The lowest lumbar vertebrae were resting above an iliac amphiarthrosis. == Physique 3. a diabetic mother was at significant risk of developing caudal regression syndrome. Our present patient exhibited type1 of Welch and Aterman classification. There was total sacral agenesis associated with subtotal lumbar agenesis. The lowest vertebrae were resting above an iliac amphiathrosis. We strongly encourage primary care providers to discuss the consequences of maternal diabetes mellitus as part of routine anticipatory guidance for antenatal/prenatal management. Careful diabetic control in the preconceptional period and the first eight weeks of pregnancy may lower the chances of congenital anomalies. == Introduction == Caudal regression syndrome and caudal dysgenesis syndrome are broad terms that refer to a heterogenous constellation of congenital caudal Cinnamic acid anomalies affecting the caudal spine and spinal cord, the hindgut, the urogenital system, and the lower limbs. About 1525% of mothers of children with caudal regression syndrome have insulin-dependant diabetes mellitus [1]. Welch and Aterman [2] classified congenital sacral anomalies into 4 unique clinical types. (1) A non-familial type associated with maternal diabetes mellitus showing complete absence of the sacrum and lower vertebrae with multiple congenital Cinnamic acid anomalies, (2) agenesis of the distal sacral or coccygeal segments, (3) hemisacral dysgenesis with presacral teratoma, and (4) hemisacral dysgenesis with anterior meningocele. Autosomal dominant inheritance was suggested for the last three types. Patients with caudal regression syndrome lack motor function below the level of the remaining normal spine, much like those with myelomeningocele. In myelomeningocele, however, sensory nerve function is usually impaired below the level of the affected vertebrae. HK2 In caudal regression syndrome, sensation tends to be present at much more caudal levels. Infants of diabetic mothers have two to three times the average incidence of congenital anomalies. == Case Presentation == The young man was referred to the orthopaedic department at the age of 3 years (fig1). A loop colostomy in the left upper quadrant was performed at birth for his imperforate anus. He was the offspring of non-consanguineous parents. Family history revealed a non-insulin diabetic mother. There was no other history of significance. At birth he had lower limb anomaly and imperforate anus. There was a history of urine incontinence. Spinal-pelvic instability, dislocation of the hip, and knee-flexion contracture associated with Cinnamic acid popliteal webbing were the prominent orthopaedic abnormalities. Clinically he showed no dysmorphic craniofacial features and he was of normal intelligence. Hearing and vision were normal. No associated upper limb abnormalities. There was total sacral agenesis and partial lumbar spinal agenesis. His left hip was flexed and partially abducted because of a ptyrigium. Equinovarus deformity of the left foot was present as well. His motor development was normal over the right lower limb but paralyses and loss of sensations over the left lower limb was obvious. He was able Cinnamic acid to move by means of his normal motor right lower limb. Radiographic paperwork showed total agenesis of the sacrum with subtotal lumbar agenesis. The lowest lumbar vertebrae were resting above an iliac amphiathrosis (fig2). Lateral lesser limb radiograph showed fixed deformity of the left knee associated with soft tissue web behind the knee extends more than halfway down the tibia to the ankle associated with oligodactyly associated with significant dysplasia of the tarsal bones, agenesis and hypoplasia of the metatarsals and agenesis of the fourth metatarsophalangeal and proximal phalanx (fig3). For his left hip ptyrigium, correction has been carried out by the plastic surgeon, followed by open reduction of the left hip, faced with the fixed flexion contracture of the left knee. Elected amputation (subtrochanteric amputation) and prosthetic fitted might be our choice. The advantage of subtrochanteric amputation over amputation at a more distal level is the ease of prosthetic.