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Martin M. Mortazavi, R. Shane Tubbs, Maja Andrea Brockerhoff, Marios Loukas and W. Jerry Oakes

herniation occurs as an imprecise continuum ranging from mild tonsillar herniation through the foramen magnum to severe cerebellar and brainstem herniation into a posterior encephalocele leading to syringomyelia, medullary compression, and ischemia; 3) despite their common anatomical location, the 4 anomalies have distinct clinicoanatomical features; and 4) although the large majority of cases are congenital, cases of acquired Chiari I malformation have been reported. Other Early Reports of Hindbrain Herniation Chiari was not the first to observe and report on Type

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R. Shane Tubbs, Daniel B. Webb and W. Jerry Oakes

T he exact mechanism of syrinx formation in the presence of hindbrain herniation is unknown; however, techniques aimed at removing tissue from the region of the midline fourth ventricle outlet, such as decompressive procedures, are used in the treatment of this clinical entity. Some authors have discussed the use of suboccipital craniectomy with and without duraplasty, 14 tonsillar coagulation only, 13 and direct shunting of the fluid cavity. 8 Duraplasty in cases of concomitant syringomyelia may lead to a more reliable reduction in the volume of the syrinx

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R. Shane Tubbs, Matthew D. Smyth, John C. Wellons III and W. Jerry Oakes

cavity confirmed. In some reports the authors have noted intradural “membranes, veils, pouches, and webs” interfering with CSF flow into the craniocervical subarachnoid space. 1–3, 6, 11, 12, 14 To date, few writers have discussed these anomalies in relation to the Chiari I malformation and associated syringomyelia. Clinical Material and Methods We retrospectively reviewed the surgical experience of the senior author (W.J.O.) in the treatment of pediatric Chiari I malformation at two institutions. The first 30 patients in this group underwent surgery at Duke

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Robert P. Naftel, R. Shane Tubbs, Joshua Y. Menendez, John C. Wellons III, Ian F. Pollack and W. Jerry Oakes

T wo broad indications exist for the surgical treatment of CM-I: 1) characteristic symptoms and signs of hindbrain herniation and 2) hindbrain herniation associated with syringomyelia. Postoperatively, patients without syringomyelia are expected to experience improvement in their symptoms. 12 Patients with syringomyelia should have radiological improvement and clinical improvement or stabilization. 5 , 12 , 13 , 16 An unexpected and uncommon result after Chiari decompression is the development of a syrinx in a patient with no preoperative syrinx or

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W. Jerry Oakes

The study in this issue by Sgouros and colleagues comes from a unit with a long and distinguished history of interest and work in the area of hindbrain hernias. The authors build on this background and provide the reader with evidence of a skull base that is wider and has a steeper angle with the clivus in patients with Chiari malformation Type I (CM-I) with syringomyelia than in children without this condition. They then go on and speculate that these skull base changes may indicate that the primary defect resulting in the CM-I is mesodermal in origin and

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R. Shane Tubbs and W. Jerry Oakes

compromised by lambdoidal synostosis. 8 Lapresle, et al., 14 have reported on a case of upper-left and lower-right muscular hypertrophy in a patient with syringomyelia and Arnold—Chiari malformation. The delineation of what type of Arnold—Chiari was not mentioned, however, although presumably it represents a Chiari I malformation because the patient's photographs depict a standing individual with good muscle bulk in the lower extremities (which would be unusual if this were a patient with the Chiari II malformation). Interestingly, Caldemeyer, et al., 4 report on a

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R. Shane Tubbs, Bermans J. Iskandar, Alfred A. Bartolucci and W. Jerry Oakes

, and a history of atlantooccipital fusion. The angulation of the odontoid process 20 ranged from 65 to 100° (mean 84.4 ± 9.3°[standard deviation]) ( Fig. 1 upper ). The distance of the obex beneath the foramen magnum ranged from 9 to 20 mm (mean 14.4 ± 3.96 mm) ( Fig. 1 center ). The anteroposterior width of the medulla oblongata (< 15 mm in all patients) resulted in a flattened appearance in the midsagittal region. 7 In 11 patients (50%) syringomyelia was demonstrated: six with a holocord syrinx, two with a cervical syrinx, one with a thoracic syrinx, and two

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R. Shane Tubbs, Matthew J. McGirt and W. Jerry Oakes

transoral odontoidectomy and occipital cervical fusion 8 days later. This patient recovered, although a left mild esotropia persisted. Unilateral tonsillar coagulation was performed in 22 patients (17%), eight of whom underwent repeated operation for continued syringomyelia. Nine patients have undergone repeated operations for continued symptoms or persistent large syringes; eight surgeries were performed for continued syringomyelia and one for continued headache that was not relieved following posterior fossa decompression without duraplasty. Indeed, the latter patient

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R. Shane Tubbs and W. Jerry Oakes

Object The authors sought to establish whether the volume of the posterior fossa in children suffering from Chiari malformation Type I (CM-I) is smaller than normal, as has been suggested previously. They also investigated the role of syringomyelia in posterior fossa development. Methods Both posterior fossa volume (PFV) and intracranial volume (ICV) were measured using segmentation techniques on preoperative magnetic resonance images obtained in 42 children who underwent surgery for CM-I (mean age 127 months, range 36–204 months); 25 (59%) of the