See the corresponding editorial in this issue, pp 330331.

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Historical vignette

Vault reduction cranioplasty for extreme hydrocephalic macrocephaly

MARLON S. MATHEWS, M.D.,1 WILLIAM G. LOUDON, M.D., PH.D.,1,2 MICHAEL G. MUHONEN, M.D.,1,2 AND MICHAEL J. SUNDINE, M.D.3
Department of 1Neurosurgery and 3Aesthetic and Plastic Surgery, University of California, Irvine; and 2 Neurosciences Institute, Childrens Hospital of Orange County, Orange, California
Due to early diagnosis and treatment of hydrocephalus, neurosurgeons rarely are called upon to treat patients with extreme hydrocephalic macrocephaly. Macrocephaly can limit mobility and hygiene. The critical evaluation and surgical correction of the morphological problem of macrocephaly secondary to hydrocephalus is complex. Various techniques such as quadrantal, picket fence, crossbar, and modified techniques have been used to reduce the size of the cranial vault to decrease cranial volume while achieving good cosmesis. Limitations of vault reduction cranioplasty include the inability to alter the anteroposterior and lateral diameters of the skull base, the inability to shorten the superior sagittal sinus, and the need to avoid infolding of the brain due to the risk of venous infarcts. Reduction cranioplasty is indicated in the occasional patient whose large head size represents a mechanical or cosmetic problem of sufficient magnitude to seriously interfere with motor development and functioning, with resultant development of pressure sores and difficulties with nursing care. Reduction cranioplasty should be avoided in patients under the age of 3 years. (DOI: 10.3171/PED-07/10/332)

KEY WORDS cranioplasty hydrocephalus macrocephaly pediatric neurosurgery vault reconstruction vault reduction

measurement of head circumference, a direct reflection of head growth, is an important step in the evaluation of childhood growth and development. Deviations from normal may be the first indication of an underlying congenital, genetic, or acquired problem. When the head circumference is greater than two standard deviations above the mean for a given age, a diagnosis of macrocephaly is made. The most common cause of macrocephaly is hydrocephalus. Untreated or partially treated hydrocephalus can cause massive enlargement of the head. Rarely, macrocephaly can give rise to serious problems in psychosocial development, hygiene, and aesthetics secondary to compromised head mobility from a heavy fluid-filled enlarged head.1 Presentation of children with extreme macrocephaly (head circumference 60 cm) secondary to hydrocephalus is uncommon due to the early treatment of hydrocephalus using various shunting devices and techniques.

HE

History of Cranial Reduction Surgery and Operative Techniques Used A number of surgical techniques for performing reduction cranioplasty have evolved over the years. In all these techniques the common goal is reduction in cranial vault size. The positioning of the patient and the number of stages vary with surgical technique. While CSF shunting procedures were still in their infancy, Ehni1 carried out reduction of head size in a patient with advanced hydrocephalus, between the years 1951 and 1953, using specialized gap-closing devices designed by the patients father. The surgical treatments were performed in 13 stages over a 22-month period. Since then several operative techniques have evolved, involving single or multiple stages and varying positioning of the patient (Figs. 15). Some of these techniques are compared in Table 1. In 1964, Sayers and Duran17 reported a reduction cranioplasty in a hydrocephalic patient; the surgery had been
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Abbreviation used in this paper: CSF = cerebrospinal fluid.

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FIG. 1. Drawing showing the sagittal (open arrow) and occipital (solid arrow) bandeaus. The bandeaus are fashioned from the removed calvaria. The dura mater covering the superior sagittal sinus is suspended from the sagittal bandeau, eliminating the need for dural placation while maintaining adequate curvature of the sinus. Remaining portions of the calvaria are then fashioned in desired dimensions and attached to the sagittal and occipital bandeaus, as well as the skull base, with absorbable plates and suture.

planned as a four-stage procedure, but the parents refused the final frontal reduction stage. The first stage was placement of a shunt, so the reduction cranioplasty involving the posterior two thirds of the calvaria was carried out in two of the three final procedures. In 1979, Vries and Habal22 described a five-stage procedure (two stages for cranial vault reduction) in which they utilized a sagittal strut of bone as the foundation to replace the reduced cranial pieces in conjunction with supraorbital repositioning. The first stage was for general reduction and was performed with the patient in a prone position; the final (fifth) stage was for supraorbital repositioning and was performed with the patient supine. The surgeons achieved a 63% reduction in intracranial volume with no postoperative complications. Ventureyra and Da Silva21 completed a single-stage reduction in a 3-yearold hydrocephalic patient using semisitting positioning, bitemporal incision, and a sagittal strut. Both halves of the cranial vault were fragmented, and the pieces were then fastened together in the desired dimensions using heavy silk. The procedure lasted 8 hours and required replacement of 500 ml of blood. In 1985, Park and colleagues13 reported performing a reduction cranioplasty as a one-stage procedure in which they utilized their previously reported modified prone positioning and were able to achieve a 50% reduction in cranial vault volume. This modification allowed exposure from nasion to inion. The authors anterior craniotomy was performed in a supraorbital position and included vertical osteotomies and greenstick fracturing as well as a crossbar craniotomy support structure supporting in sagittal and coronal directions.14 Piatt and Arguelles16 reported
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FIG. 2. Drawing illustrating the quadrantal technique. The cranial vault is removed in four quadrantal plates. After the CSF is removed via a ventricular catheter, the dura is imbricated. Each bone plate is reduced in size and molded as necessary. Some or all of the plates may be subdivided and then reassembled with wire or plate fixation to produce a smaller, better-contoured quadrant. The new plates are then secured in place and the dural envelope is reinflated with CSF. Arrows indicate reinstitution of plates molded in required dimensions.

on three patients treated with reduction cranioplasty for craniocerebral disproportion; the procedures involved complete removal of the calvaria, creation of a coronal crossbar, and delayed placement of a permanent shunt for CSF drainage. In 1995, Winston et al.23 reported on reduction cranioplasty in four patients with macrocephaly, in two of whom a two-stage procedure was used. Takahashi and colleagues19 also reported performing multistage reduction cranioplasty (one four-stage procedure and one two-stage procedure) in two children with severe macrocephaly secondary to hydrocephalus. In 1998, Erdinler et al.2 reported using the modified crossbar and modified techniques in single-stage procedures. Sundine et al.18 reported a single-stage procedure utilizing a modification of the procedure reported by Park and colleagues for prone positioning using a sagittal bandeau and frontal, temporoparietal, and occipital bone flaps bilaterally.
Indications for Reduction Cranioplasty

The consequences of untreated craniocerebral disproportion are severe. Piatt and Arguelles16 described them as follows: 1) mechanical instability of cortical mantle that may lead to intermittent hemorrhage and successful shunting becoming problematic; 2) the need for repetitive shunt revisions that may result in infection; 3) extreme enlargement of the skull that may lead to failure of head control, problems with positioning, and skin breakdown; and 4) deformity
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FIG. 3. Drawing illustrating the picket-fence technique. Relatively thin and malleable bone is a prerequisite for this technique. The anterior vault is removed and the bone plates are reduced in size, molded, and repositioned. After detailed planning, osteotomies are carried out in the parietooccipital vault to create triangular pickets. The bone pickets are then bent and molded across the vertex and secured with wires and microplates, establishing sound structural stability. Drainage of CSF, imbrication of dura, and reinflation with CSF are carried out as in the quadrantal technique.

FIG. 4. Drawing illustrating the crossbar technique. Osteotomies are made in the calvaria in the manner shown. The four quadrants (smaller than in the quadrantal technique) are removed. All four limbs of the crossbar are shortened (not necessarily equally). Structural stability is established by firmly securing each limb of the crossbar to the cranial base. Each remaining plate is reduced in size, contoured as necessary, and then attached to the crossbar and also to the cranial base. Drainage of CSF, imbrication of dura, and reinflation with CSF are the same as in the quadrantal and picket fence techniques.

leading to insurmountable obstacles to social acceptance. The indications for reduction cranioplasty are listed in Table 2. Briefly, this procedure is indicated when extreme hydrocephalic macrocephaly results in arrest of motor development and significantly limits mobility, hygiene, and the provision of nursing care. Because infants with macrocephaly often show only mild psychomotor developmental delay later in life, cranial vault reduction should be reserved until children are 3 years of age of older.
Outcome in Published Cases

According to Ehni,1 preoperatively the child that he treated used to lie on his back and would move about by digging his heels into the carpet and dragging his heavy head behind his body. After 13 procedures, Ehni reported a decrease in the childs inter-ear diameter from 53 to 42 cm. Postoperatively, the child developed the ability to walk using a walker although he remained ataxic even while sit334

ting. The child remained alert, may have experienced improvement in intellectual function, and demonstrated good control of his head. The aesthetic results of the surgical procedure were reportedly considered to be an improvement over the childs preoperative condition by the childs parents and the operating physician as well as by other observers. Takahashi and colleagues19 operated on two patients for severe macrocephaly. The first patient, an 8-year-old boy, experienced functional improvementfrom not being able to support his head without assistance to walking independentlypostoperatively. His head circumference was reduced from 85 to 68 cm as a result of the procedure. The head circumference of the second patient, a 3-year-old boy, was reduced from 54 to 50 cm, and the patient showed some developmental progress. Of the series of three patients reported on by Winston et al.,23 one patient died, another showed some improvement in head support, and the third showed good growth and development (although development was slightly delayed). The two patients reported on by Erdinler et al.2 showed improvements in head circumference and nasioninion and biparietal distances, respectively, from 72, 70, and 70 cm to 62, 44, and 43 cm in the first patient; and from 74, 71, and 68 cm to 59, 46, and 42 cm in the second. The condition of the first patient improved to the point of his being able to stand and independently support his head, but the child died
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reported significant improvements in the condition of a 1year-old girl postvault reduction with contraction osteogenesis. The patient, who preoperatively was unable to lift the weight of her own head, walk, or crawl, and had a Grade II occipital decubitus ulcer, was able to walk and hold her head up in a stable midline position after treatment. Thomson and Hoffman20 reported significant cosmetic benefits in a 2-year-old who presented with an unsightly enlarged head with patent metopic sutures. At 2-year follow-up the child was described as rather attractive with improved motor and cognitive function. Vries and Habal22 reported on a 6year-old child who had been severely incapacitated by the weight of his head preoperatively and 6 months postoperatively had developed full control of his head. The patients head circumference decreased from 73 to 50 cm representing a 63% volume reduction. Ventureyra and Da Silva21 reported head circumference reduction and cognitive gains in a 3-year-old boy who underwent surgery for macrocephaly. Piatt and Arguelles16 reported the results of surgical cranial vault reduction in three infants (ages 3, 2, and 3 months) with successful reduction in head circumference in all three, compensatory development in the first child, and modest benefits in the other two. In a recent series of four cases reported by Sundine et al.,18 all the patients survived without any significant postoperative morbidity. The indication for surgery in all patients was to facilitate nursing care. The underlying conditions were congenital hydrocephalus in two patients and holoprosencephaly with hydrocephalus in the other two. The averages for blood loss, operating room time, and duration of intensive care unit stay were 1037.5 ml, 9.23 hours, and 16.25 days, respectively. All four patients had severe developmental delay preoperatively; none had any language development, nor could any of them walk. One of the four patients showed significant compensatory development at the 1-year follow-up examination, including initiation of language development and walking without assistance. In the other three patients no significant developmental advancement was seen, but nursing care was significantly improved, with reduction of occipital decubitus ulceration and easier head lifting. Discussion Hydrocephalus is a pathological condition characterized by increased ventricular volume that is not the result of primary atrophy or dysgenesis of the brain. In most cases, this accumulation of CSF results from anatomical obstruction to the egress of CSF and is associated with present or previous elevation of CSF pressure.11 Congenital hydrocephalus is hydrocephalus of unknown cause present in utero and discovered in the newborn.12 Quantitative measures of hydrocephalus include absolute measurements, corrected for the reduction in size that occurs in imaging, as well as patient age and ratios of the width of the ventricles to standard landmarks on the skull. Ratios most frequently used to assess hydrocephalus by computed tomography include the Evans ratio,3,4,15,24 the frontal horn ratio,5 the ventricular size index,6 the Huckman number,7 and the cella media index.9,10 Once the diagnosis of hydrocephalus is made, early treatment generally allows for prevention of macrocephaly. Total cranial vault reduction cranioplasty is an uncommon procedure. It is rare that a surgeon is called upon to evalu335

FIG. 5. Drawing illustrating the modified technique. Two paramedian osteotomies are created after removal of a bifrontal bone flap. The craniotomy is extended first posteroinferiorly and then anteroinferiorly toward the temporal bone. The bifrontal bone flap is reformed to the desired shaped and size before being replaced. The calvaria that remains after removal of the two bone strips is then reapproximated with a resultant decrease in cranial vault size. The size of the bifrontal bone flap and the paramedian bone strips can be varied from patient to patient to achieve the desired results.

4 months postoperatively from peritonitis possibly related to ventriculoperitoneal shunt infection. The second patient, who was unable to support the weight of her head preoperatively, was able to stand up with minimal assistance postoperatively. She had a cosmetic defect, however, due to unequal growth of her reconstructed bones. Mansour et al.8
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TABLE 1 Advantages and disadvantages of various surgical techniques in reduction cranioplasty
Technique Advantages Disadvantages

quadrantal plate technique* excellent control of resulting contour of cranial vault difficulty in obtaining reliable structural stability equally satisfactory for all cranial sizes & shapes inappropriate for patients w/ malleable cranial bones picket fence technique* appropriate for patients w/ extremely thin & malle- difficult in older children & adults w/ a thick & able cranial bones & those w/ widely separated rigid cranium sutures & large fontanelles removal of entire sagittal suture, potentially limideal in infants iting subsequent vertical expansion absence of free bone flaps, maximizing chance of healing crossbar technique* good structural stability limits extent to which curvature of sagittal plane well suited for staging can be altered sagittal sinus not stripped from overlying bone, dedifficult in patients w/ large open anterior fontacreasing risk of tearing or kinking nelle or separated sutures modified technique advantageous when large reduction in the anteropos- possibility of cosmetic problems due to impropterior diameter of the cranium is required (for exer apposition (stepping) between frontal & ample, in scaphocephaly) parietal bones * As described by Mansour et al. As described Vries and Habal.

ate or treat patients with macrocephalic hydrocephalus in this era of early diagnosis and treatment of hydrocephalus. Winston et al.23 discuss six tactical considerations for vault reduction procedures, as follows. 1) Operative positioning should be planned preoperatively with the anesthetiststhe size and weight of the head, calvarial thickness, and separation of the sutures being important factors. 2) Careful attention should be given to intraoperative CSF drainage, as inadequate drainage can cause increased intracranial pressure on closure, while overdrainage can cause the dural covering to lose its opposition to the overlying bone, sag, and fold over on itself from gravity. This can cause direct mechanical damage to the brain or ischemic damage from vascular impairment. 3) In the management of hydrocephalus, they propose that the intracranial pressure in the immediate postoperative period should be maintained slightly above normal to keep the dura snugly against the calvarial bone. 4) Redundant dura can be imbricated along multiple longitudinal elliptical strips parallel to the superior sagittal sinus. 5) Kinking or obstruction of the venous sinuses should be avoided during the operative procedure, and dural imbrications should be planned to avoid this complication. Sundine and colleagues18 suspended the dura around the superior sagittal sinus to the sagittal bandeau to avoid this complication. 6) Winston et al. recommend avoiding resection of redundant scalp in general, although in patients with grossly excess scalps causing folds, excision of excess scalp is suggested. While the strategic goal of reduction cranioplasty is to improve the patients quality of life, the technical goal is reduction of cranial volume. Macrocephaly exists in many
TABLE 2 Indications for reduction cranioplasty
large head size (60 cm), patient age 3 yrs, & at least one of the following due to head size &/or weight: limitation of normal development or activities reduced quality of life (even if patient is neurologically impaired) impediment to nursing care (even if neurologically impaired) cosmetic impairment severe enough to interfere w/ psychosocial development or function

shapes, and the technique selected must fit the specific structural configuration. No one technique is best for all patients. It is extremely important to develop a surgical plan preoperatively in terms of the placement of osteotomies, the identification of areas of bone to be removed and contoured, and the establishment of structural stability of the new vault. Generally one-stage calvarial reconstruction is preferable to multistage operations,13 although decisions regarding staging should be made on a case-by-case basis. One of the limitations of vault reduction cranioplasty is that although the vault size can be reduced, the anterioposterior and lateral diameters of the skull base remain unaltered. Also, the sagittal sinus cannot be shortened. Although it seems possible to shorten the anterior one third of the sagittal sinus, such an attempt would entail a risk of injury to the brain. It is indeed possible that vault reduction results in marked folding of the brain with resultant venous infarcts. These could go undetected in this patient population unless specifically looked for. While vault reduction cranioplasty may be an option in properly selected patients, it is clearly not without risks. On the basis of our review on the procedure, we estimate a mortality rate of 4.35% based on currently published data (one death in 23 reported cases). Because cases with adverse outcomes are unlikely to have been reported in the literature, it is possible that the actual mortality rate is higher. The decision to proceed with total cranial vault reduction cranioplasty is complex. The surgeon should only proceed after careful, critical analysis of the underlying cause of the pathological condition, clear discussions with all relevant parties regarding the risks, potential benefits, possible alternatives, and proper preoperative planning. Limitations of vault reduction cranioplasty include the inability to alter the anteroposterior and lateral diameters of the skull base, the inability to shorten the superior sagittal sinus, and the need to avoid infolding of the brain due to the risk of venous infarcts. Conclusions Reduction cranioplasty may be an option for patients with macrocephalic hydrocephalus who have very large heads
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and in whom the size and weight of the head significantly interfere with motor development. Not all patients are good candidates for reduction cranioplasty. The procedure is indicated in the occasional patient whose large head size is a mechanical or cosmetic problem of sufficient magnitude to seriously interfere with motor development and function, with resultant development of pressure sores and difficulties with nursing care. Reduction cranioplasty should be avoided in patients under the age of 3 years.
References 1. Ehni G: Reduction of head size in advanced hydrocephalus: a case report. Neurosurgery 11:223228, 1982 2. Erdinler P, Kaynar MY, Canbaz B, Etus V, Ciplak N, Kuday C: Two different surgical techniques for reduction cranioplasty. Childs Nerv Syst 14:372377, 1998 3. Evans WA: An encephalographic ratio for estimating ventricular enlargement and cerebral atrophy. Arch Neurol Psychiatry 47:931937, 1942 4. Evans WA Jr: An encephalographic ratio for estimating the size of the cerebral ventricles: further experience with serial observations. Dis Child 64:820830, 1942 5. Hahn FJY, Rim K: Frontal ventricular dimensions on normal computed tomography. AJR Am J Roentgenol 126:593596, 1976 6. Heinz ER, Ward A, Drayer BP, Dubois PJ: Distinction between obstructive and atrophic dilatation of ventricles in children. J Comput Assist Tomogr 4:320325, 1980 7. Huckman MS, Fox J, Topel J: The validity of criteria for the evaluation of cerebral atrophy by computed tomography. Radiology 116:8592, 1975 8. Mansour N, Sobel L, Lee M, Larumbe J, Stelnicki E: A new method for the treatment of macrocephaly caused by hydrocephalus. Cleft Palate Craniofac J 41:16, 2005 9. Meese W, Kluge W, Grumme T, Hopfenmller W: CT evaluation of the CSF spaces of healthy persons. Neuroradiology 19: 131136, 1980 10. Meese W, Lankseh W, Wende S: Diagnosis and postoperative follow-up studies of infantile hydrocephalus using computerized tomography, in Lanksch W, Kazner E (eds): Cranial Computerized Tomography. Berlin: Springer-Verlag, 1976, pp 424429 11. Milhorat TH: Pediatric Neurosurgery. Philadelphia: F.A. Davis, 1978 12. Naidich TP, Schott LH, Baron RL: Computed tomography in eval16. 17. 18. uation of hydrocephalus. Radiol Clin North Am 20:143167, 1982 Park TS, Grady MS, Persing JA, Delashaw JB: One-stage reduction cranioplasty for macrocephaly associated with advanced hydocephalus. Neurosurgery 17:506509, 1985 Park TS, Haworth CS, Jane JA, Bedford JA, Persing JA: A modified prone position for cranial remodeling procedures in children with craniofacial dysmorphism: a technical note. Neurosurgery 16:212214, 1985 Pedersen H, Gyldensted M, Gyldensted C: Measurement of the normal ventricular system and supratentorial subarachnoid space in children with computed tomography. Neuroradiology 17: 231237, 1979 Piatt JH, Arguelles JH: Reduction cranioplasty for craniocerebral disproportion in infancy: indications and technique. Pediatr Neurosurg 16:265270, 19901991 Sayers MP, Duran RJ: Reduction cranioplasty in hydrocephalus, in Transaction of the Third International Congress of Plastic Surgery. Amsterdam: Excerpta Medica, 1964, pp 828832 Sundine MJ, Wirth GA, Brenner KA, Loudon WG, Muhonen MG, Greene CS, et al: Cranial vault reduction cranioplasty in children with hydrocephalic macrocephaly. J Craniofac Surg 17: 645655, 2006 Takahashi Y, Tajima Y, Okura A, Tokutomi T, Shigemori M, Kiyokawa K: Reduction cranioplasty for macrocephaly. Two case reports. Neurol Med Chir (Tokyo) 39:459462, 1999 Thomson GH, Hoffman HJ: Intracranial use of a breast prosthesis to temporarily stabilize a reduction cranioplasty. Plast Reconstr Surg 55:704709, 1975 Ventureyra EC, Da Silva VF: Reduction cranioplasty for neglected hydrocephalus. Surg Neurol 15:236238, 1981 Vries JK, Habal MB: Cranio-orbital correction for massive enlargement of the cranial vault. Plast Reconstr Surg 63:466472, 1979 Winston KR, Ogilvy CS, McGrail K: Reduction cranioplasty. Pediatr Neurosurg 22:228234, 1995 Zatz LM: The Evans ratio for ventricular size: a calculation error. Neuroradiology 18:81, 1979

13. 14.

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19. 20. 21. 22. 23. 24.

Manuscript submitted April 9, 2007. Accepted June 20, 2007. Address correspondence to: Marlon S. Mathews, M.D., 101 The City Drive South, Bldg. 56, Suite 400, Orange, California 928683874. email: mmathews@uci.edu.

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