Management of Chiari 1 Malformation and Hydrocephalus in Syndromic Craniosynostosis
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Abstract:
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.Keywords:
Cranial vault
Foramen magnum
Posterior cranial fossa
Chiari Malformation
Craniosynostosis describes the premature pathologic partial or complete fusion of 1 or more of the cranial sutures. Over the past few decades, research on craniosynostosis has progressed from gross description of deformities to an understanding of some of the molecular etiologies behind premature suture fusion. Studies on patients with syndromic craniosynostosis have resulted in the identification of several genes, molecular events, and deformational forces involved in abnormal growth and development of the cranial vault. Conservation of craniofacial development and sequence homology between humans and other species have also led to insightful discoveries in cranial suture development. In this review, we discuss the development of the cranial vault and explain the basic science behind craniosynostosis in humans as well as in animal models and how these studies may lead to future advances in craniosynostosis treatments.
Cranial vault
Craniosynostoses
Craniofacial surgery
Dysostosis
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Remodelling the cranial vault in an attempt to increase the intracranial volume and thus control intracranial hypertension, whilst at the same time improving the patient's appearance, has been the mainstay of surgery for syndromic craniosynostosis. We report a case of craniosynostosis in whom cranial vault expansion was followed by the development of hind-brain herniation and hydrocephalus. This prompted a review of our other cases of craniosynostosis who had been evaluated by magnetic resonance imaging following surgery in order to assess the frequency of hind-brain herniation and hydrocephalus in these children. Magnetic resonance imaging had been performed in the postoperative evaluation of 34 cases of craniosynostosis who had undergone procedures intended to increase the intracranial volume. The position of the cerebellar tonsils and the presence or otherwise of hydrocephalus was recorded for all cases. The effectiveness of surgery in treating raised intracranial pressure (ICP) was evaluated by means of postoperative ICP monitoring and had been performed in 22 cases. Herniation of the hind-brain below the level of the foramen magnum was observed in 18 cases (53%). Hydrocephalus, requiring the insertion of a ventriculoperitoneal shunt, was present in 14 cases (41 %) and had developed after the cranial vault procedure in 9. The mean sleeping ICP measured postoperatively was normal (< 10 mm Hg) in 5, borderline (10–15) in 7, and raised (> 15 mm Hg) in 10 cases. Cranial vault expansion in complex craniosynostosis may fail to address the underlying aetiology of intracranial hypertension. Furthermore, both hydrocephalus and hind-brain herniation may develop following such surgery. Neither the increase in intracranial volume afforded by cranial vault expansion nor the shunting of hydrocephalus precludes the persistence of abnormal ICP. These findings are discussed in the light of possible mechanisms, in addition to cephalocranial disproportion responsible for intracranial hypertension in complex craniosynostosis. The implications for the surgical management of complex craniosynostosis are reviewed.
Cranial vault
Foramen magnum
Synostosis
Intracranial pressure monitoring
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Foramen magnum
Ventriculomegaly
Chiari Malformation
Occipital bone
Synchondrosis
Chiari I malformation
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Standard craniofacial techniques to expand the cranial vault were successful in treating elevations of intracranial pressure (ICP) in 7 older children. Of the 7 patients, a diagnosis of craniosynostosis was made in 6 and deformational head deformity with slit ventricle syndrome in 1. There were 5 boys and 2 girls, whose ages ranged from 3 1/2 to 8 years (mean, 5.2 yr). Three patients had previously undergone surgical treatment of craniosynostosis. One patient presented with visual changes and papilledema. Another with a deformational skull deformity was shunt-dependent and was diagnosed with slit ventricle syndrome. This patient had undergone several temporal craniectomies to control recurrent symptoms of increased ICP. The remaining 2 patients appeared to have craniosynostosis as an explanation for reduced cranial vault size and elevated ICP, but the specific sites of sutural fusion could not be identified. Total follow-up ranged from 3 to 18 months, and evidence of resolution of signs and symptoms of increased ICP was achieved in each of the 7 patients. Plastic surgeons involved in caring for children with craniosynostosis should be particularly aware of the possibility of increased ICP developing or recurring following craniofacial surgery.
Cranial vault
Papilledema
Craniofacial surgery
Cranioplasty
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Citations (45)
The authors describe a comprehensive diagnostic algorithm,
individual pre-operative care and postoperative follow-up
procedures, established at the author’s workplace, to address
tailored pre-operative haematological preparation and cranial
vault remodelling surgery in craniosynostosis patients.
Materials and methodology: A set of 14 patients newly operated
upon using the remodelling technique is presented and compared
to a set of patients operated upon using strip craniectomy, in
terms of cosmetic effects, the need for transfusion, surgery
time and complications. Results: Remodellation technique
surgery patients showed significant improvement in cephalic
index and a better cosmetic effect compared with the strip
craniectomy patient group. There was no significant difference
in surgery time between the operational techniques.
Pre-operative haematological preparation was sufficient to
eliminate the higher transfusion requirements of very young
patients. Conclusions: The remodelling surgery technique was
found to provide better cosmetic and therapeutic effects
compared with strip craniectomy. Cranial vault remodelling
surgery combined with comprehensive, tailored pre-operative
care is a safe and efficient procedure in craniosynostosis
treatment even in very young children.
Cranial vault
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This chapter, provides an excellent review of a core pediatric surgical topic; craniosynostosis and cranial vault remodeling. The authors describe the pathophysiology of the four main cranial sutures and the characteristics associated with each of their premature closures. The syndromes associated with the synostoses are reviewed. The perioperative considerations for cranial vault reconstruction are presented.
Cranial vault
Craniosynostoses
Vault (architecture)
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Craniosynostosis is a group of common congenital craniomaxillofacial deformities. Syndromic craniosynostosis is usually accompanied by increased intracranial pressure, craniocerebral growth restriction, craniofacial deformities and even chiari malformation. Traditional anterior or posterior skull reshaping has its drawbacks such as high surgical risk, high recurrence rate and unsatisfactory therapeutic efficacy, and so on. The operation technique of posterior cranial vault distraction is relatively simple. The treatment process is controllable, accurate and stable. It can largely expand the intracranial volume and improve the anterior and posterior skull configuration with a low risk of complications. To sum up, posterior cranial vault distraction osteogenesis can be a surgical procedure of choice when syndromic craniosynostosis is treated.
Key words:
Craniosynostosis; Syndrome; Osteogenesis, distraction; Increased intracranial pressure
Cranial vault
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Craniosynostosis is a congenital disease which consists of premature fusion of one or more cranial sutures, resulting in an abnormal head shape. Patients are usually treated by cranial vault expansion surgery to minimize the potential for brain damage. Full thickness cranial defects result from the expansion surgery, with the size directly proportional to the degree of expansion. The growing cranial skeleton has a unique regenerative capacity to heal small defects; however, when this regenerative capacity is exceeded, the defect is classed as one of critical size and requires surgical treatment to restore protection to the underlying brain. Although what constitutes a critical cranial defect is well known in animal models, it is not as clear for pediatric human skulls. The purpose of this study is to investigate a method that can effectively quantify healing of the pediatric cranial defect surface after cranial vault expansion surgery for craniosynostosis.
Cranial vault
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Foramen magnum
Chiari Malformation
Chiari I malformation
Syrinx (medicine)
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Craniosynostosis describes a fusion of one or more sutures in the skull. It can occur in isolation or as part of a syndrome. In either setting, it is a condition which may lead to raised intracranial pressure. The exact cause of raised intracranial pressure in craniosynostosis is unknown. It may be due to; a volume mismatch between the intracranial contents and their containing cavity, venous hypertension, hydrocephalus or airway obstruction, which is often a sequela of an associated syndrome. At Great Ormond Street Hospital, after hydrocephalus and airway obstruction have been treated, the next surgical treatment of choice is cranial vault expansion. This expansion has been shown to reduce intracranial pressure, interestingly despite its success, the reasons behind its benefits are not fully understood. Using reconstructed 3-dimensional imaging, accurate measurement of cranial volumes can now be achieved. The aim of this project is to use the advances in 3-dimensional imaging and image processing to provide novel information on the volume changes that occur following cranial vault expansion. This information will be combined with clinical metrics to create a greater understanding of the causes of raised intracranial pressure in craniosynostosis, why cranial vault expansion treats them and whether there is an optimal volume expansion.
Cranial vault
Crouzon syndrome
Intracranial pressure monitoring
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