INTRODUCTION: CRS is a rare congenital anomaly characterized by distal vertebral malformations, usually co-existing with spinal cord malformations, neurological decit in lower limb & neurogenic bladder. The incidence is 1 in 40,000-60,000 live births. The syndrome occurs more frequently in offsprings of diabetic than 1 nondiabetic mothers .The Incidence is about 1 in 350 infants of diabetic mothers which is about 200fold increase in diabetic 2patients than in general population . CRS may range from absent coccyx as an isolated nding without neurological sequelae, to sacral or lumbosacral 2agenesis . It can affect the lower extremities, the lumbar and coccygeal vertebrae and corresponding segment of the spinal cord. The neurological orthopedic, gastrointestinal, genitourinary and cardiac anomalies,imperforate anus malformed genitalia and renal dysplasia/aplasia and 3congenital heart defects are commonly seen . CASE REPORT: A 5yrs 8months old girl, the only child of non-consanguinous healthy parents. According to the mother's report, she had gestational diabetics and TIFFA scan was not done. Antenatal USG'S were done and were told normal to parents. The child was delivered preterm with 1kg birth weight. Postnatal examination showed hypoplastic lower limb with abducted hips with hypoplastic gluteal muscles. FIG 1A: showing picture of baby with Caudal Regression Syndrome FIG 1B :showing Polydactyly X-Ray &MRI of the lumbosacral region suggests of CRS/sacral agenesis. Neuro sonogram was normal; also had b/l contractures of knee with right femur fracture. FIG 2: showing x-ray of baby with Caudal Regression Syndrome USG abdomen revealed right pelvic kidney with b/l mild hydro uretro nephrosis, TFT's Normal, and all other routine blood investigations were normal. At the age of 4yrs,due to acute pyelonephritis, the child was taken to higher center and evaluated. USG abdomen suggestive of right pelvic kidney,b/l hydrouretronephrosis, trabeculations & diverticular & post-void residue in urinary bladder indicative of neurogenic bladder.MCUG was done suggestive of neurogenic bladder with b/l VUR. FIG 3: showing Micturating Cystourethrogram Routine blood investigations were done, and renal function tests were slightly above the baseline. CAUDAL REGRESSION SYNDROME-A CASE REPORT Original Research Paper Vineela Dantuluri Postgraduate Resident,department Of Paediatrics,konaseema Institute Of Medical Sciences And Research Foundation,amalapuram,E.G.DIST,AP. X 35GJRA - GLOBAL JOURNAL FOR RESEARCH ANALYSIS Paediatrics BACKGROUND: Caudal Regression Syndrome(CRS) is a spectrum of congenital malformation which consists of anomalies of the rectum ,genitourinary system,lumbosacral spine and the lower limbs. CASE CHARACTERISTICS: A 5yrs 8months female child with sacral agenesis,hypoplasia of lower limb with neurogenic bladder with chronic kidney disease(CKD). OBSERVATION: CRS usually present with variety of other associated anomalies.therefore thorough examination should be done to rule out other anomalies MESSAGE: Proper diagnosis helps in therapeutic and may improve the outcome. CIC helps in prevention or delay in occurrence of CKD. ABSTRACT KEYWORDS : Caudal Regression Syndrome, Chronic Kidney Disease, Continous Intermittent Catheterization. Surya Laxmi Devi Matta* Postgraduate Resident, Department Of Paediatrics,konaseema Institute Of Medical Sciences And Research Foundation ,amalapuram, E.G.DIST,AP. *Corresponding Author RAMESH T.V Professor &head Of The Department, Department Of Paediatrics, konaseema Institute Of Medical Sciences And Research Foundation ,amalapuram, E.G.DIST,AP. VOLUME-9, ISSUE-1, JANUARY-2020 • PRINT ISSN No. 2277 - 8160 • DOI : 10.36106/gjra 36 X GJRA - GLOBAL JOURNAL FOR RESEARCH ANALYSIS Antibacterial prophylaxis with Cexime was started, and CIC was taught to the parents and was advised to do it regularly. But the child had lost to follow up for the next two years and at the age of 5yrs 8months she was referred to our hospital because of deterioration of renal function. USG abdomen and pelvis showed progression of b/l hydronephrosis and dilatation of ureters. TREATMENT: Thorough disease history taken from his mother revealed self- discontinuation of therapy at least 1 ½ years ago. Therefore therapy was restarted and diagnosis of CRS with CKD with anemia with neurogenic bladder was made. The child was kept on i.v antibiotics and nephrologist and urologist consultation was taken, and inj Ceftriaxone, inj sodium bicarbonate,inj erythropoietin 2000IU s.c every weekly and tab amlodipine and continuous bladder drainage were advised. Currently, the girl is under ambulatory nephrological care and her CKD is stable. DISCUSSION: CRS is a result of neural tube defects that appear at an early embryonic stage. Its etiopathogenesis is still unclear. Several etiological factors were suggested including genetic predisposition vascular hypoperfusion Vitamin A intoxication exposure to organic fat solvents, radiation, use of lithium salts, 4amphetamine or alcohol . The incidence of CRS is 1,3,4signicantly higher in children of diabetic mothers . In some patients with CRS, specic musculoskeletal manifestations may be observed including shortening of lower limbs due to lack of their growth, exed and abducted hips, exion contractures of hips, knees and pelvic deformity. In those patients, CRS may be suspected prenatally during routine USG examination, but it should be emphasized that the nal diagnosis can be made exclusively by performing MRI of the lumbosacral spinal cord. In the majority of the patients with CRS, different forms of lower urinary tract neurogenic dysfunctions were observed including neurogenic bladder, lack of voluntary sphincter control. Some of them cannot be recognized before toilet training. Patients who are suffering from CRS seem to represent the population at high risk of CKD development so that urological and nephrological examinations, including a urodynamic study are necessary for all children with CRS for identifying urinary system disorders. CKD may be prevented or delayed in patients with CRS by the adequate treatment that usually includes anticholinergic agents, alpha-adrenergic antagonists, antibacterial 1,5prophylaxis and CIC . The course of the disease in our patient showed importance of such therapy. Its discontinuation leads to a progression of b/l hydro uretronephrosis and signicant lowering of eGFR. In conclusion, there is no doubt that proper diagnosis helps in therapeutic decisions and may improve the outcome. REFERENCES: 1. Zaw W Stone DG, Caudal regression syndrome in twin pregnancy with type 2 diabetes, J perinatal2002,22:171-174. 2. Becerra JE, Khoury MJ, Codero JF, Erickson JD, diabetes mellitus during pregnancy and risk of specic birth defects, a population-based case-control study,pediatrics 1990 85:1-9 3. Singh SK, Singh RD, Sharma, Caudal regression syndrome-case report and review of literature. Pediatric surgery. int2005;21:578-581. 4. S.B.Bauer ;neurogenic bladder:etiology and assessment paediatric nephrology,23(2008), p.p-541-551. 5. P. Gehlot.J.Mandliya; caudal regression syndrome. Int j basic appl med sci, 1(2011), pp 126-130. VOLUME-9, ISSUE-1, JANUARY-2020 • PRINT ISSN No. 2277 - 8160 • DOI : 10.36106/gjra