SIRENOMELIA
Abstract
A case of sirenomelia was presented in a new born with perinatal death. The fetus had abnormal appearance of the head and face, single lower extremity, enlarged ears, small eyes and flattened nose. The external genitalia and anus was absent. The lungs were hypoplastic; the kidneys were agenetic. There was a single umbilical artery. A blind pouch was noted at the sigmoid colon and imperforate anus was present. The genitalia was absent; the tubes and fimbria was noted and the uterus could not be well identified. Babygram revealed malformed ischium and pubic bones. Asymmetric femurs, single tibia, and single fibula was noted.
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Van Zalen-Sprock MM, Van Vugt JMG, Van der Harten JJ, Van Geun HP. Early second- trimester diagnosis of sirenomelia. Prenatal Diagnosis 1995;15:171-7.
Nyberg Da, Mahoney BS, Pretorius DH. Diagnostic ultrasound of fetal anomalies. Text and atlas. Chicago: Year book medical publishers 1989;187-190.
Taybi H, Lachman RS. Radiology of syndromes, metabolic disorders, and skeletal dysplasias. 3rd ed. Chicago: Year book medical publishers, inc 1990:301.
Duhamel B. From the mermaid to anal imper- foration: The syndrome of caudal regression. Arch Dis Child 1961:36:152.
Adra A, Cordero D, Mjides A, Yasin S, Salman F, O'Sullivan MJ. Caudal regression syndrome: Etiopathogenesis, prenatal diagnosis, and peri- natal management. Obstetrical and Gynecolo- gical Survey 1994;49:508-15.
Perrot LJ, Williamson S, Jimenez JF. The caudal regression syndrome in infants of diabetic mothers. Ann Clin Lab Sci 1987;17:211.
Betti RJ, Traisman HS,. Sirenomelia: A spec- trum of related syndromes. Clin Pediatr 1971;238:10.
Pedersen LM, Tygstrup I, Pedersen J. Con- genital malformations in newborn infants of diabetic women: Correlation with maternal diabetic complications. Lancet 1964;1:1124.
Rusnak SL, Driscoil SG. Congenital spinal anomalies in infants of diabetic mothers. Pedia- trics 1965;36:989.
Passarge E, Lenz W. Syndrome of caudal re- gression in infants of diabetic mothers: Obser- vation of further cases. Pediatrics 1966;37:672.
Gabbe SG. Congenital malformations in infants of diabetic mothers. Obstet Gynecol Surv 1977;125:32.
Abraham E. Sacral agenesis with associated anomalies (caudal regression syndrome). Clin Orthop 1979;145:168.
Ziva SS, Golding J, Drummond JC, et al. The relation of the fat-soluble factor to rickets growth in pigs. Biochem J 1921;427:15.
Baldwin WM. The action of ultra-violet rays upon the frog's egg. The artificial production of spina bifida. Anat Rec 1915;365-9.
Warkany J, Nelson RL, Schraffenberger E. Congenital malformations induced in rats by maternal nutritional deficiency. Malformations of the extremities. J Bone Joint Surg 1943;261:25.
Kucera J. Exposure to fat solvents. A possible cause of sacral agenesis in man. J Pediatr 1968;72:857.
Welch JP, Aterman K. The syndrome of caudal dysplasia: A review, including etiologic consi- derations and evidence of heterogeneity. Pediatr Pathol 1984;2:313.
Hoyme HE. The pathogenesis of sirenomelia: An editorial comment. Teratology 1988:38:485.
Kampmeier OF. On sireniform monsters, with a consideration of the causation and the predo- minance of the male sex among them. Anat Rec 1927:34:365.
Stevenson RE, Jones KL, Phelan MC, et al. Vascular steal: The pathogenetic mechanism producing sirenomelia and associated defects of the viscera and soft tissues. Pediatrics 1986;78:451.
Stocker JT, Heifetz FSA. Sirenomelia. Perspect Pediatr Pathol 1987;10:7
Davies J, Chazen E, Nance WE. Symmelia in one of monozygotic twins. Teratology 1971;4:367.
White RI, Klauber GT. Sacral agenesis. Analysis of 22 cases. Urology 1976;8:521.
Blumel J, Evans EB, Eggers GWN. Partial and complete agenesis or malformation of the sacrum with associated anomalies. J Bone Joint Surg 1959;41A:497.
Sparnon AL, Ahmed S. Urological anomalies in the caudal regression syndrome. Aug NZ J Surg 1984;54:365.
Sirtori M Ghidini A, Romero R, et al. Prenatal diagnosis of sirenomelia. J Ultrasound Med 1989;8:83.
Twickler D, Budorick N, Pretorius D, et al. Caudal regression versus sirenomelia: Sono- graphic clues. J Ultrasound Med 1993;12:323.
Quetal TA, Mejides AA, Salman FA, et al. Amnioinfusion: An aid in the ultrasonographic evaluation of severe oligohydramnios in preg- nancy. Am J Obstet Gynecol 1992;167:333.
Savader SJ, Savader BL, Clark RA. Sireno- melia without Potter syndrome: MR charac- teristics. J Comput Assist Tomogr 1989;13:689.
Sarnat HB, Case ME, Graviss R. Sacral age- nesis. Neurologic and neuropathologic features. Neurology 1976;26:1124.
Chenoweth CK, Kellogg SJ, Abu-Yousef M. Antenatal sonographic diagnosis of sirenomelia. JCU 1991;19:167.
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