A rare case of giant terminal lipomyelocystocele with partial sacral agenesis
A 6-month-old female child presented with a progressively increasing lumbosacral swelling which was present since birth []. The child was a full-term normal vaginal delivery and had normal developmental milestones. There was no history of CSF leak or meningitis. The patient had a weakness in ankle movement in both the lower limbs since birth. No obvious history of dribbling or urinary incontinence was forthcoming. On physical examination, there was a soft lumbosacral mass measuring 14 cm × 12 cm × 10 cm in size, obliterating the intergluteal cleft and with normal skin on its surface. The swelling was partially transilluminant and increased in size when the child cried. There were no nevi, hypertrichosis, skin defect, sinus or CSF leak. The head circumference was 44 cm and the anterior fontanelle was lax. The anterior abdominal wall and the genitalia were normal. The anus was patulous.\nComputed tomography and magnetic resonance imaging (MRI) revealed a giant terminal lipomyelocystocele [Figures and ] which contained a trumpet like flaring of the central canal of the distal spinal cord and a meningocele located around the dilated central canal, which bulged into the subcutaneous region along with subcutaneous fat tissue. Associated features of partial sacral agenesis in the form of absent dorsal and left sided elements were noticed. Syrinx was seen traversing the whole of the spinal cord. At surgery lipoma infiltrating, the myelocystocele was seen. Lipoma was excised. Caudal portion of trumpet shaped myelocystocele was resected and the remaining base was repaired using 6–0 monocryl suture. Dura was then closed with 4–0 silk suture.\nPost-operatively the child had wound infection, which required secondary suturing. The child recovered well with no post-operative deficits. Histopathology confirmed the findings of meningeal tissue with adipose and few wavy nerve fibers [].