ABSTRACT
Perosomus elumbis is an occasional congenital anomaly of cattle, swine, sheep, and dogs with unknown etiology. This congenital abnormality occurs in both sexes. A dead Holstein calf characterized by musculoskeletal and external genitalia abnormalities was referred to the large animal hospital of University of Tehran. Radiographic evaluation and subsequent dissection revealed that the vertebral column was truncated at the level of first lumbar vertebra [L1]. Moreover, L2-L5, sacrum and coccygeal vertebrae were absent. The dorsum of the lumbosacral region contained only soft tissues. Urogenital tract was incomplete, and it contained agenesis of the ovaries, uterine tubes, cervix, and vulva concurrent with unilateral umbilical artery agenesis. Small and large intestine contained blind-ended sacs. No testes, scrotum, and penis were found. The intact ureter was attached to a thin-walled fluid fill sac. The laboratory finding showed that the pH of the fluid was 6 and contained hemoglobin, white blood cells, bacteria, a few red blood cells, oxalate crystalline, and epithelial cells. It was concluded that the collected fluid was urine. This is the first report of perosomus elumbis in a Holstein calf having a lot of visceral abnormalities in Iran
Subject(s)
Animals , Viscera/abnormalities , Cattle , Congenital AbnormalitiesABSTRACT
Congenital cutis laxa is a rare hereditary heterogeneous group of disorder of elastic tissue. It is characterized by abnormally loose skin with a reduction in elastic tissue throughout the dermis. Depending on the mode of transmission and phenotypic expression, congenital cutis laxa has been classified in four types: autosomal dominant; autosomal recessive: types I and II and X-linked recessive. We report a new born girl with the autosomal recessive form of congenital cutis laxa associated with visceral abnormalities. The out come for this form was fatal. The most molecular basis for CCL were known but the therapeutic options are still limited
Subject(s)
Humans , Female , Cutis Laxa/diagnosis , Elastic Tissue/pathology , Viscera/abnormalitiesABSTRACT
Se realizó un estudio retrospectivo y descriptivo con 207 pacientes de todas las edades operados en el Servicio de Cirugía General y Pediátrica en el Hospital Militar de San Cristóbal, a quienes se les practico Herniplastia en la pared abdominal anterior. Durante el periodo de dieciocho meses comprendidos desde mayo 2005 a octubre 2006. Más de la tercera parte de los pacientes tenían entre 16 y 30 años de edad, con predominio del sexo masculino, en una relación 2:1 a excepción de la Crural. El mayor número de cirugía de urgencia se realizó en personas mayores de 76 años. La hernia Umbilical fue el diagnóstico operatorio más frecuente (52.17 por ciento). La Hernia inguinal fue la segunda en frecuencia y constituyo la asociada con mayor relevancia. La Epigástrica predominó ante la Crural. La mortalidad por complicación fue nula. Cerca del 4 por ciento de los pacientes fue intervenido de emergencia. Por lo que concluimos que nuestros resultados en los diferentes tipos de hernias estudiados no escapan de los observados en las estadísticas nacionales e internacionales.
Subject(s)
Humans , Male , Female , Magnetic Resonance Spectroscopy/methods , Magnetic Resonance Spectroscopy , Hernia/classification , Hernia/diagnosis , Hernia/pathology , Abdominal Wall/anatomy & histology , Ultrasonography , Physical Examination/methods , Fascia/growth & development , Fascia/injuries , Muscle Strength , Viscera/abnormalitiesABSTRACT
A four-month-old female baby presented with cyanosis and respiratory distress. A provisional diagnosis of congenital posterolateral diaphragmatic hernia was made but on exploration there was a defect in the septum transversum along with features of Ivemark syndrome - asplenia with visceroatrial heterotaxia, malrotation and pancreatic divisum - an association not yet reported in literature. The child did well after operative correction of the hernia. Echocardiography showed situs inversus with dextrocardia with double outlet right ventricle, atrial septal defect, ventricular septal defect, patent ductus arteriosus and pulmonary stenosis.
Subject(s)
Abnormalities, Multiple , Female , Heart Defects, Congenital , Humans , Infant , Pancreas/abnormalities , Situs Inversus , Spleen/abnormalities , Syndrome , Viscera/abnormalitiesABSTRACT
Se presenta un caso de síndrome de Brida amniótica que es una malformación congénita caracterizada por la formación de bandas fibrosas derivadas del amnios y que comprimen y estrangulan partes fetales especialmente cráneoencefálicas y de las extremidades. La etiopatogenia es desconocida y se descarta la participación de factores genéticos. Las formas menores son susceptibles de fetocopia y compatibles con la sobrevida. Las formas severas son incompatibles con la vida.
Subject(s)
Male , Humans , Female , Pregnancy , Infant, Newborn , Limb Deformities, Congenital/etiology , Amniotic Band Syndrome/diagnosis , Amniotic Band Syndrome/physiopathology , Craniofacial Abnormalities/etiology , Umbilical Cord/abnormalities , Abdominal Wall/abnormalities , Amniotic Band Syndrome/complications , Amniotic Band Syndrome/epidemiology , Viscera/abnormalitiesSubject(s)
Humans , Male , Teratology , Cardiovascular System/abnormalities , Viscera/abnormalities , Spleen/abnormalitiesABSTRACT
The authors studied in a retrospective manner one case of Glanzmann thrombasthenia clinically moderated which is characterized by its association in one hand to visceral malformations and in the other hand by its type II. The relationship between Glanzmann's disease and the visceral malformations is not known in the literature. Glanzmann's thrombastenia type II is very rare, 15 to 20% of the cases according to some authors
Subject(s)
Viscera/abnormalitiesABSTRACT
Conjoined twins show varying degree of conjoining in either facing or side-by-side fashion. Cephalothoracopagus janiceps is a prototype of facing anomaly in which the two bodies demonstrated a cross symmetry to the midline, that is axial symmetry. Interfacial and intersternal lines crossed at a right angle and no abnormality of situs was associated. Dicephalus dipus dibrachius is a case of side-by-side union, in which the bodies facing nearly the same direction were symmetrical to the middle sagittal plane. Abnormal situs of one was always associated. Other types of conjoined twins as thoracopagus lie between the two extremes of facing and side-by-side union. The three dimensional architectures of the organs in each type would be explained using cross sectional figures of skull, thorax and pelvis. Although the facing twins share the internal organs without fusion, the organs in the side-by-side component are fused with modification of the situs. We postulate sixteen pairs of situs and four manners of division for the explanation of the midline organs and the presence of a dominant co-twin. The splenic locations in a given cardiopulmonary situs are evaluated for the appraisal and applicability of these hypotheses.