DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20192792

A case of diabetic fetopathy: caudal regression syndrome and associated anomalies

Somnath Pal, Syamal Kumar Sardar

Abstract


Uncontrolled periconceptional diabetes in mother  can give rise to severe malformation in the offspring. Author describe a case of  diabetic fetopathy  presenting as a case of  Caudal regression syndrome along with cardiovascular, gastrointestinal and genitourinary anomaly. Though most cases of Caudal regression syndrome are sporadic, it is the most specific lesion of diabetic fetopathy. Clinical presentation varies depending on the severity of the neurological lesion along with the presence of other anomalies, most commonly genitourinary malformation. The index case described here had a type IV lumbosacral agenesis with severe bilateral motor and sensory deficits along with D-Transposition of great arteries and Pyloric atresia. Such cases arise due to inadequate prenatal care of diabetic mother, which is not uncommon in developing country like India.


Keywords


Caudal regression syndrome, Diabetes, Fetopathy, Lumbosacral agenesis

Full Text:

PDF

References


Boulas MM. Recognition of caudal regression syndrome. Adv Neonatal Care. 2009;9(2):61-9

Stroustrup A, Grable I, Levine D. Case 66: Caudal regression syndrome in the fetus of a diabetic mother. Radiology. 2004;230(1):229-33.

Adra A, Cordero D, Mejides A, Yasin S, Salman F, O'Sullivan MJ. Caudal regression syndrome: Etiopathogenesis, prenatal diagnosis and perinatal management. Obstet Gynecol surv. 1994;49(7):508-16.

Belloni E, Martucciello G, Verdenio D. Involvement of HLXB9 homeobox gene in Currarino syndrome. Am J Hum Genet. 2000;66(1):312-9.

Chen CP, Chen CY, Lin CY, Shaw SW, Wang W, Tzen CY. Prenatal diagnosis of concomitant alobar holoprosencephaly and caudal regression syndrome associated with maternal diabetes. Prenat Diagn. 2005;25(3):264-66.

Passarge E, Lenz W. Syndrome of caudal regression in infants of diabetic mothers: Observation of further cases. Pediatrics. 1966;37(4):672-5.

Renshaw TS. Sacral agenesis. The pediatric spine-principles and practice. New York: Raven Press. 1994;1:2214.

Soler N, Walsh C, Malins J. Congenital malformations in newborn infants of diabetic mothers. Q J Med. 1976;178:303-13.

Mills JL, Baker L, Goldman AS. Malformations in infants of diabetic mothers occur before the seventh gestational week. Diabetes.1979;28(4):292-3.

Kucera J. Rate and type of congenital anomalies among offspring of diabetic women. J Reprod Med. 1971;7(2):61-70.

Mitchell SC, Sellmann AH, Westphal MC, Park J. Etiologic correlates in a study of congenital heart disease in 56,109 births. Am J Cardiol. 1971;28(6):653-7.