Rauzzino M J, Tubbs R S, Alexander E, Grabb P A, Oakes W J
Peninsula Neurosurgical Associates, Hampton, Virginia 23666, USA.
Neurosurg Focus. 2001 Jan 15;10(1):e2. doi: 10.3171/foc.2001.10.1.3.
Neurenteric cysts are infrequently reported congenital abnormalities believed to be derived from an abnormal connection between the primitive endoderm and ectoderm. The authors report a series of 13 patients treated over a 50-year period.
Of the 13 patients, seven were female and six were male. Their ages at presentation ranged widely from 5 weeks to 52 years of age. Children presented more commonly with cutaneous stigmata of occult spinal dysraphism (OSD) whereas adults presented primarily with pain. Neurological deficit as a presenting symptom was less common in our series, a finding that reflects the slow growth of these lesions. In all but one patient some form of vertebral anomaly was associated with the cystic lesions, including two patients with Klippel-Feil abnormalities. There was a high incidence of associated forms of OSD including split cord malformation, lipoma, dermal sinus tract, and tethered spinal cord. In previous reports the authors have suggested that neurenteric cysts are more common in the cervical region and in a position ventral to the cord. In the present series these cysts most commonly occurred as intradural, extramedullary masses in the thoracolumbar region, situated dorsal to the spinal cord. The median follow-up period was 7.5 years, and postoperative outcome reflected a patient's preoperative neurological status; in no patient was outcome worsened due to surgery.
Complete excision of the neurenteric cyst remains the treatment of choice, as subtotal excision is associated with recurrence.
神经肠囊肿是一种罕见的先天性异常，被认为起源于原始内胚层和外胚层之间的异常连接。作者报告了在50年期间治疗的一系列13例患者。
13例患者中，7例为女性，6例为男性。他们就诊时的年龄跨度很大，从5周龄到52岁。儿童更常见的表现是隐性脊柱裂（OSD）的皮肤体征，而成人主要表现为疼痛。在我们的系列中，以神经功能缺损作为首发症状的情况较少见，这一发现反映了这些病变生长缓慢。除1例患者外，所有患者的某种形式的椎体异常都与囊性病变相关，包括2例患有Klippel-Feil异常的患者。OSD相关形式的发生率很高，包括脊髓纵裂畸形、脂肪瘤、皮样窦道和脊髓拴系。在以前的报告中，作者曾提出神经肠囊肿在颈椎区域和脊髓腹侧位置更为常见。在本系列中，这些囊肿最常见于胸腰段硬膜内、髓外肿块，位于脊髓背侧。中位随访期为7.5年，术后结果反映了患者术前的神经状态；没有患者因手术导致结果恶化。
神经肠囊肿的完全切除仍然是首选治疗方法，因为次全切除与复发相关。
