Defectos del tubo neural y de labio y paladar hendido: un estudio morfológico / Neural tube defects, cleft lip and palate: a morphological study

El objetivo del trabajo fue realizar una evaluación morfológica externa de recién nacidos (RN) y lactantes con diagnóstico de defectos de tubo neural (DTN) y labio hendido c/s paladar hendido (LH c/s PH) para determinar el tipo de defecto, ubicación, extensión, clasificación y evaluar la proporción de RN con retardo del crecimiento intauterino (RCIU) y lactantes desnutridos. Estudio descriptivo. Se estudiaron 36 niños desde su nacimiento hasta los 12 meses de edad; 20 con DTN y 16 con LH c/s PH. El estudio se realizó en cinco hospitales de la ciudad de Chihuahua, México. Se hizo una evaluación morfológica externa y antropometría de los niños. Los DTN se clasificaron como lesiones abiertas y cerradas, como defectos altos o bajos, según el modelo de sitios múltiples y por la CIE-10. Los LH c/s PH se clasificaron como unilaterales o bilaterales, completos e incompletos y como aislados o múltiples. Se determinó RCIU en los RN y desnutrición en los lactantes. El análisis estadístico se realizó con el paquete STATA 8.0 para Windows. Se estudiaron 20 casos de DTN; 3 lactantes y 17 RN. De LH c/s PH fueron 8 lactantes y 8 RN. En cuanto a los DTN, 60 por ciento fueron mielomeningocele y correspondían a lesiones abiertas. El 85 por ciento se localizaron a nivel alto. En el modelo de cierres múltiples, el Z1 fue el 80 por ciento. Los LH c/s PH más frecuentes fueron aquellos con hendidura completa (50 por ciento). El 35 por ciento de los RN con DTN tuvieron RCIU y el 67 por ciento de los lactantes presentaron desnutrición. Es importante conocer los mecanismos del desarrollo de las anomalías congénitas ya que esto permite precisar el momento en que ocurrió la falla y permite estudiar los factores predisponentes, con lo cual se puede ofrecer asesoramiento genético para una posible prevención.

The objective of this study was to perform an external morphological evaluation of newborn (NB) and lactating children (LC) with diagnosis of neural tube defects (NTD) and cleft lip c/s palate (CL/s PH) to determine the type of defect, location, extent, classification and assess the proportion of infants with intrauterine growth retardation (IUGR) and malnourished infants. A descriptive study in 36 children from birth to 12 months of age , 20 with NTD and 16 with LH c / s PH was carried out. The study was conducted in five hospitals in the city of Chihuahua, Mexico. An external morphological assessment and anthropometry of children were performed. The DTN lesions were classified as open and closed, as defects high or low, depending on the model of multiple sites and ICD-10. The LH c / s PH were classified as unilateral or bilateral, complete or incomplete, and as isolated or multiple. IUGR was determined in the RN and malnutrition in infants. A statistic analysis was made with STATA 8.0 for Windows. We studied 20 cases of NTDs, 3 LCs and 17 RN. LH c/s PH were 8 LC and 8 RN. The DTN, 60 percent were myelomeningocele and corresponded to open lesions. Eighty five percent were located at high level. In the model of multiple closures, the Z1 was 80 percent. The LH c/s PH were more frequent with complete cleft (50 percent). The 35 percent of newborns with NTD had IUGR and 67 percent of LC had malnutrition. It is important to understand the mechanisms of development of congenital anomalies as this allows to specify the time the fault occurred and to study the underlying diseases to offer genetic counseling for possible prevention.

Morphogenesis of the lateral nasal wall from 6 to 36 weeks.

This research describes the development of the lateral nasal wall, the description of which will allow a better comprehension of its anatomic complexity. One hundred embryos and fetuses from the sixth to thirty-sixth weeks of morphologic age were studied. The seventh week shows the first buds of the three turbinates. At the ninth week the precartilaginous nucleus of the inferior turbinate is observed. Likewise, at the tenth week the uncinate process appears, and the invagination of the epithelium begins the formation of the infundibulum and the maxillar sinus. At the fourteenth week the cartilaginous nasal capsule is present. The epithelium is invaginated, starting the formation of the ethmoid cells. During the seventeenth week of development the invagination of the mucus has invaded the maxillar bone, which constitutes the maxillar sinus. At the thirty-sixth week the lateral nasal wall is well developed. In 23 fetuses the supreme turbinate (fourth) was found. Although the length of the lower three turbinates increased progressively and proportionally in intrauterine life without differences between the sexes, the fourth remains invariably at an average length of 5 mm from the fourteenth to the thirty-sixth and was present in 65% of male fetuses.

[Frequency of external congenital abnormalities in abortions]. / Frecuencia de malformaciones congénitas externas en abortos.

The purpose of this study was to calculate the incidence of external birth defects found in 1,650 aborted fetuses studied from September 1978 to February 1983 at the Department of Gynecology and Obstetrics of the University Hospital "Dr. José Eleuterio González" of the U.A.N.L. Medical School. Ninety five of fetuses had external birth defects and 85 had abnormalities in annexes. The Fisher exact test was applied to find the relationship between these abnormalities; no relationship was found. 67.4% had only one birth defect; 32.1% showed several defects. Those defects which were lethal constituted 67%, the majority being of the central nervous system. Defects found in the abdominal wall took second place in frequency followed by abnormalities in either extremity and ear defects. The birth defects seen in early gestational ages differ from those seen in live newborn babies. It is important to carry out morphological and teratological studies, not only in newborns but also during the embrion and fetal periods.