Effectiveness of Helmet Therapy in Infants with Positional Skull Deformity: A Literature Review

Background: Positional skull deformity usually manifests during first six months of life due to various factors like premature births or multiple births, improper positioning of infant’s head as the head of an infant is softer than the older children’s head, thus leading to either positional brachycephaly or positional plagiocephaly. Early helmet therapy intervention may improve the shape of the skull by reducing the risk of secondary cosmetic and nervous system complications. Aim: To study the effectiveness of helmet therapy in infants with positional skull deformity. Methods: The data source for this literature review is done by studying and reviewing articles through various data like Pub Med, Google Scholar, science direct, Elsevier and medicine Cochrane library. Conclusion: Helmet therapy is contemplated to be effective in the treatment of mild-moderate-severe positional skull deformity than repositioning therapy by improving the structure of the misshaped skull, as well as the use of helmet therapy is reviewed not to hinder the head circumference growth in infants.

Intracranial Volume Not Correlated With Severity in Trigonocephaly

Objectives: Severity of trigonocephaly varies and potentially affects intracranial volume (ICV) and intracranial pressure (ICP). The aim of this study is to measure ICV in trigonocephaly patients and compare it to normative data and correlate ICV with the severity of the skull deformity according to UCSQ (Utrecht Cranial Shape Quantifier). Design: Retrospective study. Setting: Primary craniofacial center. Patients, Participants: Nineteen preoperative patients with nonsyndromic trigonocephaly (age ≤12 months). Intervention: Intracranial volume was measured on preoperative computed tomography (CT) scans by manual segmentation (OsiriX Fondation). Utrecht Cranial Shape Quantifier was used to quantify the severity of the skull deformity. When present, papilledema as sign of elevated ICP was noted. Main Outcome Measures(s): Measured ICV was compared to Lichtenberg normative cranial volume growth curves, and Pearson correlation coefficient was used to correlate UCSQ with the ICV. Results: Mean age at CT scan was 6 months (2-11). Mean measured ICV was 842 mL (579-1124). Thirteen of h19 patients (11/15 boys and 2/4 girls) had an ICV between ±2 SD curves of Lichtenberg, 2 of 19 (1/15 boys and 1/4 girls) had an ICV less than −2 SD and 4 of 19 (3/15 boys and 1/4 girls) had an ICV greater than +2 SD. Mean UCSQ severity of trigonocephaly was 2.40 (−622.65 to 1279.75). Correlation between severity and ICV was negligible (r = −0.11). No papilledema was reported. Conclusions: Measured ICV was within normal ranges for trigonocephaly patients, in both mild and severe cases. No correlation was found between severity of trigonocephaly and ICV.

Ophthalmological findings in children with non-syndromic craniosynostosis: preoperatively and postoperatively up to 12 months after surgery

AimsCraniosynostosis is a congenital condition characterised by premature fusion of one or more cranial sutures. The aim of this study was to analyse ophthalmic function before and after cranial surgery, in children with various types of non-syndromic craniosynostosis.MethodsChildren referred to Uppsala University Hospital for surgery of non-syndromic craniosynostosis were examined preoperatively. Visual acuity was measured with Preferential Looking tests or observation of fixation and following. Strabismus and eye motility were noted. Refraction was measured in cycloplegia and funduscopy was performed. Follow-up examinations were performed 6–12 months postoperatively at the children’s local hospitals.ResultsOne hundred twenty-two children with mean age 6.2 months were examined preoperatively. Refractive values were similar between the different subtypes of craniosynostosis, except for astigmatism anisometropia which was more common in unicoronal craniosynostosis. Strabismus was found in seven children, of which four had unicoronal craniosynostosis.Postoperatively, 113 children were examined, at mean age 15.9 months. The refractive values decreased, except for astigmatism and anisometropia in unicoronal craniosynostosis. Strabismus remained in unicoronal craniosynostosis. Two new cases with strabismus developed in unicoronal craniosynostosis and one in metopic, all operated with fronto-orbital techniques. No child had disc oedema or pale discs preoperatively or postoperatively.ConclusionOphthalmic dysfunctions were not frequent in children with sagittal craniosynostosis and preoperative ophthalmological evaluation may not be imperative. Children with unicoronal craniosynostosis had the highest prevalence of strabismus and anisometropia. Fronto-orbital techniques used to address skull deformity may be related to a higher prevalence of strabismus postoperatively.

Partial suturectomy for phenotypical craniosynostosis caused by incomplete fusion of cranial sutures: a novel surgical solution

OBJECTIVE Patients presenting with head shape changes phenotypical for craniosynostosis may have incomplete fusion of the involved sutures. The surgical literature is lacking in appropriate management strategies for these patients. In this paper, the authors evaluate their experience with a novel treatment strategy: suturectomy of only the fused portion followed by helmeting therapy in patients with skull deformity secondary to incomplete suture synostosis. METHODS Patients with craniosynostosis with incomplete suture fusion requiring operative intervention between 2018 and 2020 were included for evaluation. Patients were selected for partial suturectomy if the patent portion of the suture had a normal appearance. All patients underwent craniectomy of the involved portion of the synostosed suture. Intraoperative ultrasound was used to reassess the degree of fusion at the time of surgery and incision planning. A 2- to 3-cm strip craniectomy was performed under direct visualization through a single minimal access incision. Postoperative helmeting was utilized for all patients. Demographic and perioperative data were collected, including laser scan data in the form of cranial index (CI) and cranial vault asymmetry (CVA), defined as the difference between two diagonal measurements, from the frontozygomaticus to the opposite eurion. RESULTS Four males and 1 female with a mean age of 2.8 months (range 1.1–3.9 months) at presentation were included. All patients had incomplete sagittal synostosis (one patient also had an incomplete left lambdoid synostosis and another had an incomplete left coronal synostosis). The mean age at surgery was 3.5 months (range 2.0–4.7 months) without any major complications. All patients were compliant with postoperative helmeting. The average age at the last follow-up was 12.8 months (range 5.3–23.7 months) with a mean follow-up duration of 9.3 months (range 0.5–19.6 months). Final laser scan evaluations were available for 3 patients and showed an improvement of the CI from an average of 71.3 (range 70–73) to 84.3 (range 82–86). The CVA improved from an average of 9.67 mm (range 2–22 mm) to 1.67 mm (range 1–2 mm). CONCLUSIONS Minimally invasive direct excision of the involved portion of fused cranial sutures followed by helmet therapy for phenotypical craniosynostosis is a safe and effective treatment strategy. This technique is suitable for very young patients and appears to offer similar outcomes to complete suturectomy. Further studies are required to see if this approach reduces the deformity severity for patients requiring vault remodeling later in life.

Plasmacytoma Masquerading as Meningioma

Solitary bone plasmacytoma is a rare disease in the skull. We present a 70-year-old patient who presented with a skull deformity due to the left parietooccipital tumor. Neurological examination found no deficit. The systemic scanning revealed no additional lesion. The surgery was planned, and the tumor was removed totally with the marginal bone around it. Histopathological examination confirmed solitary bone plasmacytoma. The patient is being followed-up without recurrence or progression to multiple myeloma. As the calvarial solitary bone plasmacytomas can be easily misdiagnosed preoperatively, the detailed examination of the CT features may be helpful for the planning and course of the surgery.

Craniofacial surgery

Craniofacial surgery is the subspecialist area of surgery that diagnoses and manages a large heterogeneous group of both congenital and acquired conditions. The common factor is the involvement of the cranium (and its contents) and the face. This chapter first introduces craniofacial surgery and goes on to cover craniosynostosis, single-suture synostosis, syndromic synostosis, torticollis and positional skull deformity, craniofacial procedures, craniofacial clefting disorders, tumours, vascular malformations of the head and neck, hemifacial microsomia, and Treacher Collins syndrome. Finally it outlines the members of the craniofacial team, and the organization of craniofacial services in the UK.

Lessons From Zika and Other Virus Induced Skull Deformity

Objective: Viral infections during pregnancy can cause disturbance in normal craniofacial morphogenesis. While some pathogens such as cytomegalovirus and herpes simplex are familiar to us, others remain obscure. This review examines the arbovirus-induced cranial deformities and combines biomechanics with growth dynamics to gain a deeper appreciation of this complex morphogenetic process. Materials and Methods: Using Wolfram Alpha, we analyzed the impact of cell population changes. The growth dynamics of the brain, and thus the size of the calvarium, followed 2 potential logistic curves: compensated and uncompensated. To understand the potential mechanism of cell loss, we performed literature review on maternal immune activation and viral tropism for neurons and glial cells. Results: With arboviral infections such as Zika, uncompensated loss of cells during the critical phases of fetal brain development reduces the intracranial mass and therefore decreases the tensile stress across the cranial sutures. The deflationary effect produces microcephaly by subduction and reduced osteogenesis seen clinically in these infants. Conclusion: Many viral infections cause intense maternal immune activation, some have neurotropism and can result in cell loss within the developing cranium. Unable to overcome this loss, the cranium assumes a new, abnormal shape and volume. Secondary calvarial deformities is due to, and should not cause, changes in brain development.