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Paul C. Francel, T. S. Park, Jeffrey L. Marsh and Bruce A. Kaufman

✓ Frontal plagiocephaly may arise from either synostotic or deformational forces. Deformational causes of frontal plagiocephaly can be distinguished from synostotic causes by differences seen on physical examination, which can then be confirmed by skull x-ray films and if necessary three-dimensional computerized tomography (CT). Unilateral coronal synostosis is the main synostotic cause of frontal plagiocephaly, although it has also been seen with fusion of the frontozygomatic suture. In several syndromes presenting with bilateral coronal synostosis, fusion of the frontosphenoidal and frontoethmoidal sutures is also present.

The authors report, for perhaps the first time, a case showing synostotic frontal plagiocephaly secondary to fusion of the frontosphenoidal suture alone. Although the phenotypic appearance is superficially similar to that seen in unilateral coronal synostosis, analysis of the cranial base shows markedly different effects: angulation of the anterior cranial base with respect to the posterior cranial base away from the synostotic side and angulation of the posterior cranial base with respect to the midpalatal suture also away from the synostotic side. In unilateral coronal synostosis, both angulations are toward the synostotic side. These effects on the cranial base alter its relationship to the cranial vault and the facial skeleton. Most important, frontal plagiocephaly secondary to fusion of the frontosphenoidal suture should not be overlooked as being deformational. Because this fusion is difficult or impossible to visualize by skull x-ray films, three dimensional CT must be obtained in cases that are not clearly identified as deformational plagiocephaly by physical examination.

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Elsa V. Arocho-Quinones, Amie Kolimas, Peter S. LaViolette, Bruce A. Kaufman, Andrew B. Foy, Marike Zwienenberg and Sean M. Lew


Split laminotomy is a technique for accessing the spinal canal from the posterior midline that minimizes muscle dissection and bone removal. Benefits of this approach in minimizing postoperative pain and muscle atrophy in the adult population have been reported, but pediatric data are limited. Herein, the authors evaluate the benefits of the split laminotomy technique in pediatric patients.


Data obtained in patients who underwent posterior spine surgery at Children’s Hospital of Wisconsin for an intradural midline pathology between April 2008 and June 2015 were reviewed retrospectively. Each patient was assigned to one of two groups, the split-laminotomy or conventional-laminotomy group. The primary outcomes assessed were mean daily pain score, total opioid use over a period of 72 hours after surgery, and the degree of paraspinal muscle atrophy and fat infiltration found on short-term (1–4 months) and long-term (1–4 years) follow-up spine MRI studies.


A total of 117 patients underwent lumbar-level surgery (83 conventional laminotomy, 34 split laminotomy), and 8 patients underwent thoracic-level surgery (4 in each group). No significant difference in the mean daily pain scores between groups was found. The daily opioid use was significantly lower in the split-laminotomy group on postoperative day 0 (POD0) and POD1 but not on POD2 (p = 0.01, 0.01, and 0.10, respectively). The total opioid use over the 72-hour postoperative period was significantly lower in the split-laminotomy group (p = 0.0008). The fat/muscle ratio was significantly higher in both the short-term and long-term follow-up periods in the conventional-laminotomy group (p = 0.01 and 0.0002, respectively). The rate of change of paraspinal muscle fat infiltration was significantly lower in the split-laminotomy group than in the conventional-laminotomy group (p = 0.007). The incidence of complications was not significantly different between groups (p = 0.08).


This study was of the largest series reported thus far of pediatric patients who underwent split laminotomy and the only controlled study that has involved children. The authors’ results reinforce the short-term benefit of split laminotomy in minimizing acute postoperative pain and long-term benefits of decreasing muscle atrophy and fatty degeneration, which are known to be associated with the development of chronic pain and spinal instability. Additional efforts for assessing long-term effects in the development of chronic pain, spinal instability, and spinal deformity are still necessary.

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Jeffrey R. Leonard, Dan X. Cai, Dennis J. Rivet, Bruce A. Kaufman, T. S. Park, Beth K. Levy and Arie Perry

Object. Medulloblastoma is the most common malignant central nervous system neoplasm found in children. A distinct variant designated large cell/anaplastic (LC/A) medulloblastoma is characterized by frequent dissemination of cerebrospinal fluid (CSF) at presentation and a more aggressive clinical course. The authors report on their examination of the clinicopathological and genetic features of seven such cases encountered at their institution.

Methods. Eighty cases of medulloblastomas were reviewed and seven (8.8%) of these were believed to fit the histological and immunohistochemical criteria for LC/A medulloblastoma. In three cases (43%) either desmoplastic or classic medulloblastoma was the underlying subtype, and in two cases (28%) the LC/A tumor was found within the setting of medullomyoblastoma. Fluorescence in situ hybridization was used in six of the seven cases to characterize the presence of isochromosome 17q, deletion of chromosome 22q (a deletion characteristically found in atypical teratoid/rhabdoid tumors), and c-myc amplification. The patients' clinical histories revealed CSF dissemination in all cases and lymph node metastasis in one case. Isochromosome 17q was found in five (83%) of six cases. Evidence of chromosomal gains indicated aneuploidy in three tumors (50%), and amplification of c-myc was found in three tumors (50%). No 22q deletions were encountered.

Conclusions. A high percentage of LC/A medulloblastomas arise within a background of typical medulloblastomas or medullomyoblastomas. As is the case in conventional medulloblastomas, the presence of 17q is a common early tumorigenic event; however, in a significant percentage of specimens there is also evidence of aneuploidy and/or amplification of c-myc. These findings indicate that LC/A morphological characteristics reflect a more advanced tumor stage than that found in pure medulloblastomas or in typical medullomyoblastomas.

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Robert C. Heim, T. S. Park, George P. Vogler, Bruce A. Kaufman, Michael J. Noetzel and Madeleine R. Ortman

✓ Selective dorsal rhizotomy is increasingly used for management of spastic quadriplegic cerebral palsy but rates of hip stability following the operation have not been reported. Determining hip stability by radiographic measurement of lateral migration of the femoral head beyond a lateral edge of the acetabulum after dorsal rhizotomy allows an objective assessment of the outcome of the operation. This prospective study examined the effect of selective dorsal rhizotomy on lateral migration of the femoral head in 45 children with spastic quadriplegic cerebral palsy. The children ranged in age from 2 to 9 years (average 5 years 1 month) and were grouped according to their ages with 23 children in the 2- to 4-year-old group and 22 children in the 5- to 9-year-old group. Postoperative follow up ranged from 7 to 50 months (average 20 months). The Reimers migration percentage (MP), a measure of the lateral migration of the femoral head, was calculated from anteroposterior hip radiographs taken prior to the operation and at the last follow-up examination. Of the 90 hips involved, 9% improved, 80% remained unchanged, and 11% worsened, yielding a radiographic stability rate of 89%. The hips with postrhizotomy worsening of the MP had an average preoperative MP of 14% (range 9% to 38%) and an average postoperative increase in MP of 18% (range 11% to 37%). Of the 45 children, four subsequently underwent unilateral derotational femoral osteotomies for persistent or worsening hip subluxation. There was a significant tendency for the MP to worsen in patients with lower prerhizotomy MP values (χ2 = 20.74, df = 4, p = 0.001), but the age of patients and their ambulatory status at the time of rhizotomy had no bearing on postoperative hip stability. The data indicate that selective dorsal rhizotomy prevents progressive lateral migration of the femoral head in the majority of children who undergo the operation for spastic quadriplegia.

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Surya Sri Krishna Gour, Mohit Agrawal and Sachin A. Borkar

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Paul C. Francel, Myles Koby, T. S. Park, Benjamin C. P. Lee, Michael J. Noetzel, Susan E. Mackinnon, Martin M. Henegar and Bruce A. Kaufman

✓ Neurosurgical management of birth-related brachial plexus palsy involves observing the patient for a period of several months. Operative intervention is usually undertaken at 3 to 6 months of age or more in infants who have shown little or no improvement in affected muscle groups. Ancillary tests such as electromyography and nerve conduction studies are occasionally useful. No radiological study has been consistently helpful in operative planning, except for contrast computerized tomography (CT) myelography, which requires general anesthesia in infants. This is because the infant's small size exceeds the functional resolution of the imaging modalities.

This report describes the use of a special sequence of magnetic resonance (MR) imaging entitled “fast spin echo” (FSE-MR). Unlike CT myelography, this technique provides high-speed noninvasive imaging that allows clinicians to evaluate preganglionic nerve root injuries without the use of general anesthesia and lumbar puncture. The utility of this technique is illustrated in three cases, two involving either infraclavicular exploration or a combination of infraclavicular and supraclavicular exposure based on FSE-MR findings. The FSE-MR imaging offers an excellent alternative to contrast CT myelography in evaluation of infants with birth-related brachial plexus injuries.

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Peter W. Carmel, A. Leland Albright, P. David Adelson, Alexa Canady, Peter Black, William Boydston, David Kneirim, Bruce Kaufman, Marion Walker, Mark Luciano, Ian F. Pollack, Kim Manwaring, M. Peter Heilbrun, I. Richmond Abbott and Harold Rekate

Shunt systems with differential pressure valves are prone to the complications of overdrainage. A programmable valve permits adjustment of the opening pressure of the valve. In this paper the authors report the incidence of subdural fluid collections in a randomized trial of programmable compared with conventional valves, and they describe methodologies used in management of this complication.

A multiinstitutional, prospective, randomized trial of the Codman Hakim programmable valve and conventional fixed-pressure valves was undertaken. Two classes were defined: “new” and “replacement” valves. Randomization of the type of valve in each group was performed at each study site. Clinical and radiological studies were required at fixed intervals over a 104-week period. All complications were reported. The experimental valves were required to be reprogrammed after magnetic resonance imaging studies, but all other decisions regarding pressure setting were left to each investigator.

Three hundred seventy-seven patients were randomized; 194 were treated with a programmable valve and 183 with a fixed-pressure valve. The two groups were statistically similar in demographic composition, as were the “new” and “replacement” categories. The investigators made 540 valve pressure changes (five per patient; range one-41 changes). More than half of the reprogramming adjustments were made in the first 3 months postplacement; 70% were made within 6 months. More than half of all reprogramming adjustments were required in a group of 30 patients.

Four treatment modalities were observed: 1) 30% of the fluid collections resolved spontaneously (25% in the patients with programmable valves and 36.3% in those with conventional valves) and were largely found to be hygromas in infants and children; 2) four subdural fluid collections were unresolved and under observation; 3) the subdural hematoma was drained and the shunt removed (in 8.3% of patients with the programmable valve and 36.3% of those with the control valve); 4) the pressure of programmable valve was raised in 58% of patients (seven of 12), and this increase in opening pressure was a feature used by investigators to affect treatment.

There was no significant difference in the incidence of subdural fluid collections between the programmable and fixed-pressure valve treatment groups. The programmable feature provided a considerable advantage in treatment when subdural collections occurred.