Bilateral absence of the pectoralis major muscle with accompanying abnormalities of shoulder muscles has been reported in patients without Poland anomaly (PA). However, symmetric absence of pectoralis major muscles, hypoplasia of breasts and nipples with symmetric chest wall deformity and bilateral hand anomaly has not previously been reported. A 6-year-old girl with bilateral absence of pectoralis major muscles and hand involvement and symmetric chest wall deformity is, to our knowledge, the first known case of bilateral Poland anomaly.
The rabbit fetus is one of the most commonly used animal models in experimental studies investigating fetal organ development. However, there is no detailed information about normal growth of organs of rabbit fetuses in English language literature. Fetal rabbits were studied in the second half of gestation between 18th and 30th days. Amniotic fluid volume, body mass (BM), lung, heart and liver masses (LM, HM, LiM), lung and thorax volumes (LV, TV) were determined and LM/BM, HM/BM, LiM/BM, TV/BM and LV/TV ratios were calculated. Additionally fetal lungs were evaluated histologically, BM, LM, HM, LiM and LV were increased until 27th gestational day and then remained unchanged. TV was always increased between 18th gestational day and term. The lung maturation was almost completed in the 27th-28th gestational days. Therefore, BM, LM, HM, LiM and LV are the parameters that can be used to evaluate normal fetal growth between 18th and 27th gestational days. TV seems to be the predictive parameter for evaluation of normal fetal growth during the second half of gestation in rabbit fetuses; 20th and 27th days of gestation are more appropriate for experiments to evaluate lung maturation.
An in-utero experimental study was performed to evaluate the effects of intrauterine vascular compromise on further development of corpus spongiosum and male urethra. Thirty time-mated pregnant New Zealand white rabbits on their twenty-third day of gestation were used. Deterioration of the blood supply of the corpus spongiosum and urethra was attempted by electrocauterizing the perineum adjacent to the root of the phallus without damaging the corpus spongiosum and urethra, under the operating microscope. A bipolar cautery was carried out using a specially designed forceps having 100 microns interspaced fixed tips. Five experimental and seven control live male litters were delivered at term by Cesarean sections. Their anogenital regions were examined histopathologically. While the corpus spongiosum extended as long as the corpus cavernosum to the tip of phallus in control litters, the corpus spongiosum ended under the skin in one of the experimental group and in the other four was shorter than the corpus cavernosum. In the latter group, no histopathological evidence of tissue destruction which might be related to electrocauterization, was found. Similar to the pathogenesis encountered in intestinal atresia, a vascular insult which might occur even after the completion of organogenesis, may affect the fate of the corpus spongiosum and urethra. Localized ischemia resulting from local vascular insults may explain, at least in theory, the pathogenesis of some congenital anomalies of corpus spongiosum and urethra such as hypospadias.
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