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].

• 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]

• Anemia

  New chapters, expanded and updated coverage, increased worldwide perspectives, and many new contributors keep you current on the late preterm infant, the fetal origins of adult disease, neonatal anemia, genetic disorders, and more. "...a valuable reference [books.google.com]

  It has also been reported that cephalic index is less than 2-3 in individual with sickle cell anemia than normal individual. Material & Methods: The present study was carried out with 320 (160 male & 160 female) medical students of Dr. [scinapse.io]

  】*malignant ameloblastoma 【悪性高熱症】*malignant hyperthermia 【悪性黒色腫】*malignant melanoma (略 MM) 【悪性腫瘍】*malignant tumor *malignancy 【悪性新生物】*malignant neoplasm ( 悪性腫瘍) 【悪性組織球症】*malignant histiocytosis (略 MH) 【悪性中皮腫】*malignant mesothelioma 【悪性貧血】*pernicious anemia [medo.jp]

  Complex Multisuture Synostosis Secondary Synostosis Hematologic disorders Congenital hemolytic icterus Polycythemia vera Sickle cell anemia Thalassemia Iatrogenic disorders Hydrocephalus with shunt Malformations Encephalocele Holoprosencephaly Microcephaly [cincinnatichildrens.org]

• 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]

• Torticollis

  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]

  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]

  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]

• Decrease in Height

  […] cranial height on the right side Level ears The right ear is positioned inferiorly relative to the left Frontal view Forehead and cheek more prominent on the right than the left Frontal view Forehead prominent on the left and severely flattened on the [musculoskeletalkey.com]

• Skin Rash

  rash *rash ( 皮疹) 【発疹チフス】*exanthematous typhus *epidemic typhus (哺) 【哺乳障害】*dystithia ( 授乳困難症) (本) 【本態性血小板血症】*essential thrombocythemia (略 ET) 【本態性高血圧】*essential hypertension (バ) 【バセドウ病】*Basedow's disease 【バチ状指】【バチ指】*clubbed finger ( 太鼓バチ状指、太鼓バチ指) 【バネ指 [medo.jp]

• Strabismus

  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]

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

• 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]

• 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]

• 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.