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Biomedical subjects

A Leland Albright

Publications and source records attributed to A Leland Albright.

At least 19 recordsLinked to original sources
PubMed2006

Intrathecal baclofen therapy in children.

Since its introduction in the late 1980s, intrathecal baclofen (ITB) therapy has become the standard treatment for severe generalized spasticity and dystonia in children. Treatment with ITB decreases spasticity in the upper and lower extremities and has been associated with improved function and decreased musculoskeletal contractures. In addition, ITB decreases generalized secondary dystonia and has been associated with improved comfort and ease of care in approximately 85% and with improved function in approximately 33% of patients. Continued effectiveness of ITB in treating spasticity has been observed for up to 17 years, and its effectiveness in treating dystonia has been observed for up to 10 years. Although ITB therapy is frequently associated with complications such as infections, catheter malfunctions, and cerebrospinal fluid leaks, the benefits of therapy appear to outweigh the risks. Additional investigation is needed to determine the effects of ITB on other movement disorders such as athetosis and chorea.

Baclofen↗
PubMed2006

Case report of optic atrophy in pansynostosis: an unusual presentation of scalp edema from hair braiding.

Pansynostosis (fusion of all cranial sutures) and optic atrophy were found as incidental CT scan and ophthalmological findings in an 8-year-old who presented to the emergency room with scalp edema from tight 'cornrow' hair braiding. Cranial vault expansion was successfully performed. Ophthalmological problems have stabilized but have not reversed. Late presentation of craniosynostosis and the pathophysiology of secondary optic atrophy are discussed.

Child↗
PubMed2006

"Like mother, like son:" hereditary anterior sacral meningocele. Case report and review of the literature.

Anterior sacral meningocele (ASM) is a relatively rare, congenital disorder. Usually it presents sporadically, but there are case reports of hereditary ASMs and evidence of a dominant mode of inheritance. In this article the authors describe a case illustrating the hereditary nature of ASM and present available literature on the disease. The authors present the case of a 19-month-old boy in whom an ASM was diagnosed during a workup for constipation. The child's 31-year-old mother had been treated for the same condition 20 years earlier, when she had presented with back pain. These cases are discussed in the context of previous reports of similar cases. There are several case reports in the literature in which an ASM occurred as a familial, isolated disorder (in the absence of other caudal abnormalities or syndromes). The condition is reported more commonly in women, but it is unclear whether this is a true difference in prevalence or a diagnosis or reporting bias. A review of the literature indicates an autosomal-dominant inheritance with variable penetrance and presentation. Anterior sacral meningoceles can be hereditary. Given the potential complications of the disease if left untreated and the simplicity of screening--obtaining an abdominal radiograph and the patient's clinical history--we recommend screening of immediate family members of affected individuals. Surgical treatment is recommended if an ASM is discovered.

Adult↗
PubMed2006

Best-practice surgical techniques for intrathecal baclofen therapy.

In March 2004, a multidisciplinary conference, "ITB Therapy Best Practice Forum," was held in Minneapolis, Minnesota. The goal of the conference was to develop recommendations for techniques to implant intrathecal baclofen (ITB) pump and catheter systems more effectively and with fewer complications. The authors present the techniques for optimal pump and catheter implantation, including subfascial pump placement; insertion of the Tuohy needle in an oblique, paramedian trajectory; and positioning of the catheter tip at levels commensurate with the therapeutic indication: approximately T10-12 for spastic diplegia, C5-T2 for spastic tetraparesis, and C1-4 for generalized secondary dystonia. Techniques to minimize the incidence of cerebrospinal fluid leakage are described, including the identification of preoperative occult hydrocephalus and the use of a suture ligature around the Tuohy needle at its exit site from the fascia. Techniques to minimize surgery-related infection are also detailed; most involve the use of iodine solutions multiple times intraoperatively. Techniques to insert intrathecal catheters during spinal fusion are addressed, particularly the technique of inserting the catheter cephalad to the fusion site. Panel members advocate the aforementioned techniques to improve the efficacy of and decrease the morbidity associated with ITB therapy.

Baclofen↗
PubMed2006

Intraventricular baclofen infusion for dystonia. Report of two cases.

Two children with generalized dystonia were given continuous intraventricular baclofen (IVB) infusions. The first was a 12-year-old girl with mixed dystonia and spasticity caused by methylmalonic aciduria whose spinal anatomy precluded administration of intrathecal baclofen (ITB). The second was an 11-year-old boy whose dystonia was associated with cerebral palsy and had not improved significantly in response to conventional ITB treatment. The girl improved dramatically with IVB; the boy experienced no greater improvement from IVB than from ITB. No adverse effects were noted in either child during 2 to 6 months of infusion. These are the first reports of the use of IVB in this clinical setting. The results warrant additional investigation.

Baclofen↗
PubMed2005

Individualized treatment of pediatric craniopharyngiomas.

OBJECTIVE: The treatment of children with craniopharyngiomas should be individualized because of their heterogeneous clinical and radiographic characteristics. We performed this study to correlate the clinical and radiographic features at the time of presentation with the multimodality treatments the children received. METHODS: Medical records were reviewed for children with craniopharyngiomas who presented to the Children's Hospital of Pittsburgh for their initial management between 1983 and 2004. Children were treated with microsurgical tumor resections (27), intracavitary irradiation with phosphorus 32 (32P) (12), and with gamma knife stereotactic radiosurgery (GKSR) (5). CONCLUSIONS: There were no deaths in any treatment group. Gross total resections were thought to be performed in 18 patients and were confirmed by imaging in 13 of the 18 patients. The primary operative morbidities were hormonal and visual. Every child needed at least two replacement hormones and most had panhypopituitarism. Vision worsened postoperatively in 5 of 27 children. There was no operative morbidity or mortality from 32P. After 32P treatment, one child required a later cyst aspiration and one required a craniotomy for progressive cyst enlargement. There was no morbidity or mortality from GKSR, which achieved tumor stabilization or shrinkage in 4 of 5 cases. Resections, 32P, and GKSR are complimentary treatment modalities for children with craniopharyngiomas. Their indications and outcomes differ, but all should be available in the treatment armamentarium.

Adolescent↗
PubMed2005

Occult hydrocephalus in children with cerebral palsy.

OBJECTIVE: Many children with cerebral palsy have chronic ventriculomegaly as a consequence of perinatal intraventricular hemorrhage or periventricular leukomalacia, without symptoms of hydrocephalus. Children with cerebral palsy who are treated with intrathecal baclofen have a higher rate of cerebrospinal fluid (CSF) leaks along the baclofen catheter than do adults treated with intrathecal baclofen. We postulated that the cause of the increased frequency of CSF leaks was increased CSF pressure, that is, occult hydrocephalus. METHODS: Lumbar punctures were performed in 24 children with cerebral palsy and asymptomatic ventriculomegaly. Their median age was 4.7 years. Mild or moderate ventriculomegaly was present in 23 children and severe ventriculomegaly was present in 1 child. RESULTS: Opening pressures were abnormally high in 23 (96%) of 24 children and ranged from 22 to 41 cm H2O (mean, 27.3 cm H2O). Opening pressures did not correlate with the extent of ventriculomegaly. CONCLUSION: Children with cerebral palsy and ventriculomegaly seem to have a high incidence of increased CSF pressure, and thus, of occult hydrocephalus. The increased pressure is probably a significant cause of the increased frequency of CSF leaks for these children during intrathecal baclofen therapy. The long-term risks of untreated increased CSF pressures in this patient population are not known but are cause for concern. Treatment with CSF shunts offers the potential of improved development, which was reported anecdotally in some children who were treated with ventriculoperitoneal shunts after their pressure was found to be increased. Prospective multicenter studies of this problem are needed.

Cerebral Palsy↗
PubMed2005

Endovascular management of ventricular catheter-induced anterior cerebral artery false aneurysm: technical case report.

OBJECTIVE AND IMPORTANCE: We describe the case of a 3.3-kg infant who developed an anterior cerebral artery pseudoaneurysm after ventricular catheter placement. The lesion was treated with endovascular vessel sacrifice. CLINICAL PRESENTATION: A 3.3-kg infant with a myelomeningocele underwent repair and ventricular catheter insertion for hydrocephalus. During shunt insertion, intracerebral arterial bleeding was encountered. Subsequent arteriography demonstrated an anterior cerebral artery pseudoaneurysm. TECHNIQUE: Via a transfemoral artery approach, the aneurysm and feeding vessel were catheterized with a microcatheter and wire. The aneurysm and vessel were then sacrificed with detachable platinum coils. CONCLUSION: Arterial injury is uncommon after ventricular catheter placement. When it does occur, however, an unstable pseudoaneurysm may form. Endovascular techniques can be used to remedy this problem, thus at times obviating the need for a standard craniotomy, which can be particularly difficult in a newborn.

Angiography↗
PubMed2004

Intrathecal baclofen withdrawal: emergent recognition and management.

Patients with severe spasticity are frequently encountered in the pediatric emergency department. Intrathecal baclofen therapy is being used increasingly to treat these patients. The purpose of this case series is to illustrate the importance of early recognition and treatment of intrathecal baclofen withdrawal, a potentially life-threatening complication of intrathecal baclofen therapy. Four cases of intrathecal baclofen withdrawal are presented.

Adolescent↗
PubMed2004

Feasibility and advisability of resections of thalamic tumors in pediatric patients.

OBJECT: The author conducted a retrospective review of 19 cases in which he resected thalamic tumors between 1986 and 2001. METHODS: The median age of the children was 8 years. Five different routes were used to resect the tumors; two tumors were resected via two approaches. The extent of resection was evaluated by postoperative imaging. Gross-total resections were performed in six cases and resections of greater than 90% of the tumor were conducted in 10 others; 90% or more of the tumor was resected in 84% of the cases. Seven tumors were low-grade gliomas and 12 were high grade. There was one postoperative death, and two children suffered permanent morbidity. Five of seven children with low-grade tumors are alive 2 to 12 years postoperatively; three of 11 children with high-grade tumors are alive at 2, 3, and 16 years, respectively. CONCLUSIONS: Treatment of pediatric thalamic tumors must be individualized, with consideration given to the tumor's location, enhancement, and margins as well as the published data about the correlation between extent of resection and prognosis.

Adolescent↗
PubMed2004

Performance and complications associated with the synchromed 10-ml infusion pump for intrathecal baclofen administration in children.

OBJECT: The objectives of this multicenter study were to monitor the performance of a 10-ml pump infusing intrathecal baclofen to treat 100 children with cerebral spasticity, to monitor complications associated with the pump, and to correlate pump-related complications with body habitus. METHODS: Age at implantation of the pump ranged from 1.4 to 16.8 years (mean 8.1 years). The effects of ITB on spasticity in the upper and lower extremities were evaluated using the Ashworth Scale. Data were collected regarding implant site, infection, complication, and body mass index (BMI). Ashworth Scale scores decreased significantly in the upper and lower extremities at 6 and 12 months after pump implantation (p < 0.001). There were four serious system-related complications, all specific to catheters. There were 32 serious procedure-related complications in 21 patients: 11 complications were infections that occurred in nine patients. Four of nine pump-induced infections were treated with pump removal and antibiotic therapy; five infections were treated successfully with antibiotic therapy alone, without pump removal. In children younger than 8 years of age there was a significantly higher incidence of serious procedure-related adverse events than in older children. There was no significant correlation between BMI and the incidence of pump pocket-related complications or infections. CONCLUSIONS: The 10-ml pump can be used therapeutically in small children, particularly those weighing less than 40 lbs, with greater ease and less wound tension, than the conventional 18-ml pump. The incidence of complications associated with the 10-ml pump in younger children appears to be similar to that previously reported with the 18-ml pump in larger-sized children.

Adolescent↗
PubMed2003

Movement disorders in children: definitions, classifications, and grading systems.

Disorders that affect movement in children are relatively common. However, they have received little attention, especially when compared with epilepsy and neuromuscular disorders. In this review article, we address the major types of movement disorders that affect children, their clinical characteristics and etiologies, and, when available, the scales used to grade them. A discussion on spasticity, which traditionally is not addressed in reviews of "movement disorders," is also included.

Ataxia↗
PubMed2003

Neurosurgical treatment of spasticity and other pediatric movement disorders.

For children whose spasticity and movement disorders are inadequately treated by oral medications and botulinum toxins, neurosurgical procedures are now available to effectively treat spasticity, tremor, and many cases of dystonia. Spastic diplegia can be treated with selective lumbar rhizotomies, which significantly decrease spasticity, increase range of motion, and improve Gross Motor Function Measure scores. Children with spastic quadriparesis and those with secondary dystonia can be treated with intrathecal baclofen, which diminishes both spasticity and dystonia and is associated with improved function and quality of life. Children with primary dystonia and those with tremor can be treated with deep brain stimulation of the internal globus pallidus and thalamus, respectively. Some children with chorea respond to deep brain stimulation. There are no effective neurosurgical treatments for athetosis or ataxia. The effectiveness of neurosurgical treatments of pediatric movement disorders has increased significantly in the past 15 years.

Athetosis↗
PubMed2003

Long-term intrathecal baclofen therapy for severe spasticity of cerebral origin.

OBJECT: The goal of this study was to ascertain the long-term effectiveness and safety of intrathecal baclofen (ITB) in the treatment of spasticity of cerebral origin in children and young adults. METHODS: A prospective, multicenter study was conducted in 68 patients who had been enrolled in the initial evaluation of ITB therapy and were willing to participate in long-term surveillance. Seventy-three percent of the patients were younger than 16 years of age at the time of study entry. The patients were examined at least every 3 months and were observed for an average of 70 months. At each follow-up visit, spasticity in the upper and lower extremities was evaluated by applying Ashworth scores. All adverse events and complications were recorded on standardized data forms. Spasticity in both upper and lower extremities decreased significantly (p < 0.005) and remained decreased up to 10 years. The dosage of ITB increased from a mean of 157 microg/day 3 months after pump insertion to 300 microg/day at 2 years postimplantation, and remained relatively stable thereafter. There were no significant differences in ITB dosage in children of different ages. Adverse events potentially related to ITB therapy occurred in 50% of patients within 2 months after pump insertion and in 50% of patients thereafter; hypotonia and lethargy were the two most common adverse events. The most common complications of surgery were catheter-related problems (31%), seromas (24%), and cerebrospinal fluid leaks (15%). CONCLUSIONS: Intrathecal baclofen provides effective long-term treatment of spasticity of cerebral origin and its effects do not appear to diminish with time. This therapy is frequently associated with adverse side effects that usually can be alleviated by adjustments in dosage.

Adolescent↗
PubMed2002

Operative nuances of myelomeningocele closure.

ALTHOUGH ADVANCES IN prenatal care and diagnosis have reduced the incidence of spina bifida, repair of neural tube defects remains one of the standard cases encountered by pediatric neurosurgeons. The operative techniques used in closure of these congenital defects have remained essentially unchanged during the past 2 decades; however, the operative nuances for closure often are based on the variability in configuration and size of the open defects, which can present a challenge. Repair of the open spinal dysraphism may require only a simple closure, but extensive dissection and reconstruction of the neural, meningeal, fascial, and epidermal tissues, including the use of specialized vascularized flaps, may be required in some cases. In this article, we briefly describe the preoperative evaluation, decision process, and standard techniques for the repair of myelomeningocele and myeloschisis.

Dermatologic Surgical Procedures↗