Assessment of lower limb trophism in fetuses with open spina bifida using fractional thigh volume of three-dimensional ultrasound.

OBJECTIVE: To assess the trophism of the lower limbs of fetuses with open spina bifida using fractional thigh volume (TVOL) of three-dimensional (3D) ultrasound. METHODS: A prospective cross-sectional study was carried out with normal fetuses and with open spina bifida (myelomeningocele and rachischisis) at 26 weeks' gestation. The TVOL (delimitation of five cross-sectional areas of the middle portion of the limb) was evaluated, as well as the subjective assessment of hypotrophy and lower limb movement. RESULTS: Thirty-one fetuses with open spina bifida, 21 with myelomeningocele and 10 with rachischisis, and 51 normal fetuses were included. There were no significant differences in the TVOL between normal and spina bifida fetuses (p = 0.623), as well as between normal fetuses, with myelomeningocele and with rachischisis (p = 0.148). There was no significant difference in the TVOL of fetuses with spina bifida with or without lower limb hypotrophy (p = 0.148). Fetuses with spina bifida and with lower limb movement had higher TVOL values than fetuses without lower limb movement (p = 0.002). CONCLUSION: There were no significant differences in the TVOL measurement of normal and spina bifida fetuses (rachischisis and myelomeningocele). Fetuses with spina bifida without spontaneous movement of the lower limbs had lower TVOL values.

Open intrauterine repair of spina bifida aperta: Historical aspects, current availability, and clinical outcomes from the Latin American Spina Bifida Consortium.

OBJECTIVE: To determine the historical aspects, current availability, and clinical outcomes of open intrauterine repair of spina bifida aperta (IRSBA) in Spanish-speaking Latin American countries. METHODS: Cases were collected from centers with at least 2 years of experience and a minimum of 10 open IRSBA interventions by December 2020. Clinical variables were compared to the results of the Management of Myelomeningocele Study (MOMS) trial. RESULTS: Clinical experience with 314 cases from seven centers was reviewed. Most cases (n = 189, 60.2%) were performed between 24 and 25.9 weeks' gestation. Delivery at less than 30 weeks' gestation occurred in 36 cases (11.5%) and the overall perinatal mortality rate was 5.4% (17 of 314). The rate of maternal complications was low, including the need for blood transfusion (n = 3, 0.9%) and dehiscence or a thin uterine scar (n = 4, 1.3%). No cases of maternal death were recorded. Fifteen neonates required additional surgical repair of the spinal defect (4.8%) and 63 of 167 infants (37.7%) required a cerebrospinal fluid diversion procedure. Only two of the seven centers reported preliminary experience with fetoscopic IRSBA. CONCLUSIONS: Clinical experience and outcomes were within the expected results reported by the MOMS trial. There is still very limited experience with fetoscopic IRSBA in this part of the world.

Percutaneous fetoscopic spina bifida repair: effect on ambulation and need for postnatal cerebrospinal fluid diversion and bladder catheterization.

OBJECTIVE: A trial comparing prenatal with postnatal open spina bifida (OSB) repair established that prenatal surgery was associated with better postnatal outcome. However, in the trial, fetal surgery was carried out through hysterotomy. Minimally invasive approaches are being developed to mitigate the risks of open maternal-fetal surgery. The objective of this study was to investigate the impact of a novel neurosurgical technique for percutaneous fetoscopic repair of fetal OSB, the skin-over-biocellulose for antenatal fetoscopic repair (SAFER) technique, on long-term postnatal outcome. METHODS: This study examined descriptive data for all patients undergoing fetoscopic OSB repair who had available 12- and 30-month follow-up data for assessment of need for cerebrospinal fluid (CSF) diversion and need for bladder catheterization and ambulation, respectively, from eight centers that perform prenatal OSB repair via percutaneous fetoscopy using a biocellulose patch between the neural placode and skin/myofascial flap, without suture of the dura mater (SAFER technique). Univariate and multivariate logistic regression analyses were used to examine the effect of different factors on need for CSF diversion at 12 months and ambulation and need for bladder catheterization at 30 months. Potential cofactors included gestational age at fetal surgery and delivery, preoperative ultrasound findings of anatomical level of the lesion, cerebral lateral ventricular diameter, lesion type and presence of bilateral talipes, as well as postnatal findings of CSF leakage at birth, motor level, presence of bilateral talipes and reversal of hindbrain herniation. RESULTS: A total of 170 consecutive patients with fetal OSB were treated prenatally using the SAFER technique. Among these, 103 babies had follow-up at 12 months of age and 59 had follow-up at 30 months of age. At 12 months of age, 53.4% (55/103) of babies did not require ventriculoperitoneal shunt or third ventriculostomy. At 30 months of age, 54.2% (32/59) of children were ambulating independently and 61.0% (36/59) did not require chronic intermittent catheterization of the bladder. Multivariate logistic regression analysis demonstrated that significant prediction of need for CSF diversion was provided by lateral ventricular size and type of lesion (myeloschisis). Significant predictors of ambulatory status were prenatal bilateral talipes and anatomical and functional motor levels of the lesion. There were no significant predictors of need for bladder catheterization. CONCLUSION: Children who underwent prenatal OSB repair via the percutaneous fetoscopic SAFER technique achieved long-term neurological outcomes similar to those reported in the literature after hysterotomy-assisted OSB repair. © 2021 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Fetal open spinal dysraphism repair through a mini-hysterotomy: Influence of gestational age at surgery on the perinatal outcomes and postnatal shunt rates.

OBJECTIVE: To analyze the impact of gestational age (GA) at the time of fetal open spinal dysraphism (OSD) repair through a mini-hysterotomy on the perinatal outcomes and the infants' ventriculoperitoneal shunt rates. METHODS: Retrospective study of cases of fetal OSD correction performed from 2014 and 2019. RESULTS: One hundred and ninety women underwent fetal surgery for OSD through a mini-hysterotomy, and 176 (176/190:92.6%) have since delivered. Fetal OSD correction performed earlier in the gestational period, ranging from 19.7 to 26.9 weeks, was associated with lower rates of postnatal ventriculoperitoneal shunting (P: .049). Earlier fetal surgeries were associated with shorter surgical times (P: .01), smaller hysterotomy lengths (P < .001), higher frequencies of hindbrain herniation reversal (P: .003), and longer latencies from surgery to delivery (P < .001). Median GA at delivery was 35.3 weeks. Multivariate binary logistic regression showed that both fetal lateral ventricle-to-hemisphere ratio (%; P < .001; OR: 1.14 [95% CI: 1.09-1.21]) and GA at the time of fetal surgery (P: .016; OR: 1.37 [95% CI: 1.07-1.77]) were independent predictors of postnatal ventriculoperitoneal shunting. CONCLUSION: Fetuses with OSD who were operated on earlier in the gestational interval, which ranged from 19.7 to 26.9 weeks, were less prone to receiving postnatal ventriculoperitoneal shunts.

Clubfoot release in myelodysplasia.

The purpose of this study was to evaluate our experience with treatment of clubfoot in myelomeningocele. We evaluated 45 children with clubfoot surgically treated at a mean age of 36 months. We evaluated each patient's medical record and performed a physical examination. Based on the clinical criteria by de Carvalho Neto, we obtained 61% good results, 26% fair results and 13% poor results. Neither age at surgery nor neurosegmental level have any statistical influence on final outcome. We find that a radical posteromedial release without internal fixation is a satisfactory option for clubfoot with myelodysplasia.

Association between prenatal sonographic findings and post-natal outcomes in 30 cases of isolated spina bifida aperta.

This study presents 30 cases of fetal isolated spina bifida aperta (SBA) to identify prenatal ultrasound findings that could predict the prognosis. Comparisons between surviving patients who had normal (group 1) and abnormal (group 2) post-natal neurological outcomes were made for three different prenatal signs, that is, site of vertebral lesion, presence and degree of ventriculomegaly and presence of talipes. The site of the lesion was the most significant outcome predictor, as high spinal dysraphisms were observed in 2 patients (2/7-28.6%) in group 1 and in 15 patients (15/19-79.0%) in group 2 (p = 0.03). The presence of fetal ventriculomegaly was associated with impaired post-natal neurological development, as it occurred in 4 patients (4/7-57.1%) in group 1 and in 18 patients (18/19-94.7%) in group 2 (p = 0.04). The presence of talipes did not significantly differ between the two groups. Patients with abnormal intellectual outcome (8/26-30.8%) had significantly greater (p = 0.018) lateral ventricle/hemisphere ratios (mean = 0.74, standard deviation = 0.13) than those with normal intellectual development (mean = 0.54, standard deviation = 0.18). Mean post-natal follow-up was at 23 months (standard deviation = 15 months).

Frecuencia y factores de riesgo de las malformaciones congénitas en la maternidad "German Urquidi" 1982-1983 / Frequency and risk factors for congenital malformations at the Obstetric Hospital German Urquidi

Se realiza un estudio epidemiológico prospectivo de malformaciones congénitas en la MGU de Cochabamba, sobre un total de 7409 nacidos vivos y muertos ocurridos en 24 meses desde el 1o de enero de 1982 hasta el 31 de diciembre de 1983, observandose una frecuencia de 62 por diez mil nacimientos, siendo las más frecuentes las malformaciones del Sistema Nervioso Central 11 (14.8 por ciento) seguidas de labio leporino 7 (9.4 por ciento), ano imperforado 6(8.0 por ciento) y pie contrahecho 5(6.7 por ciento): con estas cuatro malformaciones se realiza un enfoque de cohorte para cuatro factores de riesgo, encontrandose una mayor frecuencia de malformaciones en los recien nacidos de bajo peso (- 2500 gr.). Finalmente se hace incapie en la importancia de contar con un sistema de registro de malformaciones congenitas, que tenga una cobertura amplia de ser posible nacional e internacional para asi llegar a detectar picos epidemiologicos en la frecuencia de las malformaciones, resultando entonces facil una investigacion etiologica posterior.