Surgery for Craniosynostosis Workup

  • Author: John A Jane Jr, MD; Chief Editor: Allen R Wyler, MD   more...
 
Updated: Apr 2, 2010
 

Laboratory Studies

  • No particular laboratory tests are specific to the workup for craniosynostosis. All patients undergoing surgical treatment have their hemoglobin and electrolyte levels checked to ensure these levels are within the reference range so they can be submitted to general anesthesia.
  • In some institutions, anticoagulation studies are performed preoperatively, although this is not a universal policy. Certainly, craniofacial deformities are not associated with an increased risk of coagulation defects.
  • Cranial remodeling operations are associated with significant blood loss, and the usual practice is to secure at least 3 units of suitable cross-matched blood before surgery. As part of general investigations, blood samples are obtained for use in chromosomal analysis.
Next

Imaging Studies

  • From the early days of craniofacial surgery, radiological investigations were used to study the morphology of the cranial and facial skeleton and monitor the effects of corrective surgery. Initially, plain radiographs, later CT scan, and recently MRI have been used both in clinical practice for preoperative planning and postoperative follow-up and in research, exploring the pathophysiological mechanisms implicated in causing the skull deformity. Common practice in most institutions includes at least plain radiographs and CT scans prior to surgery. MRI scans are used in long-term follow-up of syndromic cases or in children presenting late, after the first few months of life.
  • Plain radiographs are easy to obtain and demonstrate osseous anatomy well. Plain radiographs are used extensively in patients with craniosynostosis. They are useful for identifying the prematurely fused sutures; demonstrating the overall morphology of the cranium; identifying the presence of localized problems, eg, constricting bony bands impairing growth; and identifying the presence of generalized problems, eg, the copper-beaten appearance, indicating intracranial hypertension (see the image below). Sagittal craniosynostosis Sagittal craniosynostosis
    • Serial radiographs in borderline cases or in patients with possible recurrent stenosis after surgical treatment are used to assess the evolution of the deformity.
    • The use of standardized views allows measurement of cephalometric features and serial follow-up. Several indices have been devised and used for comparisons. The most popular index has been the cranial index described by Cronqvist.[10] A considerable body of scientific work exists that explores the value of cephalometric studies on the growth of the head and face in a normal state and in craniosynostosis, in which the effect of corrective surgery can be monitored.
    • Growth of the cranial vault and maturation of the facial skeleton have been demonstrated to occur at different ages. Also, different types of craniosynostosis have been shown to have differential growth of the skull vault and skull base, caused by differential pathological involvement of the sutures of the anterior and posterior skull base, supporting earlier views that the sutures of the skull base are implicated in the morphogenesis of some types of craniosynostosis.
    • In the case of positional posterior plagiocephaly, the plain skull radiographs show normal lambdoid sutures.
  • The introduction of CT scan provided a more detailed way of visualizing the intracranial pathologic condition. Visualization of detailed anatomy of the calvaria and the brain parenchyma became possible. The fused sutures are clearly identified; the abnormal contour of the skull is better appreciated; and the associated cerebral abnormalities, such as hydrocephalus, are demonstrated (see the image below). The relationship of soft and hard tissues of the craniofacial skeleton can be studied in detail. The skull base in particular, which is not clear on plain radiographs, is clear on CT scan images.[11, 12] Unilateral coronal craniosynostosis Unilateral coronal craniosynostosis
    • The principle of cephalometry has also been applied to CT scans. Using various fixed bony landmarks, the growth of various parts of the skull, in particular the skull base and orbital complex, have been investigated in the normal state and in craniosynostosis, and the effect of corrective surgery has been quantified. Skull base growth has been demonstrated to be different in the various types of craniosynostosis, depending on the involved prematurely fused suture, and is morphologically more abnormal for single-suture and nonsyndromic bilateral coronal synostoses than for syndromic forms. In syndromic craniosynostosis, the skull has been shown to be wider at the level of the coronal sutures and shorter at the midsagittal plane. The orbits are smaller, wider spaced, and more protruding, a numerical confirmation of the clinical observations of brachycephaly and exophthalmos.
    • The effect of corrective surgery has been well documented. The morphology of the cranial base has been shown to be normalized following cranial expansion surgery in the single-suture and nonsyndromic bilateral coronal synostoses. Also, following successful surgery for Apert syndrome, persistent brachycephalic features are still demonstrated in the form of wide intercoronal distance and short skull anteroposterior length, while the vault remains tall, although the shape of the skull has changed. Following surgery, the brain has been shown to be slow to expand; the new space is mostly occupied by cerebrospinal fluid (CSF), and, often, persistent empty extradural space at the site of frontal advancement remains in the early postoperative period.
  • The development of 3D-CT added a new dimension to imaging craniosynostosis. The technique is particularly appropriate for bony structures, and images can demonstrate detailed skull anatomy with a perspective view, which is particularly helpful for understanding complex craniofacial deformities. The skull vault and base deformities can be appreciated in the 3-dimensional space (see the image below). Features such as a shallow anterior fossa, deformed dystopic orbits, abnormal calvarial contour, and an asymmetric cranial base can all be visualized realistically. This imaging modality has proven valuable for operative planning and assessment of the results of surgery.[13] Lambdoid craniosynostosis Lambdoid craniosynostosis
  • MRI offers improved definition of intracranial soft tissue structures compared with CT scan. Only recently, attention extended beyond the skull bones toward their contents. A wealth of information is gradually emerging. A high incidence of acquired hindbrain herniation has already been shown to occur in children with syndromic craniosynostosis, particularly children with Crouzon and Apert syndrome. Hindbrain herniation has been observed in as many as 70% of these patients (see the image below). Hindbrain herniation has been demonstrated to be caused by early closure of the lambdoid sutures. Other abnormalities assessed well using MRI are hydrocephalus and cerebral developmental defects, such as myelination defects and deformities of airway pathways, associated with the maxillary hypoplasia seen in Crouzon and Apert syndromes. Girl aged 11 months with Apert syndrome, with bracGirl aged 11 months with Apert syndrome, with brachycephaly and turricephaly. The low-set ears and flat eyebrows are indicative of the deformed skull base. The maxillary hypoplasia is seen clearly, even at this age, and will become more obvious later on in life.
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Contributor Information and Disclosures
Author

John A Jane Jr, MD  Associate Professor of Neurosurgery and Pediatrics, Department of Neurosurgery, Assistant Program Director, University of Virginia School of Medicine; Director of Pediatric Neurosurgery Program, Co-Director of Neuroendocrine Program, Neurosurgeon, University of Virginia Health System

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.

Coauthor(s)

M Sean McKisic, MD  Resident Physician, Department of Neurosurgery, University of Virginia Health System

M Sean McKisic, MD is a member of the following medical societies: American Association of Neurological Surgeons, American College of Surgeons, American Medical Association, Christian Medical & Dental Society, Congress of Neurological Surgeons, and North American Skull Base Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Scott C Dulebohn, MD  Neurological Surgeon, Appalachian Neurosurgical

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Ryszard M Pluta, MD, PhD  Associate Professor, Neurosurgical Department Medical Research Center, Polish Academy of Sciences at Warsaw, Poland; Clinical Staff Scientist, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health (NIH); Fishbein Fellow, JAMA, Chicago ,IL

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.

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.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthor Spyros Sgouros, MD, FRCS, to the development and writing of this article.

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Normal anatomic suture configuration
Metopic craniosynostosis
Sagittal craniosynostosis
Unilateral coronal craniosynostosis
Lambdoid craniosynostosis
Girl aged 11 months with Apert syndrome, with brachycephaly and turricephaly. The low-set ears and flat eyebrows are indicative of the deformed skull base. The maxillary hypoplasia is seen clearly, even at this age, and will become more obvious later on in life.
Plain skull radiograph of a child with Apert syndrome (same child as in Image 6). The prominent feature is the bilateral coronal synostosis. The prematurely closed coronal sutures are seen as white sclerotic bands. This resulted in a shallow anterior fossa and shallow orbits.
CT scan (bone windows) images of a patient with Apert syndrome. The bilateral coronal synostosis has resulted in a shallow anterior fossa, brachycephaly, and increased biparietal distance. The orbits are shallow. The ventricular system has a normal configuration (same patient as in Images 6 and 7).
Three-dimensional CT scan of a patient with Apert syndrome. The 3-dimensional visualization of the anatomy of the skull offers a more realistic interpretation of the pathological process of craniosynostosis. The anterior fossa is shallow, and the prematurely fused coronal suture is seen as a prominent ridge in the inner surface of the skull. Also, the posterior fossa is shallow. The facial skeleton and the relationship of the facial skeleton with the skull base can also be appreciated (same patient as in Images 6, 7, and 8).
MRI scan of a patient with Apert syndrome. The abnormal configuration of the brain parenchyma is seen. The multiple-suture synostosis has resulted in oxycephaly, with corresponding distortion of the corpus callosum and the ventricular system. The posterior fossa is shallow and a degree of hindbrain herniation is present (same patient as in Images 6, 7, 8, and 9).
Fronto-orbital advancement techniques indicated in any form of bilateral coronal synostosis. On the left, the lines indicate osteotomy sites. The fused coronal sutures are removed (shaded area), and a free supraorbital bar is created and moved forward to enlarge the anterior fossa. The resulting free frontal bone flap is used to reconstruct the forehead, often rotated 180°. In the middle figure, the "floating forehead" technique is shown. The forehead is disconnected from the coronal suture system and the skull base by extending the bone removal in the infratemporal fossa. The supraorbital bar is fixed to the zygomatic bone. On the right, the "tongue-in-groove" technique is shown. The supraorbital bar is fixed to the adjacent temporal bone, in a purposefully created groove, with the goal of minimizing the bone defect under the temporalis muscle.
Postoperative photograph of a 4-year-old girl with Apert syndrome. She had fronto-orbital advancement when aged 12 months. The orbits are well covered as a result of the advancement, but the ears remain low-set and the turricephaly has not changed significantly. The cosmetic appearance is satisfactory (same patient as Images 6, 7, 8, 9, and 10).
Sagittal craniosynostosis.
Bicoronal incision with placement of Raney clips.
Full view of skull from patient's right.
Intraoperative planning.
Separation of the anterior fontanelle from the surrounding skull.
Removing the sutures with the craniotome.
After suture removal.
Reshaping the bossed frontal bone.
Evaluating placement of the reshaped frontal bones.
Final configuration secured in place.
Final result after closing.
Left unilateral craniosynostosis.
Left unilateral craniosynostosis.
Removing the frontal bones.
Removing the superior orbital rim.
Superior orbital rim.
Remodeling the superior orbital rim.
Superior orbital rim remodeled.
Completed correction of left unilateral coronal craniosynostosis.
The closure is complete.
 
 
 
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