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Biomedical subjects

Leslie N Sutton

Publications and source records attributed to Leslie N Sutton.

29 records · Page 2Linked to original sources
PubMed2003

Fetal myelomeningocele repair: short-term clinical outcomes.

OBJECTIVE: The study was undertaken to evaluate short-term clinical outcomes with antenatal myelomeningocele (MMC) repair. STUDY DESIGN: Retrospective review of 50 fetuses that underwent open fetal MMC closure was performed. Inclusion criteria included less than 26 weeks' gestation, thoracic to S1 level defect, absent clubfoot deformity, Arnold-Chiari malformation, ventriculomegaly less than 17 mm, normal karyotype, and no other anomalies. RESULTS: Perinatal survival was 94% (47/50 fetuses). Mean age at delivery was 34 weeks 3 days. All fetuses demonstrated reversal of hindbrain herniation. Forty-three percent of the 50 fetuses have required ventriculoperitoneal shunting compared with 100% thoracic, 88% lumbar, and 68% sacral (85% overall) in 297 historic controls. Better-than-predicted leg function was seen in 57% of thoracic and lumbar level lesion patients. CONCLUSION: Early experience with fetal MMC repair suggests a decreased need for ventriculoperitoneal shunting, arrest or slowing of progressive ventriculomegaly, and consistent resolution of hindbrain herniation. However, further long-term follow-up is needed to evaluate neurodevelopment and bladder and bowel function.

Adult↗
PubMed2003

The effect of intrauterine myelomeningocele repair on the incidence of shunt-dependent hydrocephalus.

BACKGROUND: Intrauterine myelomeningocele repair (IUMR) was first successfully performed in 1997. Preliminary reports suggest that this procedure reduces the incidence of shunt-dependent hydrocephalus when compared to conventional postnatal therapy. However, the existing cohort of IUMR patients has not yet been systematically compared to a comparable group of conventionally treated controls. METHODS: Patients 1 year old or greater who had undergone IUMR at either Vanderbilt University or the Children's Hospital of Philadelphia (CHOP) were compared to a group of conventionally treated historical controls treated and followed at CHOP. In order to measure any differences between the groups, patients were stratified according to the level of the myelomeningocele lesion and the gestational age at the time of IUMR. RESULTS: One hundred and four IUMR patients were compared to 189 conventionally treated controls. IUMR resulted in statistically significant reductions in the incidence of shunt-dependent hydrocephalus at both lumbar and sacral lesion levels. When lumbar lesion levels were further stratified, from L1 to L5, it appeared that the benefit of IUMR was statistically significant only at levels below L2. Other factors with a significant impact on hydrocephalus were estimated gestational age and ventricular size at the time of surgery. In particular, statistically significant differences compared to controls were seen in the younger (< or =25 weeks) group but not in the older (>25 weeks) group. CONCLUSIONS: IUMR appears to substantially reduce the incidence of shunt-dependent hydrocephalus when compared to conventional treatment even when lesion level is taken into account. Patients with lesions above L3 may not share in this benefit. IUMR cannot be justified in fetuses older than 25 weeks of gestation. Additional improvements might be obtained by further reducing the average age at which fetuses are operated upon. It remains to be determined whether this benefit outweighs the potential risks of intrauterine surgery.

Cerebrospinal Fluid Shunts↗
PubMed2003

Ventriculosagittal sinus shunt placement: technical case report.

OBJECTIVE AND IMPORTANCE: Determining an appropriate site for distal catheter placement for ventricular shunting for some hydrocephalic patients can be difficult. We describe a simplification of the technique for sagittal sinus shunt placement using a guidewire. CLINICAL PRESENTATION: A 20-month-old infant with hydrocephalus secondary to Alexander's disease developed erosion of her parieto-occipital ventriculoperitoneal shunt reservoir through an occipital decubitus scalp ulceration. Her hydrocephalus was temporarily treated with a ventriculostomy; however, she developed pneumatosis intestinalis while in the hospital. TECHNIQUE: The patient underwent placement of a ventriculosagittal sinus shunt. The ventricular catheter and shunt valve were placed through a burr hole at Kocher's point, and the distal end of the catheter was placed in the superior sagittal sinus by using the Seldinger technique. CONCLUSION: Ventriculosagittal sinus shunting may be used as an alternative to traditional methods for patients for whom distal shunt placement is problematic. Our technique has the theoretical advantage of reducing the risks of blood loss or air embolism by not requiring a scalpel incision into the sinus.

Cerebral Ventricles↗
PubMed2002

Overexpression of cyclooxygenase-2 (COX-2) in human primitive neuroectodermal tumors: effect of celecoxib and rofecoxib.

In this study the role of cyclooxygenase-2 (COX-2) in primitive neuroectodermal tumor (PNET) the most malignant brain tumors of childhood was investigated. COX-2 expression in human brain tumor biopsy samples (seven/seven) was about 6-8-fold higher than normal brain tissue and several PNET cell lines also express COX-2. The effect of selective COX-2 inhibitors, celecoxib and rofecoxib on the growth of two PNET cell lines (DAOY and PFSK) was determined. Celecoxib was more potent than rofecoxib in suppressing cell growth. Growth inhibition by celecoxib and rofecoxib was independent of Bcl-2 expression. Celecoxib suppressed the expression of Akt and activated the caspase-3 in DAOY and PFSK, whereas rofecoxib did not have such an effect.

Antineoplastic Agents↗
PubMed2002

Prenatal diagnosis of terminal myelocystocele in the fetal surgery era: case report.

OBJECTIVE AND IMPORTANCE: We report a case of a child with terminal myelocystocele (TMC). This case exemplifies the importance of performing a multidisciplinary evaluation and magnetic resonance imaging in the patient with suspected meningomyelocele when fetal surgery is being considered. CLINICAL PRESENTATION: This patient was evaluated at the Center for Fetal Diagnosis and Treatment and considered for fetal surgery to repair meningomyelocele. TECHNIQUE: A follow-up based on both ultrasound and magnetic resonance imaging was performed throughout pregnancy. CONCLUSION: Although a definitive diagnosis of TMC was not established prenatally, the patient was not offered fetal surgery, based on multiple selection criteria. We correlate the pathophysiology of TMC with the radiographic and amniocentesis findings. TMC must be considered in the differential diagnosis in a fetus with a dysraphic defect.

Adult↗
PubMed2002

Delayed cranial vault reconstruction for sagittal synostosis in older children: an algorithm for tailoring the reconstructive approach to the craniofacial deformity.

An algorithm for the management of sagittal synostosis in older children who underwent delayed cranial vault reconstruction is presented. This algorithm tailors the surgical approach to the specific craniofacial deformity present in each case. The scaphocephalic deformity characteristic of sagittal synostosis varies significantly when presentation is delayed beyond the first year of life, the time during which reconstruction is usually performed. Sixteen patients with sagittal synostosis who presented after 12 months of age, and were a mean of 3.2 years of age at the time of cranial vault reconstruction, were reviewed. Four patients demonstrated preoperative symptoms and objective findings indicative of increased intracranial pressure, including frequent headaches and emesis, papilledema, or digital markings on computed tomographic scan. Each of the 16 patients underwent either (1) single-stage total vault reconstruction with or without concomitant fronto-orbital expansion; (2) two-stage total vault reconstruction with anterior two-thirds vault expansion followed by transverse occipital expansion and recession a mean of 8.7 months later; or (3) anterior two-thirds vault reconstruction with or without fronto-orbital expansion. In each case, the extent of the scaphocephalic deformity determined the procedure used. The presence of severe frontal bossing associated with transverse restriction of the orbitotemporal region was an indication for fronto-orbital expansion in addition to vault reconstruction, whereas significant occipital protrusion was an indication for transverse posterior vault expansion and recession in addition to anterior two-thirds vault reconstruction. Excellent aesthetic results were obtained in all cases regardless of the type of reconstruction performed. However, it is essential that the extent of the deformity be carefully evaluated preoperatively to permit selection of the appropriate technique for reconstruction.

Algorithms↗
PubMed2002

A new look at myelomeningoceles: functional level, vertebral level, shunting, and the implications for fetal intervention.

OBJECTIVE: Previous reports have suggested that 80% to 85% of patients who have a myelomeningocele (MMC) and undergo surgical repair after birth develop hydrocephalus and require the placement of a ventricular shunt. However, the rate of shunting as a function of spinal level is not well established. We sought to determine the distribution of postnatally repaired MMC lesions as characterized by both functional and radiologic assessment, as well as the incidence of shunting when patients were categorized according to these 2 methods. METHODS: A retrospective chart review of 297 patients who were born with open MMCs and followed in the spina bifida clinic at the Children's Hospital of Philadelphia was performed. The presence or absence of a shunt was determined for each patient. Functional spinal level was determined by the best-recorded neurologic examination and vertebral level by spine radiographs. RESULTS: The overall rate of ventricular shunting was 81%. The level of the lesion significantly affected the incidence of shunting, with more cephalad lesions correlating with higher rates. This was true both for functional and radiologic categorizations. A significantly higher shunt rate was found among patients with sacral lesions when categorized by radiologic rather than functional criteria. In 86% of patients, the functional level was found to be equal to or higher (worse) than the radiologic level. CONCLUSIONS: This study describes the natural history of ventricular shunting in MMC patients with relation to both radiologic and functional criteria. Fetal MMC closure is being performed in some centers in an attempt to decrease the incidence of shunting and to improve leg function in selected patients. The present data may serve as a comparison group and aid in the design and analysis of a prospective trial to assess the efficacy of this new procedure.

Cerebrospinal Fluid Shunts↗
PubMed2002

Conus medulla-cauda compression from nerve root hypertrophy in a child with Dejerine-Sottas syndrome: improvement with laminectomy and duraplasty. Case report.

This 7-year-old boy with Dejerine-Sottas syndrome caused by a mutation in the myelin protein zero gene began to suffer rapid deterioration with increasing leg weakness, loss of the ability to ambulate, and bowel and bladder incontinence. Magnetic resonance imaging of the spine revealed nerve root hypertrophy resulting in compression of the conus medullaris and cauda equina. Decompressive surgery was successful in reversing some of his deficits.

Amino Acid Substitution↗
PubMedDate not supplied

Spina bifida.

Spina bifida results from failure of fusion of the caudal neural tube, and is one of the most common malformations of human structure. The causes of this disorder are heterogeneous and include chromosome abnormalities, single gene disorders, and teratogenic exposures. However, the cause is not known in most cases. Up to 70% of spina bifida cases can be prevented by maternal, periconceptional folic acid supplementation. The mechanism underlying this protective effect is unknown, but it is likely to include genes that regulate folate transport and metabolism. Individuals with spina bifida need both surgical and medical management. Although surgical closure of the malformation is generally done in the neonatal period, a randomised clinical trial to assess in utero closure of spina bifida has been initiated in the USA. Medical management is a lifelong necessity for individuals with spina bifida, and should be provided by a multidisciplinary team.

Animals↗
PubMedDate not supplied

Treatment of pediatric intracranial arteriovenous malformations with linear-accelerator-based stereotactic radiosurgery: the University of Pennsylvania experience.

There are relatively few reports detailing the outcome of children and adolescents with arteriovenous malformations (AVMs) treated with stereotactic radiosurgery (SRS). We reviewed our experience over the past decade to determine whether the outcomes and toxicity were similar to those reported in adults. Seventeen patients 18 years of age or younger underwent linear-accelerator-based SRS. The targeted volume was greater than 3 cm(3) in 65% of cases (range 0.7-25 cm(3); median volume 6.9 cm(3)). The prescribed radiation doses varied from 16 to 18 Gy, with 70% receiving the highest dose. Using only angiographic data, the obliteration rate was 80% (8 of 10 patients), but using both MRA/MRI and angiographic data, it was 53% (9 of 17 patients). Four patients developed late effects potentially attributable to the radiation between 2 months and 3 years following SRS. One of these was transient and disappeared completely within a few days, but in the other 3 patients, the neurologic sequelae were permanent. Two of the 4 complications appeared to be due to radiation necrosis/edema, whereas the remaining 2 may have been due to vasculopathy. All 4 patients with complications had treatment volumes greater than 5 cm(3) (5.4, 6.9, 11.1, 16.4 cm(3)), all had a prescribed dose of 18 Gy and all had initially presented with an AVM hemorrhage. Linear-accelerator-based SRS is effective in obliterating most AVMs in children; however, the potential for late effects exists, especially for those patients with larger target volumes.

Adolescent↗