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NEUROIMAGES
Year : 2017  |  Volume : 65  |  Issue : 1  |  Page : 223-224

Type 1 neurofibromatosis with intracanalicular rib head dislocation


Department of Neurosurgery, Sri Venkateswara Institute of Medical Sciences (SVIMS), Tirupati, Andhra Pradesh, India

Date of Web Publication12-Jan-2017

Correspondence Address:
Venkata R C Vemula
Department of Neurosurgery, Sri Venkateswara Institute of Medical Sciences (SVIMS), Tirupati - 517 507, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.198226

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How to cite this article:
Vemula VR, Bodapati CP, Mohan K, Agarwal S. Type 1 neurofibromatosis with intracanalicular rib head dislocation. Neurol India 2017;65:223-4

How to cite this URL:
Vemula VR, Bodapati CP, Mohan K, Agarwal S. Type 1 neurofibromatosis with intracanalicular rib head dislocation. Neurol India [serial online] 2017 [cited 2017 Feb 23];65:223-4. Available from: http://www.neurologyindia.com/text.asp?2017/65/1/223/198226


Scoliosis is the most (occurring in 10–64% patients ) skeletal manifestation of neurofibromatosis type 1 (NF1).[1] Two patterns of scoliosis are seen in these patients.[2] Type 1 or nondystrophic scoliosis is similar to idiopathic scoliosis and is treated accordingly. Type 2 or dystrophic scoliosis is a short, segmental, angulated curve with severe kyphosis, and is often accompanied by rib penciling, vertebral wedging and enlargement of the neural foramina.[3] A rare condition associated with the dystrophic scoliotic curves is dislocation of the rib heads on the convex side of the curve, with their displacement into the spinal canal through the enlarged neural foramina causing neurological impairment.[4] Owing to the rarity of this condition, there is no consensus on the management of rib dislocations. Based on the available literature, rib head resection [5] is done in patients presenting with neurological impairment, and if the rib head is unresectable due to cord adhesions, a rib shaft osteotomy [6] is advocated. In asymptomatic patients with no evidence of spinal cord compression, rib head resection is questionable. A spontaneous rib head reduction is documented in patients following the curve correction with posterior fusion and segmental instrumentation.[7] Dystrophic scoliosis should be suspected in any patient with NF1 and spinal anomaly, and a thorough imaging should be done to rule out rib dislocation.

A 14-year-old boy, a known case of NF1, presented with a 4-month history of deformity over the mid back and difficulty in walking, being bedridden for the past 2 months. On examination, the patient had café au lait spots [Figure 1]a, Lisch nodules, axillary freckling, and deformity [Figure 1]b in the thoracic region. Neurological examination revealed weakness of bilateral lower limbs (3/5 power), spasticity, and a sensory loss below D10 vertebral level. The imaging revealed a short segmental scoliotic curve with the apex at D8 vertebral level with convexity towards the right side [Figure 1]d and the presence of internal dislocation of the rib heads into the spinal canal at the vertebral levels D8,9, and 10 [Figure 1]c. The patient underwent deformity correction with segmental posterior instrumentation from D5 to D12 after resecting the dislocated rib heads under intraoperative neuromonitoring. There was significant correction of the curve [Figure 1]e, and the patient improved neurologically; the patient was ambulant at a 6-month follow-up.
Figure 1: (a) Clinical photograph showing the cafe au lait spots; (b) clinical photograph showing the dorsal deformity; (c) axial computed tomography showing the internal dislocation of the rib head into the spinal canal; (d) X-ray of the dorsal spine showing a short segmental scoliotic curve with the apex at D8 and convexity to the right; (e) Postoperative X-ray of the dorsal spine showing a significant correction of the scoliosis

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  References Top

1.
Akbarnia BA, Gabriel KR, Beckman E, Chalk D. Prevalence of scoliosis in neurofibromatosis. Spine 1992;17:S244-8.  Back to cited text no. 1
    
2.
Crawford AJ, Bagamery N. Osseous manifestations of neurofibromatosis in childhood. J Pediatr Orthop 1986;6:72-88.  Back to cited text no. 2
    
3.
Winter RB, Moe JH, Bradford DS, Lonstein JE, Pedras CV, Weber AH. Spine deformity in neurofibromatosis. A review of one hundred and two patients. J Bone Joint Surg Am 1979;61:677-94.  Back to cited text no. 3
    
4.
Deguchi M, Kawakami N, Saito H, Arao K, Mimatsu K, Iwata H. Paraparesis after rib penetration of the spinal canal in neurofibromatous scoliosis. J Spinal Disord 1995;8:363-7.  Back to cited text no. 4
    
5.
Yalcin N, Bar-on E, Yazici M. Impingement of spinal cord by dislocated rib in dystrophic scoliosis secondary to neurofibromatosis type 1: Radiological signs and management strategies. Spine 2008;33:E881-6.  Back to cited text no. 5
    
6.
Abdulian MH, Liu RW, Son-Hing JP, Thompson GH, Armstrong DG. Double rib penetration of the spinal canal in a patient with neurofibromatosis. J Pediatr Orthop 2011;31:6-10.  Back to cited text no. 6
    
7.
Sun D, Dai F, Liu YY, Xu JZ. Posterior-only spinal fusion without rib head resection for treating type I neurofibromatosis with intra-canal rib head dislocation. Clinics (Sao Paulo) 2013;68:1521-7.  Back to cited text no. 7
    


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