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
Friday, July 3, 2009
Fetal myelomeningocele: Natural history, pathophysiology, and in-utero intervention.
Adzick NS.(2009) Fetal myelomeningocele: Natural history, pathophysiology, and in-utero intervention. Seminars in Fetal and Neonatal Medicine. 2009 Jun 17. [Epub ahead of print]
Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, 34th Street & Civic Center Blvd, Philadelphia, PA 19104, USA.
Myelomeningocele (MMC) is a common birth defect that is associated with significant lifelong morbidity. Little progress has been made in the postnatal surgical management of the child with spina bifida. Postnatal surgery is aimed at covering the exposed spinal cord, preventing infection, and treating hydrocephalus with a ventricular shunt. In-utero repair of open spina bifida is now performed in selected patients and presents an additional therapeutic alternative for expectant mothers carrying a fetus with MMC. It is estimated that about 400 fetal operations have now been performed for MMC worldwide. Despite this large experience, the technique remains of unproven benefit. Preliminary results suggest that fetal surgery results in reversal of hindbrain herniation (the Chiari II malformation), a decrease in shunt-dependent hydrocephalus, and possibly improvement in leg function, but these findings might be explained by selection bias and changing management indications. A randomized prospective trial (the MOMS trial) is currently being conducted by three centers in the USA, and is estimated to be completed in 2010. Further research is needed to better understand the pathophysiology of MMC, the ideal timing and technique of repair, and the long-term impact of in-utero intervention.
PMID: 19540177
Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, 34th Street & Civic Center Blvd, Philadelphia, PA 19104, USA.
Myelomeningocele (MMC) is a common birth defect that is associated with significant lifelong morbidity. Little progress has been made in the postnatal surgical management of the child with spina bifida. Postnatal surgery is aimed at covering the exposed spinal cord, preventing infection, and treating hydrocephalus with a ventricular shunt. In-utero repair of open spina bifida is now performed in selected patients and presents an additional therapeutic alternative for expectant mothers carrying a fetus with MMC. It is estimated that about 400 fetal operations have now been performed for MMC worldwide. Despite this large experience, the technique remains of unproven benefit. Preliminary results suggest that fetal surgery results in reversal of hindbrain herniation (the Chiari II malformation), a decrease in shunt-dependent hydrocephalus, and possibly improvement in leg function, but these findings might be explained by selection bias and changing management indications. A randomized prospective trial (the MOMS trial) is currently being conducted by three centers in the USA, and is estimated to be completed in 2010. Further research is needed to better understand the pathophysiology of MMC, the ideal timing and technique of repair, and the long-term impact of in-utero intervention.
PMID: 19540177
Early shunt complications in 46 children with hydrocephalus.
Braga MH, Carvalho GT, Brandão RA, Lima FB, Costa BS. (2009) Early shunt complications in 46 children with hydrocephalus. Arquivos de neuro-psiquiatria. 2009 Jun;67(2A):273-7.
Department of Neurosurgery, Santa Casa de Belo Horizonte, MG, Brazil.
OBJECTIVE: To determine the causes of early shunt complications in 46 children with hydrocephalus. METHOD: A retrospective study was conducted on 46 children submitted to ventriculoperitoneal shunt surgery between February 2005 and February 2007. RESULTS: Thirteen (28%) patients presented complications, which were due to infection in 9 (69%) and to malfunction of the shunt system in 4 (31%).The mean number of surgical procedures performed on patients who presented complications was 2.8 per patient, with a total of 46 surgeries in this group. All patients with infectious complications were identified during their hospital stay. CONCLUSIONS: Infection was the most common complication. The infection rate was proportional to the length of hospital stay. All patients with hydrocephalus due to tumors or myelomeningocele presented complications. A higher incidence of infections was observed in children older than 2 years.
PMID: 19547822
Department of Neurosurgery, Santa Casa de Belo Horizonte, MG, Brazil.
OBJECTIVE: To determine the causes of early shunt complications in 46 children with hydrocephalus. METHOD: A retrospective study was conducted on 46 children submitted to ventriculoperitoneal shunt surgery between February 2005 and February 2007. RESULTS: Thirteen (28%) patients presented complications, which were due to infection in 9 (69%) and to malfunction of the shunt system in 4 (31%).The mean number of surgical procedures performed on patients who presented complications was 2.8 per patient, with a total of 46 surgeries in this group. All patients with infectious complications were identified during their hospital stay. CONCLUSIONS: Infection was the most common complication. The infection rate was proportional to the length of hospital stay. All patients with hydrocephalus due to tumors or myelomeningocele presented complications. A higher incidence of infections was observed in children older than 2 years.
PMID: 19547822
Fetal surgery for myelomeningocele.
Hirose S, Farmer DL. (2009) Fetal surgery for myelomeningocele. Clinics in Perinatology. 2009 Jun;36(2):431-8, xi.
Division of Pediatric Surgery, Department of Surgery, Fetal Treatment Center, University of California, San Francisco, CA 94143-0570, USA. hiroses@surgery.ucsf.edu
Fetal intervention for myelomeningocele (MMC) may improve hydrocephalus and hindbrain herniation associated with the Arnold-Chiari II malformation and may reduce the need for ventriculoperitoneal shunting. As of now, there is little evidence that prenatal repair of MMC improves neurologic function. MMC is the first nonlethal disease under consideration and study for fetal surgery. As a result, potential improvements in outcome must be balanced with maternal safety and well-being, in addition to that of the unborn patient.
PMID: 19559329
Division of Pediatric Surgery, Department of Surgery, Fetal Treatment Center, University of California, San Francisco, CA 94143-0570, USA. hiroses@surgery.ucsf.edu
Fetal intervention for myelomeningocele (MMC) may improve hydrocephalus and hindbrain herniation associated with the Arnold-Chiari II malformation and may reduce the need for ventriculoperitoneal shunting. As of now, there is little evidence that prenatal repair of MMC improves neurologic function. MMC is the first nonlethal disease under consideration and study for fetal surgery. As a result, potential improvements in outcome must be balanced with maternal safety and well-being, in addition to that of the unborn patient.
PMID: 19559329
Intradural inclusion cysts following in utero closure of myelomeningocele: clinical implications and follow-up findings.
Danzer E, Adzick NS, Rintoul NE, Zarnow DM, Schwartz ES, Melchionni J, Ernst LM, Flake AW, Sutton LN, Johnson MP. (2008) Intradural inclusion cysts following in utero closure of myelomeningocele: clinical implications and follow-up findings.
Journal of Neurosurgery: Pediatrics. 2008 Dec;2(6):406-13.
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia; and The University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.
OBJECT: The goal in this study was to evaluate the incidence and clinical implications of the development of cutaneously derived intradural inclusion cysts (ICs) following fetal myelomeningocele (fMMC) closure.
METHODS: Retrospective databases and responses to a parental questionnaire were reviewed to determine the incidence, clinical presentation, and outcomes of fMMCs in children in whom ICs developed at follow-up.
RESULTS: Prior to the National Institutes of Health (NIH)-sponsored Management of Myelomeningocele Study (MOMS), 54 patients underwent fMMC closure at the authors' institution. Sixteen (30%) presented with symptomatic tethered cord syndrome (TCS) at a median age of 27 months (range 4-93 months). Ten (63%) of the 16 (19% of the total) developed TCS in association with an intradural IC. In 9 (90%) of 10 patients, the IC was seen on preoperative MR imaging, and in 1 it was found during surgery. Four additional children (7% of the total) with evidence of an IC on surveillance MR imaging are currently asymptomatic at 94, 84, 60, and 60 months of age, respectively. All but 1 (an L-3 level lesion) IC developed in infants with L-4 and L-5 defects. After cyst removal, 6 children are asymptomatic at a median follow-up of 36 months (range 12-63 months). Following IC removal, 4 children lost normal bladder function and now require clean intermittent catheterization, and 1 lost normal leg function and now requires a walking aid for ambulation. Histologically, 8 lesions were dermoid, 1 was an epidermoid, and 1 was a mixed dermoid-epidermoid IC. Three patients developed another IC and required its removal at 24, 39, and 51 months, respectively. One required another tethered cord release within 57 months after IC removal.
CONCLUSIONS: Cutaneously derived intradural ICs can develop following fMMC surgery. Deterioration of bladder function, risk of recurrence, and loss of lower-extremity function appear to be the most important long-term complications of IC in children with fMMCs. The ongoing NIH-sponsored MOMS may help determine whether children with fMMC are at increased risk of IC development compared with children treated with postnatal MMC closure. Parents seeking fMMC closure should be informed about the possibility of IC formation and the potential clinical consequences.
PMID: 19035686
Journal of Neurosurgery: Pediatrics. 2008 Dec;2(6):406-13.
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia; and The University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.
OBJECT: The goal in this study was to evaluate the incidence and clinical implications of the development of cutaneously derived intradural inclusion cysts (ICs) following fetal myelomeningocele (fMMC) closure.
METHODS: Retrospective databases and responses to a parental questionnaire were reviewed to determine the incidence, clinical presentation, and outcomes of fMMCs in children in whom ICs developed at follow-up.
RESULTS: Prior to the National Institutes of Health (NIH)-sponsored Management of Myelomeningocele Study (MOMS), 54 patients underwent fMMC closure at the authors' institution. Sixteen (30%) presented with symptomatic tethered cord syndrome (TCS) at a median age of 27 months (range 4-93 months). Ten (63%) of the 16 (19% of the total) developed TCS in association with an intradural IC. In 9 (90%) of 10 patients, the IC was seen on preoperative MR imaging, and in 1 it was found during surgery. Four additional children (7% of the total) with evidence of an IC on surveillance MR imaging are currently asymptomatic at 94, 84, 60, and 60 months of age, respectively. All but 1 (an L-3 level lesion) IC developed in infants with L-4 and L-5 defects. After cyst removal, 6 children are asymptomatic at a median follow-up of 36 months (range 12-63 months). Following IC removal, 4 children lost normal bladder function and now require clean intermittent catheterization, and 1 lost normal leg function and now requires a walking aid for ambulation. Histologically, 8 lesions were dermoid, 1 was an epidermoid, and 1 was a mixed dermoid-epidermoid IC. Three patients developed another IC and required its removal at 24, 39, and 51 months, respectively. One required another tethered cord release within 57 months after IC removal.
CONCLUSIONS: Cutaneously derived intradural ICs can develop following fMMC surgery. Deterioration of bladder function, risk of recurrence, and loss of lower-extremity function appear to be the most important long-term complications of IC in children with fMMCs. The ongoing NIH-sponsored MOMS may help determine whether children with fMMC are at increased risk of IC development compared with children treated with postnatal MMC closure. Parents seeking fMMC closure should be informed about the possibility of IC formation and the potential clinical consequences.
PMID: 19035686
Reversal of hindbrain herniation after maternal-fetal surgery for myelomeningocele subsequently impacts on brain stem function.
Danzer E, Finkel RS, Rintoul NE, Bebbington MW, Schwartz ES, Zarnow DM, Adzick NS, Johnson MP. (2009) Reversal of hindbrain herniation after maternal-fetal surgery for myelomeningocele subsequently impacts on brain stem function. Neuropediatrics. 2008 Dec;39(6):359-62.
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.
The aim of our study was to delineate whether the reversal of hindbrain herniation (HH) following fetal myelomeningocele (fMMC) closure subsequently reduces the incidence and severity of HH-associated brainstem dysfunction (BSD). Prior to the NIH-sponsored Management of Myelomeningocele Study (MOMS) trial, 54 children underwent fMMC closure at our institution. Forty-eight (89%) families participated in a structured survey focusing on HH-associated BSD (e.g., apnea, neurogenic dysphagia [ND], gastro-esophageal reflux disease [GERD], neuro-ophthalmologic disturbances [NOD]). Median age at follow-up was 72 months (range: 46-98). Fifty-percent required shunting. HH-related symptoms were completely absent in 15 (63%) non-shunted and 10 (42%) shunted children (P=0.15). No HH-related death occurred and none developed severe persistent cyanotic apnea. ND was reported in 2 (8%) non-shunted and 9 (38%) shunted infants (P=0.03). Mild GERD (medically managed) developed in 2 (8%) without and 6 (25%) with shunt placement (P=0.24). NOD was found in 6 (25%) and 13 (54%) of non-shunted and shunted children, respectively (P=0.07). The majority of fMMC children developed no or only mild BSD at follow-up. Our data support the hypothesis that neurodevelopmental deficits associated with MMC are at least partially acquired and that reversal of HH following fMMC surgery may help to reduce the incidence and severity of BSD.
PMID: 19569004
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.
The aim of our study was to delineate whether the reversal of hindbrain herniation (HH) following fetal myelomeningocele (fMMC) closure subsequently reduces the incidence and severity of HH-associated brainstem dysfunction (BSD). Prior to the NIH-sponsored Management of Myelomeningocele Study (MOMS) trial, 54 children underwent fMMC closure at our institution. Forty-eight (89%) families participated in a structured survey focusing on HH-associated BSD (e.g., apnea, neurogenic dysphagia [ND], gastro-esophageal reflux disease [GERD], neuro-ophthalmologic disturbances [NOD]). Median age at follow-up was 72 months (range: 46-98). Fifty-percent required shunting. HH-related symptoms were completely absent in 15 (63%) non-shunted and 10 (42%) shunted children (P=0.15). No HH-related death occurred and none developed severe persistent cyanotic apnea. ND was reported in 2 (8%) non-shunted and 9 (38%) shunted infants (P=0.03). Mild GERD (medically managed) developed in 2 (8%) without and 6 (25%) with shunt placement (P=0.24). NOD was found in 6 (25%) and 13 (54%) of non-shunted and shunted children, respectively (P=0.07). The majority of fMMC children developed no or only mild BSD at follow-up. Our data support the hypothesis that neurodevelopmental deficits associated with MMC are at least partially acquired and that reversal of HH following fMMC surgery may help to reduce the incidence and severity of BSD.
PMID: 19569004
The outcome of tethered cord release in secondary and multiple repeat tethered cord syndrome.
Al-Holou WN, Muraszko KM, Garton HJ, Buchman SR, Maher CO. (2009) The outcome of tethered cord release in secondary and multiple repeat tethered cord syndrome.
Journal of Neurosurgery Pediatrics. 2009 Jul;4(1):28-36.
Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan.
Object After primary repair of a myelomeningocele or a lipomyelomeningocele, patients can present with symptoms of secondary tethered cord syndrome (TCS). After surgical untethering, a small percentage of these patients can present with multiple repeat TCS. In patients presenting with secondary or multiple repeat TCS, the role as well the expected outcomes of surgical untethering are not well defined. Methods Eighty-four patients who underwent spinal cord untethering after at least 1 primary repair were retrospectively evaluated using scaled and subjective outcome measures at short-term and long-term follow-up visits. Outcomes were analyzed for predictive measures using multivariate logistic regression. Results Surgical untethering was performed in 66 patients with myelomeningoceles and 18 patients with lipomyelomeningoceles. Fourteen patients underwent multiple repeat spinal cord untethering. Patients were followed up for an average of 6.2 years. Most patients had stability of function postoperatively. Motor function and weakness improved in 7 and 16% of patients at 6 months, respectively, and 6 and 19% of patients at long-term follow-up evaluation, respectively. Of the patients who presented with back pain, 75% had improvement in symptoms at 6 months postoperatively. Younger age at untethering was significantly associated with worse long-term neurological outcomes. The number of previous untethering procedures, original diagnosis, sex, anatomical level, and degree of untethering had no effect on surgical outcomes. Conclusions Patients presenting with secondary or multiple repeat TCS may benefit from surgical untethering.
PMID: 19569907
Journal of Neurosurgery Pediatrics. 2009 Jul;4(1):28-36.
Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan.
Object After primary repair of a myelomeningocele or a lipomyelomeningocele, patients can present with symptoms of secondary tethered cord syndrome (TCS). After surgical untethering, a small percentage of these patients can present with multiple repeat TCS. In patients presenting with secondary or multiple repeat TCS, the role as well the expected outcomes of surgical untethering are not well defined. Methods Eighty-four patients who underwent spinal cord untethering after at least 1 primary repair were retrospectively evaluated using scaled and subjective outcome measures at short-term and long-term follow-up visits. Outcomes were analyzed for predictive measures using multivariate logistic regression. Results Surgical untethering was performed in 66 patients with myelomeningoceles and 18 patients with lipomyelomeningoceles. Fourteen patients underwent multiple repeat spinal cord untethering. Patients were followed up for an average of 6.2 years. Most patients had stability of function postoperatively. Motor function and weakness improved in 7 and 16% of patients at 6 months, respectively, and 6 and 19% of patients at long-term follow-up evaluation, respectively. Of the patients who presented with back pain, 75% had improvement in symptoms at 6 months postoperatively. Younger age at untethering was significantly associated with worse long-term neurological outcomes. The number of previous untethering procedures, original diagnosis, sex, anatomical level, and degree of untethering had no effect on surgical outcomes. Conclusions Patients presenting with secondary or multiple repeat TCS may benefit from surgical untethering.
PMID: 19569907
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