Acharyya Saugata, Chakravarty Sanchari
Ddepartment of Pediatrics, Calcutta Medical Research Institute, Kolkata, West Bengal, India.
J Family Med Prim Care. 2018 Sep-Oct;7(5):1103-1105. doi: 10.4103/jfmpc.jfmpc_158_18.
An 11-month-old female child presented with progressive weakness involving both lower limbs and left upper limb. There was hypertonia and hyperreflexia in the affected limbs. This was associated with multiple episodes of urinary tract infection and overflow incontinence of the urinary bladder. Magnetic resonance imaging of the spine revealed extensive intramedullary expansion of a congenital dermal sinus. There was a cystic lesion along the lower end of the sinus tract. Surgical exploration had confirmed the presence of an infected epidermoid cyst along with the dermal sinus tract. The entire tract as well as the abscessed epidermoid cyst was removed. Following this, the child showed gradual improvement of neurological function and bladder dysfunction. This is perhaps the first report in current literature of a congenital dermal sinus tract with such an extensive intramedullary extension (almost the entire spine) and an infected epidermoid tumor in an infant.
一名11个月大的女童出现双下肢和左上肢进行性无力。受累肢体有张力亢进和反射亢进。这与多次尿路感染及膀胱充溢性尿失禁有关。脊柱磁共振成像显示先天性皮样窦髓内广泛扩张。窦道下端有一个囊性病变。手术探查证实存在感染性表皮样囊肿及皮样窦道。整个窦道以及脓肿性表皮样囊肿均被切除。此后,患儿神经功能和膀胱功能障碍逐渐改善。这可能是当前文献中关于婴儿先天性皮样窦道伴如此广泛的髓内延伸(几乎累及整个脊柱)及感染性表皮样肿瘤的首例报道。