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Workshop, SBoD Work Group – ERN ITHACA, eUROGEN, ERKnet – 14-15 November 2024 – Paris APHP
This two-day workshop, held from November 14-15, offers an in-depth exploration of current research and advances in genetic and perinatal…
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SBoD webinar on the new classification of dysraphisms
This is a series of webinars on spinal dysraphism organised jointly by ERN eUROGEN, OMNI-NET Ukraine and the International Federation…
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SBoD: New article on prenatal diagnosis of open dysraphism
The European SBoD (Spina Bifida and other Dysraphisms) working group, co-chaired by Professor Jean-Marie Jouannic, has published a new article…
Our publications
Guilbaud, Lucie; Carreras, Elena; Garel, Catherine; Maiz, Nerea; Dhombres, Ferdinand; Deprest, Jan; and, Jean-Marie Jouannic
In: Prenat Diagn, 2024, ISSN: 1097-0223.
@article{pmid38898590,
title = {Proposal for standardized prenatal assessment of fetal open dysraphisms by the European reference network for Intellectual disability, TeleHealth, Autism and Congenital Anomalies (ITHACA) and eUROGEN},
author = {Lucie Guilbaud and Elena Carreras and Catherine Garel and Nerea Maiz and Ferdinand Dhombres and Jan Deprest and Jean-Marie Jouannic and },
doi = {10.1002/pd.6618},
issn = {1097-0223},
year = {2024},
date = {2024-06-01},
journal = {Prenat Diagn},
abstract = {Open dysraphisms, that is, myelomeningocele and myeloschisis, are rare diseases associated with a risk of severe disability, including lower limb motor and sensory deficiency, sphincter deficiency, and potential intellectual deficiency. Open dysraphism is diagnosed in Europe in 93.5% of cases. In case of suspicion of fetal open dysraphism, a detailed fetal morphologic assessment is required to confirm the diagnosis and exclude associated structural anomalies, as well as genetic assessment. In case of isolated fetal open dysraphism, assessment of prognosis is based on fetal imaging including the level of the lesion, the presence or not of a sac, the presence and nature of intra cranial anomalies, and the anatomical and functional evaluation of the lower extremities. Based on these biomarkers, a personalized prognosis as well as comprehensive information about prenatal management alternatives will allow parents to decide on further management options. Standardization of prenatal assessment is essential to compare outcomes with benchmark data and make assessment of surgical innovation possible. Herein, we propose a protocol for the standardized ultrasound assessment of fetuses with isolated open dysraphism.},
keywords = {},
pubstate = {published},
tppubtype = {article}
}
Open dysraphisms, that is, myelomeningocele and myeloschisis, are rare diseases associated with a risk of severe disability, including lower limb motor and sensory deficiency, sphincter deficiency, and potential intellectual deficiency. Open dysraphism is diagnosed in Europe in 93.5% of cases. In case of suspicion of fetal open dysraphism, a detailed fetal morphologic assessment is required to confirm the diagnosis and exclude associated structural anomalies, as well as genetic assessment. In case of isolated fetal open dysraphism, assessment of prognosis is based on fetal imaging including the level of the lesion, the presence or not of a sac, the presence and nature of intra cranial anomalies, and the anatomical and functional evaluation of the lower extremities. Based on these biomarkers, a personalized prognosis as well as comprehensive information about prenatal management alternatives will allow parents to decide on further management options. Standardization of prenatal assessment is essential to compare outcomes with benchmark data and make assessment of surgical innovation possible. Herein, we propose a protocol for the standardized ultrasound assessment of fetuses with isolated open dysraphism.
Langlais, Tristan; Skalli, Wafa; du Cluzel, Xavier; Mainard, Nicolas; George, Samuel; Gajny, Laurent; Vialle, Raphael; Dubousset, Jean; Vergari, Claudio
In: Spine Deform, vol. 12, no. 3, pp. 689–697, 2024, ISSN: 2212-1358.
@article{pmid38347377,
title = {Spinal axial torque assessment after surgical correction in adolescent idiopathic scoliosis: a new approach to 3D barycentremetry and mass distribution based on biplanar radiographs},
author = {Tristan Langlais and Wafa Skalli and Xavier du Cluzel and Nicolas Mainard and Samuel George and Laurent Gajny and Raphael Vialle and Jean Dubousset and Claudio Vergari},
doi = {10.1007/s43390-023-00816-5},
issn = {2212-1358},
year = {2024},
date = {2024-05-01},
journal = {Spine Deform},
volume = {12},
number = {3},
pages = {689--697},
abstract = {PURPOSE: Barycentremetry in adolescent idiopathic scoliosis (AIS) allows the distribution of masses and their loading of the spine to be studied. In particular, the axial torque on the spine has been studied in AIS, but not after surgical correction. Spinal axial torque was studied in AIS before and after surgery.nnMETHODS: All AIS (Lenke 1 and 3) who underwent posterior spinal fusion surgery at our center in 2019 were included retrospectively. AIS underwent frontal and sagittal biplanar radiographs in the free-standing position before surgery, 4 months after surgery, and at the last follow-up. Their spine and external envelope were reconstructed with validated methods. Spinal axial torque at the apex and the upper and lower end vertebra was calculated. Finally, the preoperative and postoperative values were compared to a previously published reference corridor for asymptomatic subjects.nnRESULTS: Twenty-nine patients were included (54 ± 11° Cobb angle, 15 ± 2 years old at surgery). The surgical procedure decreased the Cobb angle by 36° ± 11° and decreased the spinal axial torque at the upper end vertebra by 2.5 N/m (95% CI = [1.9; 3]; p < 0.001), at the apex by 0.6 N/m (95% CI = [0.4; 1]; p = 0.004), at the lower end vertebra by 2 N/m (95% CI = [1.5; 2.8]; p < 0.001). Compared to 95th percentile of torque, which was previously evaluated in asymptomatic subjects, more than 90% of patients had higher values at the upper and lower end vertebrae before surgery. Postoperatively, 62% of patients still had higher torque at the upper end vertebra than asymptomatic subjects, while only 38% patients showed abnormal values at the lower junction.nnCONCLUSION: Results of this study confirm that AIS patients show abnormally high spinal axial torque, especially at the end vertebrae, and that this parameter is normalized postoperatively for only a small number of patients.},
keywords = {},
pubstate = {published},
tppubtype = {article}
}
PURPOSE: Barycentremetry in adolescent idiopathic scoliosis (AIS) allows the distribution of masses and their loading of the spine to be studied. In particular, the axial torque on the spine has been studied in AIS, but not after surgical correction. Spinal axial torque was studied in AIS before and after surgery.nnMETHODS: All AIS (Lenke 1 and 3) who underwent posterior spinal fusion surgery at our center in 2019 were included retrospectively. AIS underwent frontal and sagittal biplanar radiographs in the free-standing position before surgery, 4 months after surgery, and at the last follow-up. Their spine and external envelope were reconstructed with validated methods. Spinal axial torque at the apex and the upper and lower end vertebra was calculated. Finally, the preoperative and postoperative values were compared to a previously published reference corridor for asymptomatic subjects.nnRESULTS: Twenty-nine patients were included (54 ± 11° Cobb angle, 15 ± 2 years old at surgery). The surgical procedure decreased the Cobb angle by 36° ± 11° and decreased the spinal axial torque at the upper end vertebra by 2.5 N/m (95% CI = [1.9; 3]; p < 0.001), at the apex by 0.6 N/m (95% CI = [0.4; 1]; p = 0.004), at the lower end vertebra by 2 N/m (95% CI = [1.5; 2.8]; p < 0.001). Compared to 95th percentile of torque, which was previously evaluated in asymptomatic subjects, more than 90% of patients had higher values at the upper and lower end vertebrae before surgery. Postoperatively, 62% of patients still had higher torque at the upper end vertebra than asymptomatic subjects, while only 38% patients showed abnormal values at the lower junction.nnCONCLUSION: Results of this study confirm that AIS patients show abnormally high spinal axial torque, especially at the end vertebrae, and that this parameter is normalized postoperatively for only a small number of patients.
Athiel, Yoann; Jouannic, Jean-Marie; Mauffré, Vincent; Dehan, Coralie; Adam, Clovis; Blot, Stéphane; Lallemant, Pauline; Denis, Timothé De Saint; Larghero, Jérôme; Nasone, Justine; Guilbaud, Lucie
In: BJOG, vol. 131, no. 6, pp. 759–767, 2024, ISSN: 1471-0528.
@article{pmid37492999,
title = {Allogenic umbilical cord-derived mesenchymal stromal cells improve motor function in prenatal surgical repair of myelomeningocele: An ovine model study},
author = {Yoann Athiel and Jean-Marie Jouannic and Vincent Mauffré and Coralie Dehan and Clovis Adam and Stéphane Blot and Pauline Lallemant and Timothé De Saint Denis and Jérôme Larghero and Justine Nasone and Lucie Guilbaud},
doi = {10.1111/1471-0528.17624},
issn = {1471-0528},
year = {2024},
date = {2024-05-01},
journal = {BJOG},
volume = {131},
number = {6},
pages = {759--767},
abstract = {OBJECTIVE: To investigate the effects of an adjuvant allogenic umbilical cord mesenchymal stromal cell (UC-MSC) patch applied during fetal surgery on motor and sphincter function in the ovine MMC model.nnDESIGN: MMC defects were surgically created at 75 days of gestation and repaired 14 days later.nnPOPULATION: Ovine MMC model: fetal lambs.nnMETHODS: We compared lambs that received a UC-MSC patch with a control group of lambs that received an acellular patch.nnMAIN OUTCOME MEASURES: Clinical neurological assessment was performed at 2 and 24 hours of life and included determination of the Sheep Locomotor Rating scale (SLR), which has been validated in the ovine MMC model. Electrophysical examinations, spine scans and histological analyses were also performed.nnRESULTS: Of the 13 operated lambs, nine were born alive: five had of these had received a UC-MSC patch and four an acellular patch. At 24 hours of life, lambs in the UC-MSC group had a significantly higher score (14 versus 5, P = 0.04). Amyotrophy was significantly more common in the control group (75% versus 0%, P = 0.02). All the lambs in the control group and none of those in the UC-MSC group were incontinent. No significant differences were observed between the UC-MSC and control groups in terms of the presence of spontaneous EMG activity, nerve conduction or spinal evoked potentials. In the microscopic examination, lambs in the UC-MSC group had less fibrosis between the spinal cord and the dermis (mean thickness, 453 versus 3921 μm, P = 0.03) and around the spinal cord (mean thickness, 47 versus 158 μm, P < 0.001). Examination of the spinal cord in the area of the MMC defect showed a higher large neuron density in the UC-MSC group (14.5 versus 5.6 neurons/mm, P < 0.001). No tumours were observed.nnCONCLUSIONS: Fetal repair of MMC using UC-MSC patches improves motor and sphincter function as well as spinal preservation and reduction of fibrosis.},
keywords = {},
pubstate = {published},
tppubtype = {article}
}
OBJECTIVE: To investigate the effects of an adjuvant allogenic umbilical cord mesenchymal stromal cell (UC-MSC) patch applied during fetal surgery on motor and sphincter function in the ovine MMC model.nnDESIGN: MMC defects were surgically created at 75 days of gestation and repaired 14 days later.nnPOPULATION: Ovine MMC model: fetal lambs.nnMETHODS: We compared lambs that received a UC-MSC patch with a control group of lambs that received an acellular patch.nnMAIN OUTCOME MEASURES: Clinical neurological assessment was performed at 2 and 24 hours of life and included determination of the Sheep Locomotor Rating scale (SLR), which has been validated in the ovine MMC model. Electrophysical examinations, spine scans and histological analyses were also performed.nnRESULTS: Of the 13 operated lambs, nine were born alive: five had of these had received a UC-MSC patch and four an acellular patch. At 24 hours of life, lambs in the UC-MSC group had a significantly higher score (14 versus 5, P = 0.04). Amyotrophy was significantly more common in the control group (75% versus 0%, P = 0.02). All the lambs in the control group and none of those in the UC-MSC group were incontinent. No significant differences were observed between the UC-MSC and control groups in terms of the presence of spontaneous EMG activity, nerve conduction or spinal evoked potentials. In the microscopic examination, lambs in the UC-MSC group had less fibrosis between the spinal cord and the dermis (mean thickness, 453 versus 3921 μm, P = 0.03) and around the spinal cord (mean thickness, 47 versus 158 μm, P < 0.001). Examination of the spinal cord in the area of the MMC defect showed a higher large neuron density in the UC-MSC group (14.5 versus 5.6 neurons/mm, P < 0.001). No tumours were observed.nnCONCLUSIONS: Fetal repair of MMC using UC-MSC patches improves motor and sphincter function as well as spinal preservation and reduction of fibrosis.
