Quality of Life and Bladder Symptoms in Adolescents and Young Adults With Spina Bifida Who Catheterize via Urethra vs Catheterizable Channel.

PURPOSE: We sought to assess associations between health-related quality of life (QOL), bladder-related QOL, bladder symptoms, and bladder catheterization route among adolescents and young adults with spina bifida. MATERIALS AND METHODS: Clinical questionnaires administered to individuals ≥ 12 years old requiring catheterization between June 2019 to March 2020 in a spina bifida center were retrospectively analyzed. Questionnaires were completed in English or Spanish independently or with caregiver assistance. Medical records were reviewed for demographic and clinical characteristics. Primary exposure was catheterization route (urethra or channel). Primary outcome was health-related QOL, measured by Patient-Reported Outcomes Measurement Information System Pediatric Global Health 7 (PGH-7). Secondary outcomes were bladder-related QOL and bladder symptoms, measured by Neurogenic Bladder Symptom Score (NBSS). Nested, multivariable linear regression models assessed associations between catheterization route and questionnaire scores. RESULTS: Of 162 patients requiring catheterization, 146 completed both the PGH-7 and NBSS and were included. Seventy-three percent were catheterized via urethra and 27% via channel. Median age was 17.5 years (range 12-31), 58% of patients were female, and 80% had myelomeningocele. Urinary incontinence was more common among those who catheterized via urethra (60%) compared to channel (33%). On adjusted analyses, catheterization route was not significantly associated with PGH-7 or NBSS bladder-related QOL scores. More bladder symptoms were associated with worse bladder-related QOL. Patients who catheterized via channel had fewer bladder symptoms than those who catheterized via urethra. CONCLUSIONS: Catheterization route was not significantly associated with QOL. Though catheterization via channel was associated with fewer bladder symptoms, only degree of current bladder symptoms was significantly associated with bladder-related QOL.

Association Between Quality of Life and Neurogenic Bowel Symptoms by Bowel Management Program in Spina Bifida.

OBJECTIVE: To compare differences in bowel-specific quality of life (QOL), overall qQOL, and neurogenic bowel dysfunction (NBD) severity by bowel management program in patients with spina bifida (SB). METHODS: We performed a retrospective cross-sectional study of patients ≥12 years old at our multidisciplinary SB center who completed both a modified Peristeen NBD questionnaire (assessing bowel symptom severity and bowel-specific QOL) and the Patient-Reported Outcomes Measurement Information System Pediatric Global Health questionnaire (assessing overall QOL). Nested, multivariable models were fit for associations between outcomes and bowel management program (enemas, conservative management, and none). RESULTS: A total of 173 patients, 56.1% female and 64.6% with myelomeningocele, were included in our analysis. Median age was 18.2 years old. Patients reported using enemas (n = 42), conservative management (n = 63), and no bowel program (n = 68). When adjusting for covariates, there was no significant association between bowel-specific QOL nor overall QOL across bowel management programs. However, the use of conservative management compared to enemas was associated with worse bowel symptoms severity (adjusted beta=2.58, 95%CI=[0.09,5.06]). Additionally, greater bowel symptom severity was significantly associated with lower overall QOL (adjusted beta=-0.33, 95%CI=[-0.57,-0.10]). CONCLUSION: NBD symptom severity in SB is more strongly associated with QOL than the individual bowel program being utilized. Our findings suggest that different degrees of NBD require different invasiveness of bowel programs, but it is the outcome of the bowel management program and not the specific program itself that is most associated with QOL.

Resident muscle stem cell myogenic characteristics in postnatal muscle growth impairments in children with cerebral palsy.

Children with cerebral palsy (CP), a perinatal brain alteration, have impaired postnatal muscle growth, with some muscles developing contractures. Functionally, children are either able to walk or primarily use wheelchairs. Satellite cells are muscle stem cells (MuSCs) required for postnatal development and source of myonuclei. Only MuSC abundance has been previously reported in contractured muscles, with myogenic characteristics assessed only in vitro. We investigated whether MuSC myogenic, myonuclear, and myofiber characteristics in situ differ between contractured and noncontractured muscles, across functional levels, and compared with typically developing (TD) children with musculoskeletal injury. Open muscle biopsies were obtained from 36 children (30 CP, 6 TD) during surgery; contracture correction for adductors or gastrocnemius, or from vastus lateralis [bony surgery in CP, anterior cruciate ligament (ACL) repair in TD]. Muscle cross sections were immunohistochemically labeled for MuSC abundance, activation, proliferation, nuclei, myofiber borders, type-1 fibers, and collagen content in serial sections. Although MuSC abundance was greater in contractured muscles, primarily in type-1 fibers, their myogenic characteristics (activation, proliferation) were lower compared with noncontractured muscles. Overall, MuSC abundance, activation, and proliferation appear to be associated with collagen content. Myonuclear number was similar between all muscles, but only in contractured muscles were there associations between myonuclear number, MuSC abundance, and fiber cross-sectional area. Puzzlingly, MuSC characteristics were similar between ambulatory and nonambulatory children. Noncontractured muscles in children with CP had a lower MuSC abundance compared with TD-ACL injured children, but similar myogenic characteristics. Contractured muscles may have an intrinsic deficiency in developmental progression for postnatal MuSC pool establishment, needed for lifelong efficient growth and repair.

Cerebral Palsy: Hip Surveillance.

Patients with cerebral palsy are known to be at risk for hip displacement and dislocation. Progressive hip displacement is known to cause a variety of problems including pain, impaired sitting balance, difficulty with perineal care, and decreased quality of life. To avoid these problems, hip reconstruction may be recommended and has been shown to lead to significant improvements in pain and health-related quality of life. To properly identify patients in need of intervention for hip displacement, hip surveillance is an active program consisting of clinical and radiographic monitoring in patients with cerebral palsy that allows for early detection of hip displacement in those at risk. As such, all children with cerebral palsy should be referred for hip surveillance at age 2 years. A growing body of literature has shown that hip surveillance along with appropriate orthopedic management decreases or prevents the incidence of hip dislocations, with direct implications on overall quality of life, in children with cerebral palsy. [Pediatr Ann. 2022;51(9):e353-e356.].

The Association of Health Literacy with Health-Related Quality of Life in Youth and Young Adults with Spina Bifida: A Cross-Sectional Study.

OBJECTIVE: The objective of the study was to determine if health literacy is associated with health-related quality of life (HRQOL) in adolescents and young adults (AYAs) with spina bifida. STUDY DESIGN: Between June 2019 and March 2020, the Patient-Reported Outcome Measurement Information System Pediatric Global Health-7 (PGH-7), a measure of HRQOL, and the Brief Health Literacy Screening Tool (BRIEF) were administered to patients ≥12 years old with a diagnosis of spina bifida seen in our multidisciplinary spina bifida center. Questionnaires were completed at scheduled clinic visits. The primary outcome was the PGH-7 normalized T-score. The primary exposure was the BRIEF score. Demographic and clinical characteristics were obtained from the medical record. Nested, multivariable linear regression models assessed the association between health literacy and the PGH-7 score. RESULTS: Of 232 eligible patients who presented to clinic, 226 (97.4%) met inclusion criteria for this study. The median age was 17.0 years (range: 12-31). Most individuals were female (54.0%) and had myelomeningocele (61.5%). Inadequate, marginal, and adequate health literacy levels were reported by 35.0%, 28.3%, and 36.7% of individuals. In univariable analysis, higher health literacy levels were associated with higher PGH-7 scores. In nested, sequentially adjusted multivariable linear regression models, a higher health literacy level was associated with a stepwise increase in the PGH-7 score. In the fully adjusted model, adequate health literacy and marginal health literacy, compared with inadequate health literacy, were associated with increases in a PGH-7 score of 3.3 (95% CI: 0.2-6.3) and 1.1 (95% CI: -2.0 to 4.2), respectively. CONCLUSIONS: Health literacy was associated with HRQOL after adjusting for demographic and clinical factors. Strategies incorporating health literacy are needed to improve HRQOL in AYAs with spina bifida.

Multidisciplinary spina bifida clinic: the Chicago experience.

Open spina bifida (open SB) is the most complex congenital abnormality of the central nervous system compatible with long-term survival. Multidisciplinary care is required to address the effect of this disease on the neurological, musculoskeletal, genitourinary, and gastrointestinal systems, as well as the complex psychosocial impact on the developing child. Individuals with SB benefit from the involvement of neurosurgeons, orthopedic surgeons, urologists, physical medicine and rehabilitation specialists, pediatricians, psychologists, physical/occupational/speech therapists, social workers, nurse coordinators, and other personnel. Multidisciplinary clinics are the gold standard for coordinated, optimal medical and surgical care. Ann and Robert H. Lurie Children's Hospital, formerly known as Children's Memorial Hospital, was one of the first hospitals in the USA to manage patients with this complex disease in a multidisciplinary manner. We describe the longitudinal experience of the multidisciplinary Spina Bifida Center at our institution and highlight the advances that have arisen from this care model over time. This clinic serves as an exemplar of organized, effective, and patient-centered approach to the comprehensive care of people living with open SB.

Surgical management of myelocystocele: a single-center experience with long-term functional outcomes.

OBJECTIVE: Myelocystocele (MCC) is an uncommon form of skin-covered spinal dysraphism. The authors aimed to present long-term functional outcomes of patients treated for MCC with and without associated abnormalities of cloacal development (ACD). METHODS: All patients with MCC and at least one tethered cord release (TCR) treated at a single institution between 1982 and 2019 were retrospectively reviewed. Demographic, operative, and functional outcome data were analyzed. RESULTS: Of 51 children with MCC, 30 (58.8%) had MCC only and 21 (41.2%) had associated ACD (MCC/ACD). Thirty-two patients (62.7%) had undergone one TCR, while 19 patients (37.3%) had multiple TCRs. Urinary continence assessment was possible in 41 patients (80.4%), and bowel continence assessment was possible in 43 patients (84.3%) who were either older than 6 years or toilet trained. Although patients with MCC only were more likely to void volitionally (p = 0.0001), there was no difference in overall bladder continence based on the presence of ACD (p = 0.15) or the need for additional untethering procedures (p = 0.15). Those with MCC only were more likely to have overall bowel continence (p = 0.0001) and not require any management (p = 0.002), while those with MCC/ACD were more likely to have an ileostomy (p = 0.01). Of the 30 patients with MCC only, 29 (96.7%) were able to ambulate in the community. Of 21 patients with MCC/ACD, 14 (66.7%) were able to ambulate in the community, 5 (23.8%) were not ambulating, and 2 (9.5%) were therapeutic ambulators. A greater proportion of children in the MCC cohort were ambulating in the community (p = 0.01). There was no difference in community ambulation based on the number of TCRs (p > 0.99), but those with multiple TCRs were more likely to use braces (p = 0.01) and require lower-extremity orthopedic surgery (p = 0.01). CONCLUSIONS: Patients born with an MCC, with or without an associated ACD, attained long-term favorable outcomes in bladder and bowel continence and ambulation.

Assessment of Health Literacy and Self-reported Readiness for Transition to Adult Care Among Adolescents and Young Adults With Spina Bifida.

Importance: Health literacy has been shown to play an important role in transitions of care in adult populations, with low health literacy associated with adverse health outcomes. The role of health literacy in the transition from pediatric to adult care has been less well studied. Among adolescents and young adults with spina bifida, high rates of unsuccessful transition have been shown, but how patient health literacy affects transition readiness remains unknown. Objective: To determine whether health literacy is associated with transition readiness in adolescents and young adults with spina bifida. Design, Setting, and Participants: This cross-sectional study involved collection of patient-reported questionnaires between June 2019 and March 2020 at a multidisciplinary spina bifida center at a single, free-standing children's hospital. Patient demographic and clinical characteristics were obtained from medical record review. Patients were aged 12 years or older with a diagnosis of spina bifida (myelomeningocele and nonmyelomeningocele) whose primary language was English or Spanish. Data analysis was performed from October 2020 to March 2021. Exposures: Health literacy as assessed by the Brief Health Literacy Screening Tool. Main Outcomes and Measures: The primary outcome was total Transition Readiness Assessment Questionnaire (TRAQ) score, normalized into units of SD. Nested, multivariable linear regression models assessed the association between health literacy and TRAQ scores. Results: The TRAQ and Brief Health Literacy Screening Tool were completed by 200 individuals (median [range] age, 17.0 [12.0-31.0] years; 104 female participants [52.0%]). Most of the patients were younger than 18 years (110 participants [55.0%]) and White (136 participants [68.0%]) and had myelomeningocele (125 participants [62.5%]). The mean (SD) TRAQ score was 3.3 (1.1). Sixty-six participants (33.0%) reported inadequate health literacy, 60 participants (30.0%) reported marginal health literacy, and 74 participants (37.0%) reported adequate health literacy. In univariable analysis, health literacy, age, type of spina bifida, level of education, self-administration vs completion of the questionnaires with assistance, ambulatory status, and urinary incontinence were associated with total TRAQ score. In all nested, sequentially adjusted, multivariable models, higher health literacy remained a significant, stepwise, independent variable associated with higher TRAQ score. In the fully adjusted model, having adequate compared with inadequate health literacy was associated with an increase in normalized TRAQ score of 0.49 SD (95% CI, 0.19-0.79). Conclusions and Relevance: Patient-reported transition readiness is associated with health literacy, even after adjustment for education level and other demographic and clinical factors. Developing and implementing health literacy-sensitive care programs during the transition process may improve patient transition readiness.

Differential DNA methylation and transcriptional signatures characterize impairment of muscle stem cells in pediatric human muscle contractures after brain injury.

Limb contractures are a debilitating and progressive consequence of a wide range of upper motor neuron injuries that affect skeletal muscle function. One type of perinatal brain injury causes cerebral palsy (CP), which affects a child's ability to move and is often painful. While several rehabilitation therapies are used to treat contractures, their long-term effectiveness is marginal since such therapies do not change muscle biological properties. Therefore, new therapies based on a biological understanding of contracture development are needed. Here, we show that myoblast progenitors from contractured muscle in children with CP are hyperproliferative. This phenotype is associated with DNA hypermethylation and specific gene expression patterns that favor cell proliferation over quiescence. Treatment of CP myoblasts with 5-azacytidine, a DNA hypomethylating agent, reduced this epigenetic imprint to TD levels, promoting exit from mitosis and molecular mechanisms of cellular quiescence. Together with previous studies demonstrating reduction in myoblast differentiation, this suggests a mechanism of contracture formation that is due to epigenetic modifications that alter the myogenic program of muscle-generating stem cells. We suggest that normalization of DNA methylation levels could rescue myogenesis and promote regulated muscle growth in muscle contracture and thus may represent a new nonsurgical approach to treating this devastating neuromuscular condition.

Myelomeningocele: a new functional classification.

PURPOSE: In myelomeningocele, several classifications have been used. The present manuscript proposes a new functional classification to better assess the prognosis and management of these patients. METHODS: The manual muscle test is what defines the actual group in which the patient should be included. Furthermore, this new classification brings information about the bracing and external supports recommended to each functional level. We also recommend that the patient's Functional Mobility Scale should always be mentioned together with their functional level. RESULTS: The four levels in this classification are MMFC1, MMFC2, MMFC3 and MMFC4. The MMFC1 group includes patients with significant muscle weakness. They need to use high braces crossing the hip joint with a walker to achieve some ambulation. The MMFC2 group includes patients who have functional hip flexors, knee extensors and knee flexors. However, the hip abductors are quite weak. These patients usually need to use a walker - or crutches - and Ankle-Foot Orthosis (AFOs). The MMFC3 group includes patients with functional hip flexors, knee extensors, knee flexors and hip abductors. However, the ankle plantar flexion function is absent. Most of them are able to walk independently, only using AFOs without any external support. The MMFC4 group includes patients who have preserved function in the entire lower limb musculature. These patients don't need any assistive devices to achieve an adequate ambulation pattern. CONCLUSIONS: We hope that this new classification is a system that is simple to understand, serves as a gait prognosis guide and facilitates communication among healthcare professionals. LEVEL OF EVIDENCE: V.

Hip Status and Long-term Functional Outcomes in Spina Bifida.

BACKGROUND: Nearly 50% of individuals with myelomeningocele will develop a dislocated hip by skeletal maturity. The purpose of this study was to determine the influence of hip status on functional outcomes in a cohort of adult patients with myelomeningocele. METHODS: Patients with a diagnosis of myelomeningocele >18 years were prospectively enrolled over a 12-month period. Neurological level of involvement was obtained from chart review and interview. Clinical examination included hip range of motion and leg-length discrepancy. Reimer's migration index was calculated from a current anteroposterior pelvic radiograph. All subjects completed the VR-12 and the NIH PROMIS outcomes measures for pain interference and physical function. The χ, the Pearson correlation coefficients, and linear regression models were applied to evaluate the influence of hip status on functional outcomes. RESULTS: In total, 31 patients (average age 31, range 19 to 49) were included. Eight thoracic, 9 lumbar, and 14 sacral level patients participated. Twenty had bilaterally located hips, 5 had a unilateral subluxation or dislocation, and 6 had bilaterally subluxated or dislocated hips. In univariate analysis, patients with bilaterally located hips performed better in lower extremity function than those with unilateral subluxation/dislocation (36.7 vs. 26.0; P=0.03) but worse in pain interference than those with bilateral subluxation/dislocation (52.0 vs. 43.3; P=0.03). After controlling for neurological level, there was no statistically significant difference in the VR-12 mental (P=0.32) or physical component summary (P=0.32) scores, nor in the PROMIS lower extremity function (P=0.26) or pain interference scores (P=0.33) between groups. Decreased extension and abduction were indirectly correlated with VR-12 mental component scores (P=0.0038, 0.0032). Leg-length discrepancy was not associated with any outcome measure. CONCLUSIONS: Long-term outcomes are not associated with hip status in adult patients with myelomeningocele. Functional outcomes are more closely correlated with neurological level and hip range of motion. These results suggest efforts to keep myelomeningocele hips reduced are likely without functional benefit and should be avoided in favor of maintaining motion with contracture release as needed. LEVEL OF EVIDENCE: Level III.

Midterm Results of the Ponseti Method for Treatment of Clubfoot in Patients With Spina Bifida.

BACKGROUND: Clubfoot occurs in 30% to 50% of patients with spina bifida. The Ponseti casting method has changed treatment of idiopathic clubfoot to a primarily nonoperative regimen. The Ponseti method is now widely applied to clubfoot in spina bifida, however, few studies report treatment outcomes. Most available studies include heterogeneous diagnoses or short-term results. The purpose of this study is to report midterm outcomes in patients with spina bifida and clubfoot treated with the Ponseti method. METHODS: IRB-approved retrospective chart review of 17 consecutive patients (26 feet) below 1 year of age with spina bifida and clubfoot treated with Ponseti method. Charts reviewed for age at treatment initiation, number of casts, surgeries performed, recurrence of deformity, and further treatments. Primary outcome was recurrence of deformity requiring further treatment. Data were analyzed using t tests for means and χ tests for categorical data. RESULTS: Initial correction was achieved in 26 of 26 feet patients. A total of 23 of 26 feet patients underwent a surgical procedure for the tendo-Achilles at an average age of 105 days, 12 percutaneous tenotomies (percT) and 11 open tendonectomy (openT). At average follow-up of 5 (1.8 to 7.5) years, 11 feet (42.3%) in 8 patients were successfully treated with Ponseti method. Of the 15 feet (57.7%) with recurrence, 10 required posterior releases, 4 posterior-medial-lateral releases and 1 tendon transfers. Average age at further treatment was 1.5 years (0.9 to 3.1 y). Those with recurrence required more casts before tendon surgery (7.6 vs. 6.1, P=0.02). A total of 100% patients (12/12) with percT had recurrence of deformity, compared with 18% (2/11) of patients with openT (P<0.0005). CONCLUSIONS: Midterm evaluation of Ponseti method for clubfoot in spina bifida shows a successful outcome in 42.3%. Recurrence with openT was significantly lower than percT and also substantially lower than previously published recurrence rates in spina bifida (33.3% to 68%). In spina bifida, Ponseti method leads to reliable initial correction and is useful to decrease extensive soft tissue release. An open excision of the Achilles should be performed. Families should be counseled about high risk of recurrence and potential need for further treatment. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

Outcomes of Tibial Derotational Osteotomies Performed in Patients With Myelodysplasia.

BACKGROUND: Rotational deformities of the tibia are common in patients with myelodysplasia. The current recommended treatment is tibial derotational osteotomy to improve gait biomechanics. Previously reported complication rates are widely variable. The purpose of this study is to review the outcomes of derotational osteotomies as a function of complication and revision surgery rates as compared with previous studies. METHODS: A retrospective chart review was performed on all tibial derotational osteotomies performed in patients with myelodysplasia from 1985 to 2010 in patients older than 5 years with > 2 years of follow-up. Charts were reviewed for demographics, amount of derotation at index surgery, incidence of complications, and the need for repeat derotational surgery. Descriptive statistics were used to determine the incidence of complications as well as need for reoperation. Further analysis was performed using Fisher Exact Test and the Student t test to identify independent risk factors for complication and rerotation. RESULTS: Eighty-two patients (129 limbs) had sufficient data for inclusion. The average follow-up was 7.15 years. Surgery was indicated for symptomatic torsion measuring > 20 degrees. The average amount of derotation was 28 ± 12 degrees. The incidence of complications was 10.85%, with a 3.10% incidence of major complications including fracture, deep infection, and hardware failure. The repeat derotation rate was 16.28%, all in patients initially treated for external tibial torsion. Age at initial surgery had no effect on complication rate or need for reoperation. Level of spinal involvement was not associated with complication risk; however, lumbar-level involvement was an independent risk factor for rerotation. CONCLUSIONS: With meticulous operative technique, derotational osteotomy of the tibia in patients with myelomeningocele remains a safe and effective method to treat tibial torsion, with an acceptable overall complication rate of 10.85% and a major complication rate of 3.10%. The data presented will aid providers in appropriate counseling of patients considering tibial derotational osteotomy.

Long-term retrospective study of patients with idiopathic clubfoot treated with posterior medial-lateral release.

BACKGROUND: It has been demonstrated that an important component of clubfoot deformity is related to pathologic external rotation of the talus with respect to the remainder of the foot. The purpose of the present study was to review the long-term results for a cohort of patients with idiopathic clubfoot who were managed by a single surgeon with a uniform surgical protocol consisting of extensive posterior medial-lateral release in addition to the use of a temporary Kirschner wire to derotate the talus prior to fixation. METHODS: Eighty patients (120 clubfeet) with idiopathic clubfoot without previous surgery were managed with posterior medial-lateral release. At an average of twenty-one years postoperatively, patients underwent a detailed physical examination and completed four quality-of-life surveys (the Short Form-36, the Laaveg and Ponseti scale, the Foot Function Index, and the modified Atar scale). RESULTS: Thirty-two clubfeet (27%) had required additional procedures at the time of follow-up, with only one patient requiring complete revision posterior medial-lateral release and none requiring subtalar or triple arthrodesis. In patients with unilateral clubfoot, clinical examination demonstrated a significant decrease in the range of motion (p < 0.001), foot length (p = 0.045), and calf circumference (p = 0.008) on the affected side as compared with the unaffected, contralateral side. The results on all four quality-of-life scales remained durable, with no decline in relation to the time from the index procedure (p ≥ 0.48). Significantly worse scores were found for patients who required additional surgical procedures (p ≤ 0.03). CONCLUSIONS: Previous studies have demonstrated inconsistent long-term results following the treatment of clubfoot with extensive soft-tissue releases. With our technique, thirty-two clubfeet (27%) required additional procedures and only one clubfoot required revision posterior medial-lateral release. We demonstrate that our technique, which involves aggressive posterior medial-lateral soft-tissue release in addition to manual derotation of the talus with a Kirschner wire to correct pathologic external rotation, produces acceptable results.

Standard radiographs and computed tomographic scan underestimating pediatric acetabular fracture after traumatic hip dislocation: report of 2 cases.

Posterior wall acetabular fractures are rare in the pediatric patient but require proper management to prevent significant complications. Plain radiographs and computed tomography scans are standard diagnostic imaging used with clinical examination of hip stability to determine if the injury requires open reduction and internal fixation. Two cases are presented of adolescent traumatic hip dislocations and posterior wall fractures in which radiography underestimated the extent of the posterior wall fracture. In one case, a magnetic resonance imaging study more clearly characterized the injury. Because the ossification of the posterior wall occurs throughout adolescence, magnetic resonance imaging may be a useful tool in characterizing these fractures and assisting with surgical planning.

Orthopaedic management of spina bifida-part II: foot and ankle deformities.

Both congenital and acquired orthopaedic deformities are common in patients with spina bifida. Examples of congenital deformities, which are present at birth, include clubfoot and vertical talus. Acquired developmental deformities are related to the level of neurologic involvement and include calcaneus and cavovarus. Orthopaedic deformities may also result from postoperative tethered cord syndrome. The previously published Part I reviewed the overall orthopaedic care of a patient with spina bifida, with a focused review of hip, knee, and rotational deformities. This paper will cover foot and ankle deformities associated with spina bifida, including clubfoot, equinus, vertical talus, calcaneus and calcaneovalgus, ankle and hindfoot valgus, and cavovarus. In addition, this paper will address the issues surrounding skin breakdown in patients with spina bifida.

Radical posterior capsulectomy improves sagittal knee motion in crouch gait.

BACKGROUND: Knee flexion contracture leading to crouch gait is commonly seen in children with myelomeningocele. Progressive increase in knee flexion contracture increases energy cost, which interferes with efficient, functional ambulation. To prevent this, surgical release has been recommended when a knee flexion contracture exceeds 15° to 20°. QUESTIONS/PURPOSES: We therefore asked whether knee flexion contracture release improved dynamic sagittal motion and walking velocity using computerized gait analysis. PATIENTS AND METHODS: We retrospectively studied 11 patients (20 knees) with high-sacral-level or low-lumbar-level myelomeningocele and knee flexion contracture of greater than 15°. All patients underwent dynamic gait analysis pre- and postoperatively. Surgery consisted of selective hamstring lengthening (medial and lateral), gastrocnemius release from the femoral condyles, and posterior knee capsulectomy. RESULTS: We observed improvements postoperatively in clinical measurements and sagittal kinematics. The clinical knee flexion contracture improved from a mean of 24.9° preoperatively to 5.9° postoperatively. The knee flexion at initial contact improved from 37.6° to 9.0°, and minimum knee flexion in single-leg stance improved from 48.2° to 16.4. Walking velocity improved from 72.2% to 80.0% of age-matched normal. CONCLUSIONS: Surgical treatment of knee flexion contracture in patients with myelomeningocele using radical posterior knee capsulectomy leads to improvement in clinical knee flexion contracture, dynamic sagittal kinematics, and walking velocity.

Orthopedic management of spina bifida. Part I: hip, knee, and rotational deformities.

Children with spina bifida develop a wide variety of congenital and acquired orthopedic deformities. Among these are hip deformities such as contracture, subluxation, or dislocation. Patients may also have problems with the knee joint, such as knee flexion or extension contracture, knee valgus deformity, or late knee instability and pain. In addition, rotational deformities of the lower extremities, either internal or external torsion, are common as well. This paper will review both the overall orthopedic care of a patient with spina bifida and provide a focused review of the diagnosis and management of the above deformities. In addition, this paper will review the incidence, etiology, classification, and prognosis of spina bifida. The use of gait analysis and orthoses will be covered as well. The forthcoming Part II will cover foot and ankle deformities in spina bifida.

Difficult-to-treat Ortolani-positive hip: improved success with new treatment protocol.

BACKGROUND: The aim of this paper was to review the outcomes of treatment of dislocated but reducible (Ortolani-positive) hips. METHODS: A comparative retrospective review was conducted of all patients aged younger than 6 months who presented for treatment of developmental dysplasia of the hip for two 10-year periods. The inclusion criteria consisted of a clinical evaluation documenting Ortolani-positive examination, no previous treatment, and an initial treatment with Pavlik harness only. Patients with insufficient follow-up, neuromuscular disease, provocative dislocatable hips, or dislocated but not reducible hips were excluded. In group 1 (1984-1994), treatment involved initiation of Pavlik harness and parental education at the time of initial diagnosis. Group 2 (1997-2007) was treated the same as group 1 with the addition of serial orthopaedic office-based ultrasound examinations and use of a hip abduction orthosis in hips remaining unstable after 3 weeks in the Pavlik harness. Records were assessed for successful achievement and maintenance of hip reduction, need for and type of further treatment, and development of avascular necrosis. RESULTS: In group 1, the mean time of follow-up was 28 months. Forty-four (85%) of 52 hips were treated successfully. Eight patients required further operative treatment. In group 2, the mean follow-up to the final evaluation was 34 months. Forty-one (93%) of 44 hips were treated successfully. Three hips required further treatment. There were no documented cases of avascular necrosis in patients treated for orthoses only in either group. CONCLUSIONS: In 1994, we presented a series of 52 Ortolani-positive hips (group 1) with a success rate of reduction of 85%. Now, our protocol for treating Ortolani-positive hips has evolved to include serial orthopaedic office-based ultrasound in all patients and use of a hip abduction orthosis in hips remaining unstable after 3 weeks in a Pavlik harness. Three previous studies at major centers reported successful reduction in only 63% to 71%. In group 2, our current success rate of 93% exceeds that previously reported by us and is significantly greater than these 3 previous studies (all P < 0.006). Furthermore, our current treatment module has decreased our rate of open reduction from 10% in group 1 to 5% in group 2. LEVEL OF RELEVANCE: Therapeutic study, level 3.

Talonavicular fusion for dorsal subluxation of the navicular in resistant clubfoot.

UNLABELLED: Dorsal rotatory subluxation of the navicular, a common sequela of resistant surgically treated clubfeet, presents a challenging treatment problem. This subluxation typically progresses after posteromedial release. Patients develop a cavovarus foot deformity and complain of pain, gait problems, and difficulty with shoe wear. Previous attempts at soft tissue release and reduction have been largely unsuccessful. We reviewed 13 patients with dorsal rotatory subluxation of the navicular treated with talonavicular arthrodesis. The minimum followup after surgery was 6 months (average, 36 months; range, 6 to 93 months). At last followup 12 of 13 patients were symptom-free. The mean preoperative subluxation of 42% was reduced to a mean of 6% at last followup. We noted improvement in the talo-first metatarsal angle from an average of 18 masculine preoperatively to 8 masculine postoperatively. One patient treated by another surgeon with attempted talonavicular arthrodesis developed a nonunion; we observed no other complications. We believe talonavicular arthrodesis a reasonable option to correct the deformity and symptoms associated with dorsal rotatory subluxation of the navicular in a single surgical setting with a low complication rate. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.