Myelomeningocele sac associated with worse lower-extremity neurological sequelae: evidence for prenatal neural stretch injury?

OBJECTIVE: To determine whether the presence of a myelomeningocele (MMC) sac and sac size correlate with compromised lower-extremity function in fetuses with open spinal dysraphism. METHODS: A radiology database search was performed to identify cases of MMC and myeloschisis (MS) diagnosed prenatally in a single center from 2013 to 2017. All cases were evaluated between 18 and 25 weeks. Ultrasound reports were reviewed for talipes and impaired lower-extremity motion. In MMC cases, sac volume was calculated from ultrasound measurements. Magnetic resonance imaging reports were reviewed for hindbrain herniation. The association of presence of a MMC sac and sac size with talipes and impaired lower-extremity motion was assessed. Post-hoc analysis of data from the multicenter Management of Myelomeningocele Study (MOMS) randomized controlled trial was performed to confirm the study findings. RESULTS: In total, 283 MMC and 121 MS cases were identified. MMC was associated with a lower incidence of hindbrain herniation than was MS (80.9% vs 100%; P < 0.001). Compared with MS cases, MMC cases with hindbrain herniation had a higher rate of talipes (28.4% vs 16.5%, P = 0.02) and of talipes or lower-extremity impairment (34.9% vs 19.0%, P = 0.002). Although there was a higher rate of impaired lower-extremity motion alone in MMC cases with hindbrain herniation than in MS cases, the difference was not statistically significant (6.6% vs 2.5%; P = 0.13). Among MMC cases with hindbrain herniation, mean sac volume was higher in those associated with talipes compared with those without talipes (4.7 ± 4.2 vs 3.0 ± 2.6 mL; P = 0.002). Review of the MOMS data demonstrated similar findings; cases with a sac on baseline imaging had a higher incidence of talipes than did those without a sac (28.2% vs 7.5%; P = 0.007). CONCLUSIONS: In fetuses with open spinal dysraphism, the presence of a MMC sac was associated with fetal talipes, and this effect was correlated with sac size. The presence of a larger sac in fetuses with open spinal dysraphism may result in additional injury through mechanical stretching of the nerves, suggesting another acquired mechanism of injury to the exposed spinal tissue. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Morphometric changes in talus of club foot A gross observation in human foetuses / Cambios morfométricos en talus del pie zambo, una observación macroscópica en fetos humanos

Both the feet of six human foetuses of different age groups having unilateral club feet, were dissected for morphological study. Six morphometric parameters considered for comparing gross anatomical changes in normal and deformed feet, were 1-Maximum length of the talus, 2-Longitudinal dimension of head of talus, 3-Anterior trochlear breadth, 4-Maximum medial talar height, 5-Talar neck and calcaneal angle, 6-Talocalcaneal angle. All the foetuses with congenital club feet have almost similar deformity of foot skeleton. The gross anomalies observed were the smaller size of club foot talus and increased medial and planter deviation of a stunted, misshapen head and neck region. A medial plantar subluxation of the navicular bone with a consequent deformity of the articular facets of the talar head was also observed. Uniformity and consistency of anatomical abnormalities were striking features in present study.

Para su estudio morfológico fueron disecados ambos pies de seis fetos humanos de distintas edades, uno de los pies era zambo. Seis parámetros morfométricos fueron considerados para la comparación de graves alteraciones anatómicas en los pies normales y deformes; estos fueron: 1. Longitud máxima del talus, 2. Dimensión longitudinal de la cabeza del talus, 3. Ancho troclear anterior, 4. Altura medial máxima del talus, 5. Cuello talar y ángulo calcáneo, 6. Ángulo talocalcáneo. Todos los fetos con pie zambo congénito tienen una deformidad similar del esqueleto del pie. Las anomalías graves observadas fueron el menor tamaño del talus del pie zambo, aumento de la desviación media y retraso en el crecimiento plantar, deformación de la cabeza y región del cuello talar. También se observó una subluxación medial plantar del hueso navicular, con un consecuente deformidad de las facetas articulares de la cabeza del talus. La uniformidad y consistencia de las anomalías anatómicas fueron los rasgos más llamativos en este estudio.

Pseudoapêndice caudal associado a pé torto congênito: relato de caso / Caudal pseudo tail associated with congenital clubfoot: case report

O apêndice caudal é uma rara anomalia congênita, localizada na região lombossacral. Classifica-se, segundo Dao e Netsky, em pseudoapêndice e apêndice caudal verdadeiro,com implicações prognósticas e terapêuticas.O apêndice caudal pode estar associado a outras anormalidades congênitas, sendo necessários exame físico detalhado e exames de imagem para diagnóstico e tratamento precoce das doenças concomitantes. Os autores relatam um caso de pseudoapêncice caudal lombossacro (fibrolipoma congênito), associado a pé torto congênito, em uma criança submetida a tratamento cirúrgico excisional do pseudoapêndice. Fez-se revisão da literatura sobre casos semelhantes e não foi encontrada descrição da associação com pé torto congênito.