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一例罕见的四级脊柱裂病例:多发性复合型1型和2型脊髓纵裂畸形、背侧脊髓脊膜膨出和脑积水。

An unusual case of 4 level spinal dysraphism: Multiple composite type 1 and type 2 split cord malformation, dorsal myelocystocele and hydrocephalous.

作者信息

Khandelwal Ashutosh, Tandon Vivek, Mahapatra Ashok K

机构信息

Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.

出版信息

J Pediatr Neurosci. 2011 Jan;6(1):58-61. doi: 10.4103/1817-1745.84411.

Abstract

The authors here have reported a rare case of a child with a complex spina bifida with two different levels of split cord malformation (SCM) type 1 and single level type 2, a non terminal myelocystocele, coccygeal dermal sinus, bifid fatty filum and hydrocephalus, which substantiates the neuroenteric canal theory and have further tried to highlight the importance of complete Magnetic resonance imaging (MRI) screening of the whole spine and brain with SCM to rule out other associated conditions. The patient was admitted with a leaking myelocystocele with bilateral lower limb weakness. MRI of whole spine with screening of brain was done. Patient underwent 5 operations in the same sitting- (According to classification given by Mahapatra et al.) removal of SCM type 1a at D7-8; removal of SCM type1c at L2-3; removal of SCM type 2 at D10; repair of non terminal myelocystocele at D6-D10; low pressure ventriculoperitoneal shunt on right side with excision of dermal coccygeal sinus; and, excision of bifid fatty filum. The clinicoradiological findings in our patient further substantiate the multiple accessory neuroenteric canal theory in the development of composite type of SCM. The physical and neurological signs of SCM and nonterminal myelocystocele should prompt the neurosurgeon to consider performing the screening MRI of whole spine with brain to rule out other composite types of SCM and hydrocephalus.

摘要

本文作者报告了一例罕见病例,患儿患有复杂脊柱裂,伴有两个不同节段的1型脊髓纵裂畸形(SCM)和一个节段的2型脊髓纵裂畸形、非终末型脊髓脊膜膨出、尾骨皮样窦、双叉脂肪终丝和脑积水,这证实了神经肠管理论,并进一步强调了对患有SCM的患者进行全脊柱和脑部完整磁共振成像(MRI)筛查以排除其他相关病症的重要性。该患者因脊髓脊膜膨出渗漏并伴有双侧下肢无力入院。进行了全脊柱MRI检查并筛查了脑部。患者在同一次手术中接受了5次手术——(根据Mahapatra等人给出的分类)在D7 - 8节段切除1a型SCM;在L2 - 3节段切除1c型SCM;在D10节段切除2型SCM;在D6 - D10节段修复非终末型脊髓脊膜膨出;右侧行低压脑室腹腔分流术并切除尾骨皮样窦;以及切除双叉脂肪终丝。我们患者的临床放射学表现进一步证实了复合型SCM发生过程中的多条副神经肠管理论。SCM和非终末型脊髓脊膜膨出的体格检查和神经学体征应促使神经外科医生考虑对全脊柱和脑部进行筛查MRI,以排除其他复合型SCM和脑积水。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce9a/3173919/c4b1fc1f6c49/JPN-6-58-g001.jpg

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