Aim: To summarize the experience of the author with the treatment of hypertelorism. Settings and Design: The author has been heading a high-caseload department of craniofacial surgery for 38 years; the research is based on his experience with this pathology by this time. Materials and Methods: The charts of 38 patients were used for this research. Statistical Analysis Used: No statistic was used; the author has just given his personal insights as the result of a professional life devoted to the problem. Results: Most of the hypertelorism cases requiring surgical correction are rare interorbital clefts (Tessier’s 14–12). Among the syndromic ones, cranio-fronto orbital dysplasia is the most demanding for surgery because it is associated with craniosynostosis, which has to be addressed at the same time. Among the technics published for hypertelorism correction, craniofacial bipartition has our preference for several reasons: easily done and redone when necessary, safer to the vascularization, and trophicity of displaced parts of the skeleton. Conclusions: Complex craniofacial conditions such as hypertelorism have to be treated only in specialized craniofacial centers by a multidisciplinary team. The caseload has to be high, and the follow-up is very strict to get the benefits of experience to improve the results.

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Craniosynostosis is premature fusion of sutures of the cranium, resulting in an abnormal skull shape and restriction of brain growth. It may affect either a single suture or multiple sutures. In most cases, craniosynostosis is secondary to an underlying abnormality of the growing brain; however, syndromic craniosynostosis is not uncommon. It might lead to several complications such as raised intracranial pressure, neurological deficits, and neurodevelopmental disabilities. Pediatric neurologists do play a significant role in early identification and treatment, and thereby ensure a better clinical and neurodevelopmental outcome in such children.

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Nonsyndromic craniosynostosis (NSC) is more common than syndromic craniosynostosis and predominantly involves single suture. It affects sagittal, coronal, metopic, and lambdoid sutures in the decreasing order of frequency. A surgery for NSC is generally recommended to avoid potential neurodevelopmental delays and sequelae of raised intracranial pressure. Open calvarial vault reconstruction, strip craniectomy with/without the use of a postoperative molding helmet, strip craniectomy with spring implantations, endoscopic suture release, and cranial distraction osteogenesis are various surgical options used for NSC cases. The ideal age for intervention is 6–12 months for open procedures and 3–4 months for endoscopic approaches. The management is directed toward minimizing operative trauma and improving the neurocognitive outcome. The role of nonsurgical intervention by the use of genetic manipulation is still not a reality because of the nature of disease and time of presentation.

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Over the past 30 years, advances in endoscopic technology and advancing interest in the benefits of minimally invasive approaches for craniofacial surgery have resulted in these techniques becoming a part of the standard of care in the treatment of craniosynostosis. In this review, we discuss the evolution and adoption of endoscopic-assisted strip craniectomy procedures. In addition to reviewing the studies describing various nuances and modifications to minimally invasive strip craniectomy, attention to comparisons in outcomes between traditional or open cranial vault reconstructions and endoscopic-assisted techniques is highlighted for different craniosynostosis diagnoses.

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Three-dimensional (3D) photography is becoming more common in craniosynostosis practice and may be used for research, archiving, and as a planning tool. In this article, an overview of the uses of 3D photography will be given, including systems available and illustrations of how they can be used. Important innovations in 3D computer vision will also be discussed, including the potential role of statistical shape modeling and analysis as an outcomes tool with presentation of some results and a review of the literature on the topic. Potential future applications in diagnostics using machine learning will also be presented.

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Context: The well-known effects of ionizing radiation on brain cells have been a major driving force toward the use of non-ionizing methods of imaging in both elective and emergency settings. Pediatric neurosurgery has certainly leveraged on this shift in clinical practice, however patients with craniofacial disorders could not fully benefit from the adoption of magnetic resonance imaging (MRI) because computed tomography (CT) scans still retain superior imaging power on bone tissue. Aims: To explore the knowledge available on the use of MRI as surrogate for CT scan in the assessment of craniosynostosis. Settings and Design: A scoping review was designed to identify landmark studies and ongoing clinical trials exploring the accuracy of MRI-based bone imaging in the preoperative planning of pediatric patients with craniosynostosis. Materials and Methods: A total of 492 records were screened from Pubmed, Ovid Medline, Scopus, and Cochrane Library databases; while 55 records were retrieved from ClinicalTrials.gov register. Only clinical studies revolving around the use of Gradient Echo Black-Bone (BB) and Zero Time Echo (ZTE) MRI sequences for the preoperative planning of pediatric craniosynostosis were retained for inclusion. Results and Conclusions: This review identified only five clinical studies reporting a high accuracy of MRI-based 3D bone reconstruction in 47 pediatric candidates to surgical correction of craniosynostosis. Although promising, limited evidence (Level IV) exist that BB and ZTE MRI could help in the surgical planning for craniosynostosis management. The results of two ongoing randomized clinical trials, which are actively enrolling patients, will hopefully help answering this research question.

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Chiari 1 malformation and hydrocephalus are frequent findings in multi-suture and syndromic craniosynostosis patients. In this article, we review the pathogenesis, clinical significance, and management options for these conditions with comments from our own experience. The role of premature fusion of skull base sutures leading to a crowded posterior fossa and venous outflow obstruction resulting in impaired cerebrospinal fluid (CSF) absorption is highlighted. Management options are unique in this group and we advocate early (prior to 6 months of age) posterior vault expansion by distraction osteogenesis (DO) in the management of Chiari 1 malformation. Foramen magnum decompression is recommended for a select few either as part of posterior vault expansion or at a later date. Treatment of hydrocephalus, utilizing a ventriculoperitoneal (VP) shunt with preferably a programmable high-pressure valve and anti-siphon device, is required in a small percentage of cases despite successful posterior vault expansion. Patients need to be carefully selected and managed as hydrocephalus often serves as an important cranial vault growth stimulus. Further, they require careful monitoring and thought to ensure the management of these conditions and the timing of any intervention provides the optimal long-term outcome for the patient.

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Most complex craniosynostoses are managed the same way as syndromic craniosynostoses (SCs), as these patients often experience similar problems regarding cognition and increased intracranial pressure (ICP). The evaluation and treatment plan for craniosynostoses is complex, and this, additionally, is complicated by the age at presentation. In this article, the authors review the complexity of SCs in the presentation and management. An algorithm is necessary for such multifaceted and multidimensional pathology as craniosynostoses. In most algorithms, posterior calvarial distraction is a consistent early option for complex craniosynostoses presenting early with raised ICP. Addressing the airway early is critical when significant airway issues are there. All other surgical interventions are tailored on the basis of presentation and age.

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Patients presenting with craniofacial conditions present a unique challenge from an ophthalmological view point. There are no set guidelines as to their management or their long-term monitoring and follow-up. Largely, this should be the remit of a dedicated craniofacial team. Here we present pertinent ophthalmological pathology occurring in combination with craniosynostosis alongside the protocol employed in Birmingham Children’s Hospital for the management of these patients.

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