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Craniostenosis

With Notes on a Modified Operation for the Brachycephalic Form

David Fairman and Gilbert Horrax

life and triples its weight in 2½ years, completing 80 per cent of its entire growth in the first 3 years of life. For this reason craniostenosis has practical importance when it occurs during the intra-uterine period or first year of life. Based on anatomical and clinical facts we have suggested the following classification: 4 Premature closure of the sagittal suture results in a long narrow skull (scaphocephaly) and early synostosis of the coronal suture develops a broad, short and high skull (brachycephaly). When premature synostosis affects practically

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David Fairman and Gilbert Horrax

to distinguish between chondrodystrophies and scaphocephaly or oxycephaly, his great merit consists in his observations that the premature synostosis of the sutures was the direct cause of the restriction of growth and, therefore, the primary cause of the cranial deformity, terming these conditions craniostenosis. PATHOLOGIC ANATOMY In order to understand the mechanism by which these conditions are produced, it is necessary to review the most important facts regarding the development of the skull in infancy. From the embryologic standpoint, the cranium is

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Dominique Renier, Christian Sainte-Rose, Daniel Marchac and Jean-François Hirsch

T he need for surgery is obvious in many cases of craniosynostosis, either because the patient's family is motivated by the aesthetic problem or because the child presents clinical signs of intracranial hypertension. In other cases, especially when only one cranial suture is involved or when the child is over 3 or 4 years old, it is more difficult to make a decision whether to operate, because it is then usually impossible to infer increased intracranial pressure (ICP) from the clinical features alone. Moreover, in craniostenosis, true papilledema is uncommon

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Christian Sainte-Rose, Jacques LaCombe, Alain Pierre-Kahn, Dominique Renier and Jean-François Hirsch

V arious degrees of hydrocephalus are known to be associated with craniostenosis in some patients and with most cases of achondroplasia. 8, 13, 26, 29, 36 In a previous article, 26 we have shown that several clinical, radiological, and surgical arguments favor the hypothesis that, in achondroplasia, hydrocephalus is caused by an impairment of the venous outflow from the brain. In these cases, the computerized tomography (CT) scans show an enlargement of the ventricles and of the cerebral sulci, and angiography and sagittal sinus venography demonstrate a

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William A. Roy, Robert J. Iorio and Glenn A. Meyer

. Coleman EN , Foote JB : Craniostenosis and familial vitamin-D-resistant rickets. Br Med J 1 : 561 – 562 , 1954 Coleman EN, Foote JB: Craniostenosis and familial vitamin-D-resistant rickets. Br Med J 1: 561–562, 1954 10. Cowgill LD , Goldfarb S , Lau K , et al : Evidence for an intrinsic renal tubular defect in mice with genetic hypophosphatemic rickets. J Clin Invest 63 : 1203 – 1210 , 1979 Cowgill LD, Goldfarb S, Lau K, et al: Evidence for an intrinsic renal tubular defect in mice with

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Computerized tomography of cranial sutures

Part 2: Abnormalities of sutures and skull deformity in craniosynostosis

Yoshimi Furuya, Michael S. B. Edwards, Charles E. Alpers, Brian M. Tress, David Norman and Douglas K. Ousterhout

Akrocephalosyndaktylie. Z Kinderheilk 90 : 301 – 320 , 1964 Pfeiffer RA: Dominant erbliche Akrocephalosyndaktylie. Z Kinderheilk 90: 301–320, 1964 18. Renier D , Sainte-Rose C , Marchac D , et al : Intracranial pressure in craniostenosis. J Neurosurg 57 : 370 – 377 , 1982 Renier D, Sainte-Rose C, Marchac D, et al: Intracranial pressure in craniostenosis. J Neurosurg 57: 370–377, 1982 19. Saethre H : Ein Beitrag zum Turmschädelproblem (Pathogenese, Erblichkeit und Symptomatologie). Z

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K. Maurits Persson, William A. Roy, John A. Persing, George T. Rodeheaver and H. Richard Winn

, Towne EB: Early operation in premature cranial synostosis for the prevention of blindness and other sequelae. Five case reports with follow-up. J Pediatr 22: 286–307, 1943 9. Foltz EL , Loeser JD : Craniosynostosis. J Neurosurg 43 : 48 – 57 , 1975 Foltz EL, Loeser JD: Craniosynostosis. J Neurosurg 43: 48–57, 1975 10. Freeman JM , Borkowf S : Craniostenosis. A review of the literature and report of thirty-four cases. Pediatrics 30 : 57 – 70 , 1962 Freeman JM, Borkowf S

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Per Alberius and Göran Selvik

, craniostenosis may not be apparent until the child is more than 1 or 2 years old. In these patients, early routine examinations do not reveal any abnormalities associated with the calvarial sutures. Evidently, “late” premature suture fusion is possible, although the etiology is obscure. The compensatory reactions of nonfused cranial vault regions are also unclear, both in relation to duration of suture growth restriction and the following surgical intervention. The purpose of this investigation was to elucidate these intricate issues in the rabbit, using highly accurate

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Bodil Rune, Göran Selvik, Sven Kreiborg, Karl-Victor Sarnäs and Erik Kågström

✓ Craniectomy was performed on a boy with Crouzon's disease at 22 months of age. Metallic implants (tantalum balls, 0.5 mm in diameter) were inserted in the calvaria during surgery, and the child was examined postoperatively by roentgen stereometry at intervals of about 100 days (total observation time, 309 days). The thyroid radiation dose was 250 µGy for one examination. The effect of craniectomy was recorded with a high degree of accuracy in terms of motion of bones and volume changes in the calvaria. Expansion occurred almost entirely through motion of free bone flaps in the frontal region, while a linear craniectomy in the region of the sagittal suture caused small changes. The rate of expansion decreased rapidly and stabilization was recorded about 250 days after surgery.