Urological Results after Fetal Myelomeningocele Repair in Pre-MOMS Trial Patients at the Children's Hospital of Philadelphia

2014 ◽  
Vol 37 (3) ◽  
pp. 211-218 ◽  
Author(s):  
Michael C. Carr
Keyword(s):  
Lower Extremity ◽  
In Utero ◽  
Neurological Deficits ◽  
Lower Extremity Function ◽  
Medical Issues ◽  
Ventriculoperitoneal Shunting ◽  
Improved Outcomes ◽  
Chiari Ii ◽  
Myelomeningocele Repair

Introduction: Myelomeningocele patients deal with multiple medical issues, including lower extremity neurological deficits, bowel and bladder incontinence and the sequelae of hydrocephalus secondary to a Chiari II malformation. In utero intervention holds the promise of reversing some of the sequelae and improving outcome. Material and Methods: Between 1998 and 2003 (preceding the formal Management of Myelomeningocele Study, MOMS), an initial group of 58 patients underwent in utero repair of their myelomeningocele between 21 and 25 weeks' gestation. Long-term (5-year) follow-up has occurred in this cohort of patients. Previous reports have documented decreased incidence of ventriculoperitoneal shunting and neuromotor functioning, showing improved outcomes compared with historical controls. Results: Overall, 4 fetal deaths occurred, while the majority of patients returned for follow-up for up to 5 years after closure. Phone follow-up has also been conducted for those who could not return. To date, 10 patients (18.5%) have successfully toilet-trained, while 2 patients have bowel continence and 1 has bladder continence but requires enemas; 2 patients who successfully toilet-trained developed spinal dermoid cysts requiring surgical resection. Discussion: Historically, in utero repair of myelomeningocele patients yields a greater percentage of patients who have achieved continence compared with those undergoing postnatal repair. The MOMS trial will compare contemporary urological outcomes of those patients undergoing either prenatal or postnatal repair in a randomized fashion. The results of this trial showed a decreased need for ventriculoperitoneal shunting in those patients who underwent in utero repair as well as an improvement in lower extremity function.

Morphometric analysis of posterior fossa after in utero myelomeningocele repair

2011 ◽  
Vol 7 (4) ◽  
pp. 362-368 ◽  
Author(s):  
Ryan A. Grant ◽  
Gregory G. Heuer ◽  
Geneive M. Carrión ◽  
N. Scott Adzick ◽  
Erin S. Schwartz ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Surgical Repair ◽  
Correlation Coefficients ◽  
In Utero ◽  
Clinical Effects ◽  
Type Ii ◽  
Tonsillar Herniation ◽  
Myelomeningocele Repair ◽  
Repair Group

Object Myelomeningocele (MMC) is characterized by a defect in caudal neurulation and appears at birth with a constellation of neuroanatomical abnormalities, including Chiari malformation Type II. The authors investigated the effects of antenatal versus postnatal repair of MMC through a quantitative analysis of morphometric changes in the posterior fossa (PF). Methods The authors retrospectively reviewed the records of 29 patients who underwent in utero MMC repair, 24 patients who underwent postnatal repair, and 114 fetal and pediatric controls. Tonsillar displacement, cerebellum length, pons length, clivus-supraocciput (CSO) angle, and PF area were compared in antenatal and postnatal MMC repair groups as well as in controls without neural tube defects by using t-tests and correlation coefficients. Results Initially, the in utero CSO angle was significantly more acute in all patients with MMC—prenatally and postnatally repaired—as compared with controls (57.8° vs 75.4°, p < 0.001); however, the angle rapidly changed and became similar to that in controls between 30 and 31 weeks' gestation to approximately 80°, with antenatal repair having little effect. Postnatally, the CSO angle decreased in controls (R = −0.58) and in the antenatal repair group (R = −0.17). The cerebellum and pons length demonstrated no significant differences in any group. Overall, tonsil descent was corrected in the antenatal repair group as compared with postnatal repair (p < 0.001), and the PF area increased in all 3 groups in utero. Growth was less rapid in patients with MMC compared with controls, but this was corrected by antenatal repair (p = 0.015). Conclusions Myelomeningocele was associated with tonsillar herniation and a smaller PF than in control fetuses. Antenatal surgical repair corrected both abnormalities. The CSO angle began significantly more acutely in patients with MMC, but normalized with development regardless of when surgery was performed. Determining the clinical effects of antenatal repair requires further follow-up.

scholarly journals Outcomes after In Utero Myelomeningocele Repair Based on Delivery Location

2020 ◽  
Vol 9 (11) ◽  
pp. 3443
Author(s):  
Eric P. Bergh ◽  
Kuojen Tsao ◽  
Mary T. Austin ◽  
Stephen A. Fletcher ◽  
Suzanne M. Lopez ◽  
...  
Keyword(s):  
Neonatal Intensive Care ◽  
Wound Dehiscence ◽  
In Utero ◽  
Cerebrospinal Fluid Leakage ◽  
Fetal Intervention ◽  
Gestational Age At Delivery ◽  
Myelomeningocele Repair ◽  
Lost To Follow Up ◽  
Preterm Rupture Of Membranes

Maternal and pediatric delivery outcomes may vary in patients who underwent open fetal myelomeningocele repair and elected to deliver at the fetal center where their fetal intervention was performed versus at the referring physician’s hospital. A prospective cohort study of 88 patients were evaluated following in utero open fetal myelomeningocele repair at a single fetal center between the years 2011–2019. Exclusion criteria included patients that delivered within two weeks of the procedure (n = 6), or if a patient was lost to follow-up (n = 1). Of 82 patients meeting inclusion criteria, 36 (44%) patients were delivered at the fetal center that performed fetal intervention, and 46 (56%) were delivered locally. Comparative statistics found that with the exception of parity, baseline characteristics and pre-operative variables did not differ between the groups. No differences in oligohydramnios incidence, preterm rupture of membranes, gestational age at delivery or delivery indications were found. Patients who delivered with a referring physician were more likely to be multiparous (p = 0.015). With the exception of a longer neonatal intensive care unit (NICU) stay in the fetal center group (median 30.0 vs. 11.0 days, p = 0.004), there were no differences in neonatal outcomes, including wound dehiscence, cerebrospinal fluid leakage, patch management, ventricular diversion, or prematurity complications. Therefore, we conclude that it is safe to allow patients to travel home for obstetric and neonatal management after open fetal myelomeningocele repair.

Late Gestational Intrauterine Myelomeningocele Repair Does Not Improve Lower Extremity Function

2003 ◽  
Vol 38 (3) ◽  
pp. 128-132 ◽  
Author(s):  
R. Shane Tubbs ◽  
M. Renee Chambers ◽  
Matthew D. Smyth ◽  
Alfred A. Bartolucci ◽  
Joseph P. Bruner ◽  
...  
Keyword(s):  
Lower Extremity ◽  
Lower Extremity Function ◽  
Extremity Function ◽  
Myelomeningocele Repair

Fetoscopic Multilayer, Dural Patch Closure Technique for Intrauterine Myelomeningocele Repair: 2-Dimensional Operative Video

2020 ◽  
Author(s):  
Smruti K Patel ◽  
Mounira A Habli ◽  
David N McKinney ◽  
Sammy M Tabbah ◽  
Foong-Yen Lim ◽  
...  
Keyword(s):  
Vaginal Delivery ◽  
Patch Repair ◽  
Term Survival ◽  
Radiographic Outcomes ◽  
Fetal Repair ◽  
Chiari Ii ◽  
Key Steps ◽  
Myelomeningocele Repair ◽  
Dural Patch

Abstract Myelomeningocele (MMC) is the most common open neural tube defect associated with long-term survival. In 2011, The Management of Myelomeningocele Study (MOMS) trial demonstrated that fetal repair for MMC reduced the rate of shunted hydrocephalus and improved developmental, motor, and ambulation outcomes at 30 mo compared to postnatal intervention.1 Recent studies have demonstrated the safety and feasibility of fetoscopic MMC repair as well as reduction in preterm birth, lower risk of uterine dehiscence, and the option of vaginal delivery with this approach compared to open fetal repair.2-4 The patient is a 25-yr-old female, G4 P2, who presented at 20 wk's gestation with ultrasound findings concerning for MMC and Chiari II malformation. These findings were further corroborated with fetal magnetic resonance imaging. After extensive prenatal counseling in a multidisciplinary fashion and discussion regarding risks and benefits of prenatal closure of the MMC, the patient chose to undergo prenatal repair and surgical consent was obtained. At 25 wk's gestation, the patient underwent a fetoscopic multilayer closure with dural patch repair using a standardized, 3-port, carbon dioxide insufflation technique for the intrauterine treatment of MMC without any postoperative complications. The duration of the entire procedure was 275 min. At 36 wk's and 1 d's gestational age, the patient had a spontaneous vaginal delivery, resulting in a healthy male newborn. The surgical site was well healed without complications, and follow-up radiographic imaging was reassuring. This edited, 2-dimensional operative video highlights the key steps of the fetoscopic closure with follow-up postnatal clinical and radiographic outcomes.

Recurrence of hypertrophic spinal pachymeningitis

2006 ◽  
Vol 4 (6) ◽  
pp. 509-513 ◽  
Author(s):  
Zenya Ito ◽  
Yoshimitsu Osawa ◽  
Yukihiro Matsuyama ◽  
Takaaki Aoki ◽  
Atsushi Harada ◽  
...  
Keyword(s):  
Lower Extremity ◽  
Clinical Symptoms ◽  
Gait Disturbance ◽  
Neurological Deficits ◽  
Japanese Language ◽  
Steroid Pulse ◽  
First Case ◽  
Chi Square Analysis ◽  
Language Literature

✓Hypertrophic spinal pachymeningitis (HSP) is a comparatively rare disease characterized by hypertrophic inflammation of the dura mater and clinical symptoms that progress from local pain to myelopathy. The authors report two cases of recurrent HSP and review the English- and Japanese-language literature focusing on the recurrence of HSP. In the first case, a man who presented at 67 years of age with lower-extremity numbness, gait disturbance, and bladder dysfunction experienced two recurrences of HSP during the 11 years of follow up after his initial laminectomy. Both recurrences were successfully treated with laminoplasty and duraplasty. Three years after his last surgical procedure, he was still able to walk with the aid of a walker. In the second case, a man who presented at 62 years of age with lower-extremity numbness and gait disturbance was initially treated successfully with steroid pulse therapy. Approximately 8 months after his initial presentation, his symptoms recurred. He underwent laminoplasty and duraplasty. At the 2.5-year follow-up examination, he had only mild neurological deficits and was still able to walk unaided. To explore possible causes of recurrence, the authors searched the English- and Japanese-language literature for cases of HSP. Of the 96 cases identified, 11 were recurrent. Data on the presence or absence of inflammatory signs were available for 84 patients. A chi-square analysis revealed a significantly increased rate of recurrence for patients who had at least one positive inflammatory sign before surgery (six [20%] recurrent cases of 30) compared with those who had no positive inflammatory signs before surgery (two [3.7%] recurrent cases of 54) (p < 0.05). The authors conclude that HSP recurrence occurs because of active inflammation of the dura before surgery and the influence of chronic inflammation, including residual arachnoiditis.

Two-level thoracic pedicle subtraction osteotomy for progressive post-laminectomy kyphotic deformity following resection of an unusual thoracolumbar intradural extramedullary tumor

2012 ◽  
Vol 10 (4) ◽  
pp. 334-339 ◽  
Author(s):  
Brian J. Kelley ◽  
Michele H. Johnson ◽  
Alexander O. Vortmeyer ◽  
Brian G. Smith ◽  
Khalid M. Abbed
Keyword(s):  
Lower Extremity ◽  
Poor Prognosis ◽  
Pedicle Subtraction Osteotomy ◽  
Foot Drop ◽  
Kyphotic Deformity ◽  
Neurological Deficits ◽  
Lower Extremity Function ◽  
Lower Extremity Weakness ◽  
Postoperative Course ◽  
Extramedullary Tumor

The authors report a case in which multilevel thoracic pedicle subtraction osteotomy (PSO) was performed to correct post-laminectomy kyphotic deformity in a 9-year-old boy presenting with worsening lower-extremity neurological deficits. Five years prior to presentation, the patient underwent multilevel thoracolumbar laminectomies for resection of an atypical teratoid/rhabdoid tumor (AT/RT), a rare lesion that typically occurs intracranially and has a poor prognosis, making this particular presentation unusual and the patient's subsequent postoperative course remarkable. No fusion was undertaken at the time of resection, given the patient's age and presumptive poor prognosis. Over the next 5 years, the patient developed progressive thoracolumbar kyphotic deformity, with a Cobb angle greater than 110°, despite bracing, and bilateral lower-extremity weakness requiring ankle-foot orthotics for continued ambulation due to progressive foot drop. Worsening gait and the onset of respiratory issues prompted surgical intervention. Multilevel thoracic PSO and thoracolumbar fusion were performed, resulting in improved lower-extremity function and correction of the kyphotic deformity to approximately 65°. This report outlines an unusual AT/RT presentation and postoperative course and also discusses literature related to PSO within the context of pediatric kyphotic deformity. The authors' experience supports the use of multilevel PSO with fusion as a potential treatment option for significant pediatric thoracolumbar kyphotic deformity requiring surgical correction.

scholarly journals Extremity Dysfunction After Large‐Bore Radial and Femoral Arterial Access

2022 ◽  
Author(s):  
Thomas A. Meijers ◽  
Adel Aminian ◽  
Marleen van Wely ◽  
Koen Teeuwen ◽  
Thomas Schmitz ◽  
...  
Keyword(s):  
Lower Extremity ◽  
Coronary Intervention ◽  
Lower Extremity Function ◽  
Access Site ◽  
Functional Scale ◽  
Femoral Access ◽  
Arterial Access ◽  
Extremity Function ◽  
Percutaneous Coronary

Background The use of large‐bore (LB) arterial access and guiding catheters has been advocated for complex percutaneous coronary intervention. However, the impact of LB transradial access (TRA) and transfemoral access (TFA) on extremity dysfunction is currently unknown. Methods and Results The predefined substudy of the COLOR (Complex Large‐Bore Radial PCI) trial aimed to assess upper and lower‐extremity dysfunction after LB radial and femoral access. Upper‐extremity function was assessed in LB TRA‐treated patients by the Quick Disabilities of the Arm, Shoulder, and Hand questionnaire and lower‐extremity function in LB TFA‐treated patients by the Lower Extremity Functional Scale questionnaire. Extremity pain and effect of access site complications and risk factors on extremity dysfunction was also analyzed. There were 343 patients who completed analyzable questionnaires. Overall, upper and lower‐extremity function did not decrease over time when LB TRA and TFA were used for complex percutaneous coronary intervention, as represented by the median Quick Disabilities of the Arm, Shoulder, and Hand score (6.8 at baseline and 2.1 at follow‐up, higher is worse) and Lower Extremity Functional Scale score (56 at baseline and 58 at follow‐up, lower is worse). Clinically relevant extremity dysfunction occurred in 6% after TRA and 9% after TFA. A trend for more pronounced upper‐limb dysfunction was present in female patients after LB TRA ( P =0.05). Lower‐extremity pain at discharge was significantly higher in patients with femoral access site complications ( P =0.02). Conclusions Following LB TRA and TFA, self‐reported upper and lower‐limb function did not decrease over time in the majority of patients. Clinically relevant limb dysfunction occurs in a small minority of patients regardless of radial or femoral access. Registration URL: https://www.clinicaltrials.gov ; Unique identifier: NCT03846752.

scholarly journals Progressive hydrocephalus despite early complete reversal of hindbrain herniation after prenatal open myelomeningocele repair

2019 ◽  
Vol 47 (4) ◽  
pp. E13 ◽  
Author(s):  
Victor M. Lu ◽  
Kendall A. Snyder ◽  
Eniola R. Ibirogba ◽  
Rodrigo Ruano ◽  
David J. Daniels ◽  
...  
Keyword(s):  
Third Ventriculostomy ◽  
Ventriculoperitoneal Shunting ◽  
Gradual Onset ◽  
Pertinent Data ◽  
Before And After ◽  
Complete Reversal ◽  
Hindbrain Herniation ◽  
Myelomeningocele Repair

OBJECTIVEOpen prenatal myelomeningocele (MMC) repair is typically associated with reversal of in utero hindbrain herniation (HBH) and has been posited to be associated with a reduction in both postoperative prenatal and immediate postnatal hydrocephalus (HCP) risks. However, the long-term postnatal risk of HCP following HBH reversal in these cases has not been well defined. The authors describe the results of a long-term HCP surveillance in a cohort of patients who underwent prenatal MMC repair at their institution.METHODSA retrospective review of all prenatal MMC repair operations performed at the Mayo Clinic between 2012 and 2017 was conducted. Pertinent data regarding the clinical courses of these patients before and after MMC repair were summarized. Outcomes of interest were occurrences of HBH and HCP and the need for intervention.RESULTSA total of 9 prenatal MMC repair cases were identified. There were 7 cases in which MRI clearly demonstrated prenatal HBH, and of these 86% (6/7) had evidence of HBH reversal after repair and prior to delivery. After a mean postnatal follow-up of 20 months, there were 3 cases of postnatal HCP requiring intervention. One case that failed to show complete HBH reversal after MMC repair required early ventriculoperitoneal shunting. The other 2 cases were of progressive, gradual-onset HCP despite complete prenatal HBH reversal, requiring endoscopic third ventriculostomy with choroid plexus cauterization at ages 5 and 7 months.CONCLUSIONSAlthough prenatal MMC repair can achieve HBH reversal in a majority of well-selected cases, the prevention of postnatal HCP requiring intervention appears not to be predicated on this outcome alone. In fact, it appears that in a subset of cases in which HBH reversal is achieved, patients can experience a progressive, gradual-onset HCP within the 1st year of life. These findings support continued rigorous postnatal surveillance of all prenatal MMC repair patients, irrespective of postoperative HBH outcome.

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