2015 HSC Section 1 Book of Articles

with syndromic craniosynostoses, but its role in

cranial vault surgery is still limited. Steinbacher

et al.

[28] published a case series of eight syndromic

patients who underwent posterior cranial vault dis-

traction osteogenesis using mandibular distractors.

They were successful in expanding the posterior

cranial vault (mean advancement of 23mm) and

state that the technique allows greater advancement

due to the expansion of the constricting scalp.

However, there are several limitations of distrac-

tion osteogenesis for the cranial vault, including

the absence of devices specific to the cranial

vault, the need for a second surgery to remove the

devices, and the inability to mould gross calvarial

deformities.

Rare cases of treating the adult patient with

craniosynostosis have been reported. Marchac

et al.

[29] reported on a series of 13 patients (mean

age 24 years); 11 underwent cranial vault remodel-

ing and two had camouflage surgery with polyme-

thylmethacrylate implant and correction of nasal

deformities. Cranial vault remodeling for patients

presenting later in life is primarily a cosmetic pro-

cedure involving significant operative risk, as well as

the risk of irregular contour deformities from their

less malleable bone. The authors indicated the

exception in one patient with intractable headaches

and copper beating of the skull indicative of

increased intracranial pressure. The patient’s head-

aches resolved after surgery. The authors advocate

avoiding radical cranial vault remodeling in the

adult patient presenting with limited frontal asym-

metry, reserving cranial vault remodeling for

patients with neurological signs or symptoms or

those with orbital dystopia.

There is no consensus on the best operative

procedure. Proponents of endoscopic suturectomy

claim shorter operative time, less blood loss and

transfusion requirements, and shorter hospital stay.

Advocates of cranial vault remodeling argue that

contemporary surgery is much safer and new bench-

marks are necessary to compare the morbidity of

each procedure [30

]. In 1979, Whitaker

et al.

[31]

reported 2.2% mortality and 25.7% complication

rate in a combined trial of 793 craniofacial oper-

ations. In 2010, Seruya

et al.

[30

] found a compli-

cation rate of 3.3% in 212 patients who underwent

craniofacial operations (two cerebral contusions,

two hematomas, one cerebrospinal fluid leak, one

infection, and one wound breakdown). Improved

outcomes can be attributed to specialized anesthesi-

ology and the use of controlled intraoperative hypo-

tension and improved critical care. The senior

author feels that sagittal synostosis in patients under

6 months of age can be treated with craniectomy

and barrel stave osteotomies followed by helmet

therapy with predictable outcomes. Other presenta-

tions of nonsyndromic craniosynostosis are most

predictably managed with cranial vault remodeling

between ages 6 and 9 months, and rarely is helmet

therapy of benefit following surgery.

Timing of surgery is determined primarily

by the choice of surgical procedure, as described

above. Suture release procedures such as endoscopic

suturectomy, spring-mediated distraction and the

pi procedure are usually done earlier than 6 months

of age. Cranial vault remodeling is performed

between 4 and 13 months of age. We prefer to

delay cranial vault remodeling until greater than

6months of age as before this time the bones are too

malleable to retain their shape following surgical

correction. In addition, delaying major cranial

vault surgery until after 6 months provides a larger

infant who can tolerate extended surgery better

than the neonate.

CONCLUSION

The management of nonsyndromic craniosynosto-

sis is rapidly evolving with the introduction of

alternatives to cranial vault remodeling. Cranial

vault remodeling remains the gold standard treat-

ment and allows contouring without relying on

the underlying expanding brain, but the technique

is limited by significant morbidity. Newer mini-

mally invasive techniques include strip craniectomy

with helmet therapy, spring-assisted cranioplasty

and distraction osteogenesis for posterior vault

remodeling.

Acknowledgements

None.

Conflicts of interest

There are no conflicts of interest.

REFERENCES AND RECOMMENDED

READING

Papers of particular interest, published within the annual period of review, have

been highlighted as:

of special interest

of outstanding interest

Additional references related to this topic can also be found in the Current

World Literature section in this issue (p. 342).

Knoll B, Persing JA. Craniosynostosis. In: Bentz ML, Bauer BS, Zuker RM,

editors. Pediatric plastic surgery. 2nd ed. St Louis: Quality Medical Publish-

ing; 2008. pp. 541–563.

Cohen MM Jr. Epidemiology of craniosynostosis. In: Cohen MM Jr, MacLean

RE, editors. Craniosynostosis. 2nd ed. New York: Oxford University Press;

2000. pp. 112–118.

Persing JA, Knoff B, Duncan CC. Nonsyndromic craniosynostosis. In: Guyur-

on B, Eriksson E, Persing JA, editors. Plastic surgery, indications and practice.

Philadelphia: Saunders Elsevier; 2009. pp. 389–404.

Albright AI, Byrd BP. Suture pathology in craniosynostosis. J Neurosurg

1981; 54:384–387.

Sommering S. Of the human body [in German]. 2nd ed. Leipzig, Germany:

Voss; 1839.

Maxillofacial surgery