eMedicine Specialties > Neurosurgery > Miscellaneous

Craniosynostosis: Treatment

Author: John A Jane Jr, MD, Assistant Professor of Neurosurgery and Pediatrics, Director, Pediatric Neurosurgery Program, Co-Director Neuroendocrine Program, Assistant Program Director, University of Virginia Health System; Neurosurgeon University of Virginia Health System and Martha Jefferson Hospital
Contributor Information and Disclosures

Updated: May 1, 2009

Treatment

Medical Therapy

At some units, patients with positional posterior plagiocephaly, when severe, are treated with plastic caps, which are fitted externally on the head and can gradually manipulate the shape of the skull. No clear benefit has been identified, and they are not always tolerated well. Most cases are mild forms and do not require treatment.

Surgical Therapy

Early surgical treatment of craniosynostosis at the end of the 19th century included mostly linear craniectomies and excision of the affected sutures (suturectomies). At the turn of the century, Cushing observed that the complexity of the disorders in patients with craniosynostosis was such that linear craniectomies were hardly addressing the underlying cause.13 The results of such procedures were unsatisfactory for most types of craniosynostosis, particularly when involving the coronal suture complex. Head shape and exophthalmos did not improve, and further operative treatment was commonly required. For sagittal synostosis, linear craniectomy and excision of the affected suture is still effective when performed in the first few months of life. In older children presenting late with untreated sagittal synostosis, complex cranial vault reconstruction is performed if the severity of the deformity merits treatment.

For other forms of craniosynostosis, involving coronal or metopic sutures, linear craniectomies have been abandoned in favor of more complex cranial expansion and remodeling.14,15 The techniques of fronto-orbital advancement were pioneered in Paris in the late 1960s by Tessier7 and later modified by Marchac.16 In earlier years, the tendency was for monobloc facial advancement, including the forehead and midface in one osseous block. Monobloc facial advancement procedures have now waned in popularity because they constitute extensive surgery with considerable morbidity and less than superior results.

Most cranial expansion procedures performed in children with craniosynostosis constitute variations of fronto-orbital advancement. The general principle of these procedures is the independent mobilization of the supraorbital bar with a series of facial osteotomies in the appropriate sites of the medial, superior, and lateral orbital walls and the frontal bone. Subsequent advancement and stabilization of the supraorbital bar in a new more anterior position results in expansion of the floor of the anterior fossa and the roof of the orbits. A new forehead is reconstructed with frontal bone flaps designed appropriately. With this technique, the connection of the coronal suture complex with the skull base is disrupted. This standard technique is used for most types of bilateral coronal synostosis, both isolated and syndromic.

Several variations of this technique have been described, differing mainly on the alternative possibilities of fixation of the lateral ends of the supraorbital bar on the adjacent temporal or zygomatic bones. The 2 most frequently used techniques are the "floating forehead" and the "tongue-in-groove" (see Image 7). The former completely disconnects the supraorbital bar and forehead from the temporal bones, with the goal of allowing complete freedom of growth of the forehead from the skull base. The latter purposely attaches the supraorbital bars to the adjacent temporal bones with internal fixation, with the goal of maintaining synchrony of growth between the realigned forehead and the skull base. Although great claims have been made from proponents of both techniques, the clinical results are comparable.

For different types of craniosynostosis, appropriate modifications are made to the main technique of fronto-orbital advancement. In those with metopic synostosis, the ridge of the prematurely fused suture is excised and the forehead is reconstructed with suitably designed frontal flaps and supraorbital bars. In those with unilateral coronal synostosis, earlier views favored unilateral frontal advancement. Although only one coronal suture is prematurely fused, in fact, the deformity is bilateral because the normal side is attempting to compensate; bilateral forehead correction is usually necessary.

All variations of fronto-orbital advancement usually achieve satisfactory cosmetic results, with good forehead appearance and satisfactory cover of the orbits (see Image 8). A problem often encountered and difficult to correct is persistent narrowing in the temporal regions, observed after any type of fronto-orbital advancement. In addition, abnormalities related to abnormal skull growth, such as turricephaly, brachycephaly, low-set ears, and orbital dystopia, usually persist after successful surgery.

Although fronto-orbital advancement provides satisfactory correction of exorbitism and cosmetic improvement of the forehead appearance, the principle of fronto-orbital advancement does not seem to have any scientific basis. Many studies have indicated that this surgical technique is overcorrecting head volume, producing supranormal head volumes. This explains observations of a high incidence of frontal extradural collections at the site of the advancement after a successful surgery. Similarly, following successful correction of unilateral coronal synostosis, the previously compressed brain does not reexpand; instead, cerebrospinal fluid (CSF) occupies the newly created space. In the absence of another, better alternative, fronto-orbital advancement continues to prevail. It appears to have a cosmetic nature in most cases, excluding children with threatening exorbitism.

In a small, selected group of patients, despite classic syndromic appearances, the predominant problem found after careful appraisal of radiologic images is constriction of the posterior aspect of the skull. Recent attention to the posterior skull has demonstrated that posterior skull release can produce satisfactory results, often obviating the need for frontal advancement.

Syndromic patients with midface hypoplasia may require midface advancement later in life, after age 5 years. Midface advancement can be achieved with either a Le Fort III osteotomy and advancement in one operation or midface distraction osteogenesis. The latter is gaining popularity because, although it confines the patient to wearing an external frame for several weeks, it appears to achieve a better, long-lasting result. In the last 5 years, midface distraction has been developed and refined and is regarded as the technique of choice for many patients.

In recent years, the techniques of distraction osteogenesis have also been used in skull vault remodeling in attempts to avoid the extensive surgical procedure of the forehead advancement. This has also been combined with endoscopic techniques. Although these techniques are still in their infancy, they may become more common practice in the next decade as experience is gained and technology and techniques improve.

Genetic treatment of craniosynostosis with cytokines has been proposed, but it is far from becoming everyday clinical practice at present.

Whenever the issue of possible intracranial hypertension arises, ICP monitoring is usually performed over 24 hours using an invasive bolt. Using an invasive bolt requires a small operation. In some units, routine measurement of ICP is performed in all syndromic cases, although this is not a universal policy.

Patients with hindbrain herniation are commonly asymptomatic and do not require surgery, but a small minority may require surgical management if they develop symptoms or syringomyelia (ie, cavitation and CSF collection inside the spinal cord).

Preoperative Details

Children with significant midface deformity should be assessed by an anesthetist well before surgery because intubation may be a problem, and, in extreme cases, tracheostomy may be required postoperatively.

Postoperative Details

Postoperative imaging is used to demonstrate the new arrangement of the bony architecture of the cranial and facial skeleton. Radiographs obtained early in the postoperative period can be used as references for further assessment. The rate of reossification can be assessed in the regions where dura was left uncovered by calvaria as a result of the advancement.

The evolution of radiological appearances can be particularly useful if the issue of recurrent deformity arises. Although some loss of the advancement is expected in the first few years after operation, other features are also seen in cases of recurrent craniostenosis, such as a localized or generalized copper-beaten appearance and sclerotic hyperdense bands of bone in the calvaria, representing recurrent synostosis.

Recently, the issue of the migration of fixation screws, plates, and wires has gained increasing attention. As the child grows, the skull bones continuously remodel according to natural forces. As a result, the screws used to stabilize the mobilized segment end up buried in the skull or even in the intracranial cavity. Postoperative CT scan images are particularly helpful for monitoring this situation. Although the clinical significance is not known, identification and follow-up of the problem provides an opportunity for appropriate action to be taken. The recent development of bioabsorbable plates and screws may well eradicate the problem of migration.

Follow-up

Typically, each patient has early postoperative plain radiographs, a CT scan in the first few months after operation, and, in syndromic cases, MRI scans at yearly intervals to exclude the development of a hindbrain hernia. In patients with maxillary hypoplasia, yearly plain radiographs may be needed to help assess the progress of the deformity when the issue of possible midface advancement is considered.

Complications

Complications are rare after craniofacial surgery. Hypovolemic shock can occur if significant intraoperative blood loss has not been replaced in a timely manner. Intraoperative dural tears that remain unrecognized can cause postoperative CSF leaks and resultant infection. Epidural or subdural hematoma can occur due to surgical trauma. Almost all patients develop facial swelling postoperatively, more prominent around the eyes, which rarely causes problems. Wound infections are generally rare, even after midface procedures, which, by necessity, involve operating in the oral cavity. The frequency rate of these complications is less than 10%.

ICP bolt insertion has a recognized low complication rate (2%), including intracranial hemorrhage and infection.

More on Craniosynostosis

Overview: Craniosynostosis
Workup: Craniosynostosis
Treatment: Craniosynostosis
Follow-up: Craniosynostosis
Multimedia: Craniosynostosis
References
Further Reading
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Keywords

craniosynostosis, craniostenosis, craniofacial dysostosis, craniofacial defect, craniofacial surgery, cranial sutures, cosmetic facial deformity, cranial deformity, cranial malformation, intracranial hypertension, abnormal skull shape, abnormal head shape, skull growth, Crouzon syndrome, Apert syndrome, Crouzon's syndrome, Apert's syndrome, scaphocephaly, sagittal synostosis, plagiocephaly, unilateral coronal synostosis, posterior plagiocephaly, lambdoid synostosis, trigonocephaly, metopic synostosis, brachycephaly, oxycephaly, turricephaly, cloverleaf skull deformity, triphyllocephaly, pansynostosis, Saethre-Chotzen syndrome, Pfeiffer syndrome, Pfeiffer's syndrome, cranial expansion, mid-face advancement, midface advancement

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Contributor Information and Disclosures

Author

John A Jane Jr, MD, Assistant Professor of Neurosurgery and Pediatrics, Director, Pediatric Neurosurgery Program, Co-Director Neuroendocrine Program, Assistant Program Director, University of Virginia Health System; Neurosurgeon University of Virginia Health System and Martha Jefferson Hospital
John A Jane Jr, MD is a member of the following medical societies: American Association of Neurological Surgeons, American College of Surgeons, Congress of Neurological Surgeons, Endocrine Society, North American Skull Base Society, and Southern Neurosurgical Society
Disclosure: Nothing to disclose.

Medical Editor

Scott C Dulebohn, MD, Neurological Surgeon, Appalachian Neurosurgical
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Ryszard M Pluta, MD, PhD, Associate Professor, Neurosurgical Department Medical Research Center, Polish Academy of Sciences at Warsaw, Poland; Senior Researcher, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, NIH
Ryszard M Pluta, MD, PhD is a member of the following medical societies: Congress of Neurological Surgeons and Polish Society of Neurosurgeons
Disclosure: Nothing to disclose.

CME Editor

Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy
Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences
Disclosure: Nothing to disclose.

Chief Editor

Allen R Wyler, MD, Former Medical Director, Northstar Neuroscience, Inc
Allen R Wyler, MD is a member of the following medical societies: American Academy of Neurological and Orthopaedic Surgeons, American Association of Neurological Surgeons, and Society of Neurological Surgeons
Disclosure: Nothing to disclose.

 
 
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