Isolated synostotic plagiocephaly (ISP) is a congenital disorder caused by a premature unilateral closure of either the coronal or lambdoid suture. Premature coronal and lambdoid synostosis result in anterior and posterior ISP, respectively. ISP must be rigorously differentiated from syndromic and positional plagiocephaly forms. Diagnosis rests on clinical observations and imaging techniques. Genetic testing may also be considered.
ISP-associated cranial deformities can lead to severe consequences such as increased intracranial pressure. Hence, early surgical corrections are usually indicated to prevent long-term cranial and cerebral damage.
Isolated synostotic plagiocephaly (ISP) presents as an oblique head as a consequence of an anomalous unilateral fusion of the coronal or the lambdoid suture . In both cases, a palpable ridge will develop over the site of premature suture fusion . Unilateral coronal synostosis is associated with anterior ISP while posterior ISP is caused by an early fusion of the lambdoid suture . ISP affects approximately 1/100000 infants .
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   .
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   .
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  .
Entire Body System
- 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]
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 .
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. On the other hand, PP produces anteriorly malpositioned ears . Syndromic plagiocephaly is usually accompanied by developmental retardation .
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  .
Make informed clinical choices for each patient, from diagnosis and treatment selection through post-treatment strategies and management of complications, with new evidence-based criteria throughout. [books.google.com]
Treatment for deformational plagiocephaly Specific treatment will be determined by your child's doctor based on the severity of the deformational plagiocephaly. [stanfordchildrens.org]
Treatments Treatments Treatment for head shape abnormalities varies depending on the specific anomaly and its cause: Positional plagiocephaly. [rileychildrens.org]
The closing chapter addresses prognosis and complications. [books.google.com]
Treatment and prognosis Treatment is often with a cranioplasty. Abnormal intracranial pressure may affect neurocognition. Promoted articles (advertising) [radiopaedia.org]
we will not need to conduct further diagnostic exams, and we can explain to the parents the developmental dimension of the clinical features addressing the case to a pediatric physiotherapeutic treatment. 5.1.2 Developmental Risks of PPP PPP has a prognosis [musculoskeletalkey.com]
The severity of the cranial bone deformations, associated systemic abnormalities and age of diagnosis may yield variable treatment outcomes and overall prognosis . [eyewiki.aao.org]
Prognosis and outcomes Non-syndromic craniosynostosis patients if operated early have very good post-operative outcome and the need for the second surgery is minimal. [ijps.org]
Pediatrics Keywords Cranial Sutures--pathology; Cranial Sutures--physiopathology; Craniosynostoses--etiology; Frontal Bone--pathology; Frontal Bone--physiopathology; Sphenoid Bone--pathology; Sphenoid Bone--physiopathology; Synostosis--complications; [hsrc.himmelfarb.gwu.edu]
In the last two decades increased knowledge about the structure and function of the human genome has enabled the discovery of the molecular etiologies of most forms of syndromic craniosynostosis, which in turn has allowed for the analysis of normal and [books.google.com]
This suggests a yet-to-be-identified single-gene, autosomal dominant genetic etiology. [nature.com]
The etiology has been attributed to genetic factors and primary disorders of bone growth, in addition to secondary effects of other diseases and modulators of the in utero environment. [skullbaseinstitute.com]
50% of syndromic - 2% of isolated -More than 130 syndromes involve craniosynostosis -Etiology syndromic: mutation in fibroblast growth factor receptor (FGFR) most common What are the 5 most common syndromic craniosynostosis conditions and their inheritance [quizlet.com]
Information is first presented on classification, epidemiology and etiopathogenesis. [books.google.com]
[…] plagiocephaly Classification (Orphanet): - Rare bone disease - Rare developmental defect during embryogenesis - Rare eye disease - Rare genetic disease Classification (ICD10): - Congenital malformations, deformations and chromosomal abnormalities - Epidemiological [csbg.cnb.csic.es]
Epidemiology. 1995;6:306–10. 11. Muenke M, Schell U, Hehr A, Robin NH, Losken HW, Schinzel A, et al. A common mutation in the fibroblast growth factor receptor 1 gene in Pfeiffer syndrome. Nat Genet. 1994;8:269–74. 12. [aafp.org]
Linguistic Variant - Portuguese, Brazil) 2015AB LNC-RU-RU (LOINC Linguistic Variant - Russian, Russia) 2015AB LNC-TR-TR (LOINC Linguistic Variant - Turkish, Turkey) 2015AB LNC-ZH-CN (LOINC Linguistic Variant - Chinese, China) 2015AB MCM (Glossary of Clinical Epidemiologic [doctor.am]
International Journal of Epidemiology. 18 (4): 891–9. doi : 10.1093/ije/18.4.891. PMID 2621027. ^ Jentink J, Loane MA, Dolk H, Barisic I, Garne E, Morris JK, de Jong-van den Berg LT (June 2010). [en.wikipedia.org]
Regarding the pathophysiologic mechanism of PPP, it is admitted that external force applied consistently to a specific region of the infant’s head deforms the skull. [neupsykey.com]
Donata Villani, Maria Vittoria Meraviglia Springer, ١٧/٠٧/٢٠١٤ - 106 من الصفحات This book provides clear guidance on the prevention, early diagnosis, and treatment of positional plagiocephaly. [books.google.com]
Hence, early surgical corrections are usually indicated to prevent long-term cranial and cerebral damage. [symptoma.com]
0% Emergent - ED Care Needed - Preventable/Avoidable - 0% Emergent - ED Care Needed - Not Preventable/Avoidable - 0% Primary diagnosis of injury 0% Primary diagnosis of mental health problems 0% Primary diagnosis of substance abuse 0% Primary diagnosis [medicbind.com]
NCI_CTEP-SDC (Cancer Therapy Evaluation Program - Simple Disease Classification) 2015AB NCI_CareLex (Content Archive Resource Exchange Lexicon) 2015AB NCI_DCP (NCI Division of Cancer Prevention Program) 2015AB NCI_DICOM (Digital Imaging Communications [doctor.am]
Alternating your infant's sleep position by turning the head to the opposite side, and not putting infants on their backs when they are awake, may also help prevent and treat positional plagiocephaly. [stanfordchildrens.org]
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