 【太鼓バチ状指】【太鼓バチ指】*clubbed finger バチ状指、バチ指 (代) 【代謝疾患】*metabolic disease (体) 【体静脈狭窄】*systemic vein stenosis (多) 【多飲多食症】*polydipsia 【多汗症】*hyperhidrosis *polyhidrosis *hidrosis *sudoresis 発汗過多症 【多顎症】*polygnathia 【多形滲出性紅斑】*erythema [medo.jp]

• Speech Disorder

  Topics include Facial nerve rehabilitation; Septorhinoplasty; Vascular lesions; Craniofacial anomalies; Free tissue transfer; Craniomaxillofacial trauma; Cleft lip and palate; Surgical speech disorders; Otoplasty; Microtia; Soft tissu trauma and scar [books.google.com]

ISP diagnosis relies on clinical findings and a thorough analysis excluding other potential causes of plagiocephaly. Plagiocephaly is clinically diagnosed by the typical formation of a parallelogram defined by ear and maxilla positions [1].

Positional plagiocephaly (PP) often occurs concomitantly with torticollis and produces stronger facial asymmetries [1] [11] [12]; ISP is most easily identified by the posterior malpositioning of the ear on the affected side. On the other hand, PP produces anteriorly malpositioned ears [2]. Syndromic plagiocephaly is usually accompanied by developmental retardation [13].

The use of computed tomography (CT) imaging is only indicated in case of an ISP suspicion, particularly if infant repositioning does not improve cranial asymmetry over weeks. CT findings can also be used in preparation for a surgical intervention aimed to avert an increased risk of intracranial hypertension (ICH) or to correct the plagiocephaly-caused malocclusion. If ICH has already developed, appropriate immediate countermeasures are indicated [1] [14].

1. Laughlin J, Luerssen TG, Dias MS. Committee On Practice Ambulatory Medicine. Prevention and Management of Positional Skull Deformities in Infants. Pediatrics. 2011;128(6): 1236–1241.
2. Biggs WS. Diagnosis and Management of Positional Head Deformity. Am Fam Physician. 2003; 67(9):1953-1956.
3. Ghizoni E, Denadai R, Raposo-Amaral CA, Joaquim AF, Tedeschi H, Raposo-Amaral CE. Diagnosis of infant synostotic and nonsynostotic cranial deformities: a review for pediatricians. Rev Paul Pediatr. 2016; 34(4): 495–502.
4. Bruneteau RJ, Mulliken JB. Frontal plagiocephaly: synostotic, compensational, or deformational. Plast Reconstr Surg. 1992;89(1):21–31.
5. Heuzé Y, Holmes G, Peter I, Richtsmeier JT, Jabs EW. Closing the gap: genetic and genomic continuum from syndromic to nonsyndromic craniosynostoses. Curr Genet Med Rep. 2014;2(3):135–145.
6. Johnson D, Wilkie AO. Craniosynostosis. Eur J Hum Genet. 2011;19(4):369–376.
7. Raposo-do-Amaral CE, Silva MP, Menon DN, Somensi RS, Raposo-do-Amaral CA, Buzzo CL. Estudo antropométrico das assimetrias craniofaciais na craniossinostose coronal unilateral. Rev Bras Cir Plast. 2011;26(1):27–31.
8. Raposo-do-Amaral CE, Raposo-do-Amaral CA, Guidi MC, Buzzo CL. Prevalência do estrabismo na craniossinostose coronal unilateral. Há benefício com a cirurgia craniofacial? Rev Bras Cir Craniomaxilofac. 2010;13(2):73–77.
9. Matushita H, Alonso N, Cardeal DD, Andrade FG. Major clinical features of synostotic occipital plagiocephaly: mechanisms of cranial deformations. Childs Nerv Syst. 2014;30(7):1217–1224.
10. Tamburrini G, Caldarelli M, Massimi L, Gasparini G, Pelo S, Di Rocco C. Complex craniosynostoses: a review of the prominent clinical features and the related management strategies. Childs Nerv Syst. 2012;28(9):1511–1523.
11. Davis B, Moon R, Schas H, Ottolini M. Effects of sleep position on infant motor development. Pediatrics. 1998;102(5):1135–1140.
12. de Chalain T, Park S. Torticollis associated with positional plagiocephaly: a growing epidemic. J Craniofac Surg. 2005;16(3):411–418.
13. Kimonis V, Gold JA, Hoffman TL, Panchal J, Boyadjiev SA. Genetics of craniosynostosis. Semin Pediatr Neurol. 2007;14(3): 150–161.
14. Robinson S, Proctor M. Diagnosis and management of deformational plagiocephaly. J Neurosurg Pediatr. 2009;3(4):284–295.