Friday, July 3, 2009

Lifestyle, participation, and health-related quality of life in adolescents and young adults with myelomeningocele.

Buffart LM, Berg-Emons RJ, Meeteren JV, Stam HJ, Roebroeck ME. (2009) Lifestyle, participation, and health-related quality of life in adolescents and young adults with myelomeningocele. Developmental Medicine and Child Neurology. 2009 Mar 20.

Department of Rehabilitation Medicine, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.

This study aimed to describe participation and health-related quality of life (HRQoL) in adolescents and young adults with myelomeningocele and to explore their relationships with lifestyle-related factors. Fifty-one individuals with a mean age of 21 years 1 month (SD 4y 6mo) years participated (26 males, 25 females; 82% hydrocephalus, 55% wheelchair-dependent). Participation was assessed using the Life Habits Questionnaire, and HRQoL was assessed using the Medical Outcomes Study 36-item Short-form Health Survey. Physical activity was measured using an accelerometry-based activity monitor, fitness (peak oxygen uptake) was measured during a maximal exercise test, and the sum of four skin-folds was assessed to indicate body fat. Relationships were studied using logistic regression analyses. Of the participants, 63% had difficulties in daily activities and 59% in social roles. Participants perceived lower physical HRQoL than a Dutch reference population. Participants with higher levels of physical activity and fitness had fewer difficulties in participating in daily activities (odds ratio [OR]=8.8, p=0.02 and OR=29.7, p=0.02 respectively) and a higher physical HRQoL (OR=4.8, p=0.02 and OR=30.2, p=0.006 respectively), but not mental HRQoL. Body fat was not related to participation or HRQoL. In conclusion, a large proportion of individuals with myelomeningocele had difficulties in participation and perceived low physical HRQoL. Higher levels of physical activity and fitness were related to fewer difficulties in participation and higher physical HRQoL.

PMID: 19416327

Incidence of symptomatic retethering after surgical management of pediatric tethered cord syndrome with or without duraplasty.

Samuels R, McGirt MJ, Attenello FJ, Garcés Ambrossi GL, Singh N, Solakoglu C, Weingart JD, Carson BS, Jallo GI. (2009) Incidence of symptomatic retethering after surgical management of pediatric tethered cord syndrome with or without duraplasty. Child's Nervous System. 2009 May 6.

Department of Neurosurgery, Johns Hopkins School of Medicine, 600 N Wolfe Street, Meyer 8-161, Baltimore, MD, 21287, USA.

BACKGROUND: Cord retethering and other postoperative complications can occur after the surgical untethering of a first-time symptomatic tethered cord. It is unclear if using duraplasty vs. primary dural closure in the initial operation is associated with decreased incidence of either immediate postoperative complications or subsequent symptomatic retethering. It is also unclear if different etiologies are associated with different outcomes after each method of closure. We reviewed our pediatric experience in first-time surgical untethering of symptomatic tethered cord syndrome (TCS) to identify the incidence of postoperative complications and symptomatic retethering after duraplasty vs. primary closure.

MATERIALS AND METHODS: We retrospectively reviewed 110 consecutive pediatric (<18 years old) cases of first-time symptomatic spinal cord untethering at our institution over a 10-year period. Incidence of postoperative complications and symptomatic retethering were compared in cases with duraplasty vs. primary dural closure use.

RESULTS: Mean age was 5.7 +/- 4.8 years old. "Complex" etiologies included lipomyelomeningocele or prior lipomyelomeningocele repair in 22 (20%) patients, prior myelomeningocele repair in 35 (32%), and concurrent lumbosacral lipoma in 18 (16%). "Noncomplex etiologies" included fatty filum in 26 (24%) and split cord malformation in five (4%). Seventy-five (68%) cases underwent primary dural closure vs. 35 (32%) with duraplasty. Twenty-nine (26%) patients experienced symptomatic retethering at a median [interquartile range (IQR)] of 30.5 [20.75-41.75] months postoperatively. There was no difference in incidence of postoperative cerebrospinal fluid leak, surgical site infection, or median [IQR] length of stay in patients receiving primary dural closure [4 (5%), 7 (9%), and 5 (4-6) days, respectively] vs. duraplasty [3 (9%), 3 (9%), and 6 [5-8] days, respectively], p > 0.05. Complex etiologies were more likely to retether than noncomplex etiologies after primary closure (33.6% vs. 6.6%, p = 0.05) but not after duraplasty (13.7% vs. 5.4%, p = 0.33). Duraplasty graft type (polytetrafluoroethylene vs. bovine pericardium) was not associated with pseudomeningocele or retethering.

CONCLUSION: In our experience, the increased rate of symptomatic retethering observed with complex pediatric TCS (pTCS) etiologies after primary dural closures was not observed when duraplasty was instituted. Expansile duraplasty may be valuable specifically in the management of patient subgroups with complex pTCS etiologies.

PMID: 19418057

Posterior vertebral column subtraction osteotomy: a novel surgical approach for the treatment of multiple recurrences of tethered cord syndrome.

Hsieh PC, Ondra SL, Grande AW, O'Shaughnessy BA, Bierbrauer K, Crone KR, Halpin RJ, Suk I, Koski TR, Gokaslan ZL, Kuntz C. (2009) Posterior vertebral column subtraction osteotomy: a novel surgical approach for the treatment of multiple recurrences of tethered cord syndrome. Journal of Neurosurgery: Spine. 2009 Apr;10(4):278-86.

Department of Neurological Surgery, Northwestern University, Feinberg School of Medicine, Chicago, Illinois, USA. phsieh@usc.edu

Recurrent tethered cord syndrome (TCS) has been reported to develop in 5-50% of patients following initial spinal cord detethering operations. Surgery for multiple recurrences of TCS can be difficult and is associated with significant complications. Using a cadaveric tethered spinal cord model, Grande and colleagues demonstrated that shortening of the vertebral column by performing a 15-25-mm thoracolumbar osteotomy significantly reduced spinal cord, lumbosacral nerve root, and terminal filum tension. Based on this cadaveric study, spinal column shortening by a thoracolumbar subtraction osteotomy may be a viable alternative treatment to traditional surgical detethering for multiple recurrences of TCS. In this article, the authors describe the use of posterior vertebral column subtraction osteotomy (PVCSO) for the treatment of 2 patients with multiple recurrences of TCS. Vertebral column resection osteotomy has been widely used in the surgical correction of fixed spinal deformity. The PVCSO is a novel surgical treatment for multiple recurrences of TCS. In such cases, PVCSO may allow surgeons to avoid neural injury by obviating the need for dissection through previously operated sites and may reduce complications related to CSF leakage. The novel use of PVCSO for recurrent TCS is discussed in this report, including surgical considerations and techniques in performing PVCSO.

PMID: 19441983

Neuropsychological assessment of attention in children with spina bifida.

Vinck A, Mullaart R, Rotteveel J, Maassen B. (2009) Neuropsychological assessment of attention in children with spina bifida. Cerebrospinal Fluid Research. 2009 May 28;6:6.

Department of Medical Psychology, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB Nijmegen, the Netherlands. a.vinck@mps.umcn.nl.

ABSTRACT: BACKGROUND: Children with the severe form of spina bifida (SBM: spina bifida with myelomeningocele with accompanying hydrocephalus) may manifest attention deficits, and have a similar psychological profile to children with hydrocephalus due to other etiologies. It is unclear to what extent tests to assess attention in SBM are confounded by the accompanying cognitive or visual-motor impairments. The aim of this study was to analyse attention functions by administering two different types of attention tests, one with high and the other with low cognitive and motor requirements. This enabled the possible interaction between attention and cognitive and motor impairment to be assessed.

METHODS: The study group comprised 31 children with SBM with shunted hydrocephalus. Twenty children with SB-only formed a closely matched comparison group. Of these, 19 children with SBM and 18 with SB had a full-scale IQ (FSIQ) higher than 70. All had undergone spinal surgery and all children with SBM had been shunted within the first months of life. Between 6 and 15 years of age, the children were assessed on focused and sustained attention, encoding, and distractibility/impulsivity, using both traditional tests and computerized attention tests.

RESULTS: Compared to the SB group, attention scores of children with SBM were lower on the traditional tests, but when interfering cognitive and visual-motor requirements were eliminated using the computerised tasks, most differences disappeared. Furthermore, in contrast to traditional attention tasks, computerized tests showed no significant correlations with IQ-scores and visual-motor skills.

CONCLUSION: Assessment of attention functions in children with SBM by traditional tests may be misleading, because this paediatric population with complex cerebral malformations has difficulty with the cognitive and visual-motor requirements. To control for these interactions, the use of both traditional and computerized attention tests is recommended.

PMID: 19476646

Shunt complications in children with myelomeningocele: effect of timing of shunt placement. Clinical article.

Radmanesh F, Nejat F, El Khashab M, Ghodsi SM, Ardebili HE. (2009) Shunt complications in children with myelomeningocele: effect of timing of shunt placement. Clinical article. Journal of Neurosurgery: Pediatrics. 2009 Jun;3(6):516-20.

Department of Neurosurgery, Children's Hospital Medical Center, and Department of Biostatistics, Tehran University of Medical Sciences, Tehran, Iran.

OBJECT There has been controversy over whether CSF shunt insertion simultaneously with repair of myelomeningocele (MMC) might increase shunt-related complications. The purpose of this study was to evaluate shunt complication rates in patients who underwent concurrent MMC surgery and shunt placement and compare them to the rates in patients treated with shunt placement in a separate procedure.

METHODS The authors retrospectively reviewed the outcome of shunt placement in 127 patients with MMC who were followed up for >or=1 year after shunt surgery. In 65 patients shunt surgery was performed in a second operation after MMC repair and in 46 the 2 procedures were performed concurrently. In 7 patients shunt placement was the initial surgery, and in 9 it was the only procedure performed. The patients were evaluated for shunt complications.

RESULTS There was no statistically significant between-groups difference in age at which patients underwent shunt placement. The overall rates of shunt infection and shunt malfunction were 16.5 and 39.4%, respectively. There was a high rate of shunt infection and mortality in those patients treated with CSF shunting only. There was no statistically significant difference between complication rates in patients in whom the 2 procedures were performed concurrently and those who underwent separate operations.

CONCLUSIONS The order in which myelomeningocele repair and shunt placement were carried out did not have a significant effect on the rate of shunt complications. Thus, when indicated these procedures can be performed concurrently with a level of risk comparable to that associated with delayed shunt placement.

PMID: 19485738

Early results of the Ponseti method for the treatment of clubfoot associated with myelomeningocele.

Gerlach DJ, Gurnett CA, Limpaphayom N, Alaee F, Zhang Z, Porter K, Kirchhofer M, Smyth MD, Dobbs MB. (2009) Early results of the Ponseti method for the treatment of clubfoot associated with myelomeningocele. Journal of Bone and Joint Surgery 2009 Jun;91(6):1350-9.

Department of Orthopaedic Surgery, Washington University School of Medicine, St. Louis Shriners Hospital for Children, St. Louis, MO 63110, USA.

BACKGROUND: Myelomeningocele is a common birth defect that is often accompanied by clubfoot deformity. Treatment of clubfoot associated with myelomeningocele traditionally has consisted of extensive soft-tissue release operations, which are associated with many complications. The purpose of the present study was to evaluate the early results of the Ponseti method for the treatment of clubfoot associated with myelomeningocele.

METHODS: Sixteen consecutive patients with myelomeningocele (twenty-eight clubfeet) and twenty consecutive patients with idiopathic clubfeet (thirty-five clubfeet) were followed prospectively while being managed with the Ponseti method. The average duration of follow-up was thirty-four months for the myelomeningocele group and thirty-seven months for the idiopathic group. Clubfoot severity was graded at the time of presentation with use of the Diméglio system. The initial correction that was achieved, casting and/or bracing difficulties, recurrences, and subsequent treatments were evaluated and compared between the two cohorts by means of appropriate statistical analysis.

RESULTS: Eleven (39%) of the twenty-eight clubfeet in the myelomeningocele group were graded as Diméglio grade IV, compared with only four (11%) of the thirty-five clubfeet in the idiopathic group (p = 0.014). Initial correction was achieved in thirty-five clubfeet (100%) in the idiopathic group and in twenty-seven clubfeet (96.4%) in the myelomeningocele group (p = 0.16). Relapse of deformity was detected in 68% of the feet in the myelomeningocele group, compared with 26% of the feet in the idiopathic group (p = 0.001). Relapses were treated successfully without the need for extensive soft-tissue release surgery for all but four of the clubfeet in the myelomeningocele group and for all but one of the clubfeet in the idiopathic group (p = 0.16).

CONCLUSIONS: Our data support the use of the Ponseti method for the initial treatment of clubfoot deformity associated with myelomeningocele, although attention to detail is crucial in order to avoid complications. Longer follow-up will be necessary to assess the risk of late recurrence and the potential need for more extensive clubfoot corrective surgery in this patient population.

PMID: 19487512

Grice arthrodesis in the treatment of valgus feet in children with myelomeningocele: a 12.8-year follow-up study.

Høiness PR, Kirkhus E. (2009) Grice arthrodesis in the treatment of valgus feet in children with myelomeningocele: a 12.8-year follow-up study. Journal of Children's Orthopedics. 2009 Jun 16. [Epub ahead of print]

Rikshospitalet, National Hospital, 0027, Oslo, Norway, per.reidar.hoiness@rikshospitalet.no.

PURPOSE: Neurological deficit resulting in the lack of motor control in children with myelomeningocele often leads to a valgus position of the feet and ankles, usually in combination with planovalgus and pronation of the forefoot. The purpose of the study was to evaluate long-term patient satisfaction and clinical effects in ambulating children with lumbosacral myelomeningocele after having performed a Grice arthrodesis of a valgus unstable foot. The clinically most relevant radiographic measurements, such as the frontal and lateral talo-calcaneal angles, were used to evaluate the anatomical effects of the surgery, whereas the main research question was to reveal the patient satisfaction and usefulness of the procedure.

METHODS: The modified Grice-Green extraarticular subtalar arthrodesis was performed by the same surgeon on one standing and 22 walking patients (12 female) with lumbosacral myelomeningocele and valgus instability during the period 1985-1999. Twelve patients had bilateral surgery, giving a total of 35 operated feet. The patients attended a thorough check-up at a mean of 12.8 years (standard deviation [SD] +/- 3.2, range 7.7-20.2 years) after surgery. The mean age at surgery was 6.6 years (SD +/- 1.8) and at follow-up 19.4 years (SD +/- 3.8). Functional parameters, such as walking ability, pain and skin problems, and the need for braces and supportive orthopaedic shoes were noted at the follow-up interview. The parents were interviewed along with the patients in order to obtain all of the necessary information. Loaded radiographs in the lateral and frontal planes were taken of both feet and ankles pre-operatively and at follow-up, except for pre-operative radiographs in six patients that were not loaded and, thus, not included, except for the assessment of ankle valgus. Ankle valgus was assessed from lateral and frontal views of the ankle on a scale from grade 0 to grade 3 according to Malhotra. Frontal and lateral talo-calcaneal angles were measured for the assessment of subtalar varus or valgus. Lateral talo-first-metatarsal (Meary's) angles were measured to investigate the longitudinal arches of the feet. RESULTS: The mean lateral talo-calcaneal angle was reduced significantly (P < 001) from 55.1 degrees (SD +/- 8.9) to 38.8 degrees (SD +/- 8.1). The mean frontal talo-calcaneal angle was reduced from 24.7 degrees (SD +/- 9.7) pre-operatively to 16.6 degrees (SD +/- 6.3) at follow-up (P < 0.001). The mean lateral talo-first-metatarsal angle improved significantly from -16.1 degrees (SD +/- 24.7) pre-operatively to 0.9 degrees (SD +/- 15.1) at follow-up (P = 0.0015). The calcaneal pitch did not change significantly. In general, ankle valgus worsened during follow-up time, but not significantly (P = 0.113). The visual analogue scale (VAS) score of patient satisfaction improved significantly from 3.7 (SD +/- 1.7) prior to surgery to 7.2 (SD +/- 1.5) at follow-up (P < 0.005). Nineteen patients (83%) were satisfied with the surgery and would thus recommend the procedure.

CONCLUSIONS: Based on the radiological findings and patient satisfaction, the patients participating in this study benefited from having had Grice arthrodeses performed on their valgus unstable feet. The results indicate good long-term correctional effect on valgus deformity after Grice arthrodesis, as the talo-calcaneal and talo-first-metatarsal angles improved significantly. A great majority of the patients were content with the surgery, and none claimed that any residual deformity was the cause for any reduced ability to ambulate.

PMID: 19533196