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Chiari Type I Malformation with Cervicothoracic Syringomyelia

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Chiari Type I Malformation with Cervicothoracic Syringomyelia Neurology International 2017; volume 9:7336 Chiari type I malformation drome, is a gradually progressive sporadic with cervicothoracic motor neuron disorder, depicted by severe Correspondence: Jiagang Liu, Department of flaccid paralysis and muscle wasting in both Neurosurgery, Post-Graduate Training Centre, syringomyelia subterfuge as arms symmetrically, but comparatively West China Hospital, Sichuan University, 37 flail arm syndrome sparing the legs and bulbar parts, and Guo Xue Xiang Road, Chengdu, Sichuan, scarcely signs of upper motor neuron 610041, China. 7-9 Tel: +86-028-85422488 - Fax: +86-28-85422490. 1 1-3 lesions. The onset of syringomyelia Zhi Gang Lan, Seidu A. Richard, E-mail: [email protected] Jiagang Liu,1 Chao You1 although commonly chronic and gradually progressive, very few case advanced into 1Department of Neurosurgery, West Key words: Chiari type I malformation; adulthood unnoticed. The severe progres- Cervicothoracic; Syringomyelia; Subterfuge; China Hospital, Sichuan University, sive adult type is most likely to mimic mus- Flail arm syndrome. Sichuan; 2Department of Immunology, cular dystrophy’s4. Cerebellar tonsilar Jiangsu University, Jiangsu, China; ectopia bulging through the foramen mag- Ethics approval and consent to participate: 3Department of Surgery, Volta Regional num is frequently the imaging findings in The ethical committee of the hospital fully Hospital, Ho, Ghana-West Africa patients with this condition.3,4,10 The gold approved our case study. The patient was standard of treatment for this disorder is informed about our intention to involve him in surgery and the choice of surgical approach- a case study and he agreed to partake in the es for Chiari type I, depending on the sur- study. He signed the concern form before the operation was carried out according to all sur- Abstract geon’s understanding of the pathophysiolo- gy of the disease manifestation.1,4 In most gical protocols. Chiari type I malformation with cervi- cases the pathology is situated at the level of Consent for publication: the patient was dually cothoracic syringomyelia although very the foramen magnum leading to disparities informed about our intention to publish his common in clinical practice usually in chil- in the contents and the volume of the poste- case and she fully concerted to the use of these dren can progress slowly and mimic muscu- rior fossa. We present an adult case of con- documents. The hospital also concerted to the lar dystrophies in adulthood. We present a genital type I Chiari malformation with cer- use of this information for publication. rare adult case of Chiari type I malforma- vicothoracic syringomyelia subterfuge as only tion with cervicothoracic syringomyelia Flail arm syndrome and review of literature. Conflict of interest: the authors declare no subterfuge as Flail arm syndrome. A 44- potential conflict of interest. year-old man was diagnosed with congeni- tal type I Chiari malformation with cervi- use Contributions: all the authors contributed cothoracic syringomyelia about 21 years Case Report equally to the manuscript design and writing. ago without surgery. His health status dete- riorated over the years until 21 days prior to A 44-year-old man presented with Received for publication: 30 July 2017. presentation when he had severe pain in the worsening chronic motor symptoms for 21 Revision received: 7 August 2017. Accepted for publication: 21 August 2017. right knee. In his upper limbs, he had bilat- years. He is a known case of congenital type I Chiari malformation with cervicothoracic eral corresponding severe weakness of 0/5 This work is licensed under a Creative proximal strength and 0/5 strength in his syringomyelia diagnosed about 21 years ago without surgery. He was not able to Commons Attribution NonCommercial 4.0 distal muscles. Magnetic resonance imag- License (CC BY-NC 4.0). ing (MRI) revealed an enlargement of the access surgery from the time of the initial spinal cord from C1-C4 level with a mass diagnosis (21 years ago) until he presents at ©Copyright Z.G. Lan et al., 2017 that appeared hypo-dense on T1 and hyper- our facility because of financial constraints. Licensee PAGEPress, Italy dense on T2. Syringomyelia is a potentially commercialHe health status deteriorated over the years Neurology International 2017; 9:7336 until 21 days prior to presentation when he doi:10.4081/ni.2017.7336 serious neurologic condition that can mimic had severe pain in the right knee. The right other neuromuscular disorders. Early detec- knee pain is aggravated by walking, climb- tion and diagnosis with MRI is crucial to could walk about except for the right knee ing stairs or in sitting and relieved by lying avoid irreversible neurological complica- pain, which limited his movement in the in bed. There was no associated swelling, tions. We suggest that whetherNon asympto- ward. All routine laboratory investigations no neck pains, backaches or abdominal matic or symptomatic, decompressive sur- were essentially normal. Electro - pains. He denied having dysphagia, cough, gery should be carried out to allow for free physiological studies reveal bilateral difficulty in breathing or palpitations. All flow of cerebrospinal fluid thereby improv- absence of median, ulnar and radial nerves other systems were normal. Physical exam- ing the quality of life for the patient. compound muscle action potentials ination demonstrated no face weakness, (CMAP) with slightly abnormal sensory numbness, sensory deficits, unusual reflex- nerve action potential (SNAP) at the upper es, muscle fasciculations, as well as upper limbs. However, the all the nerves on the motor neuron signs. Examination of limbs Introduction lower limbs have normal CMAPs and nor- revealed severe atrophy of his bilateral fore- Syringomyelia occurs when a cyst, typ- arm flexor and extensor muscle groups with mal SNAPs. Electromyography (EMG) of ically made up of excess cerebrospinal fluid his lower extremities spared (Figure 1A). In his upper extremities revealed of diffuse (CSF), cumulates inside the spinal cord the upper limbs, he had bilateral correspon- chronic neurogenic changes in almost all either within the parenchyma as a focal ding severe weakness of 0/5 proximal the nerves but normal in all the nerves in the dilation of the central canal.1-5 Chiari mal- strength and 0/5 strength in his distal mus- lower extremities. formation type I is a form of non-communi- cles (Figure 1B). He could only lift his Preoperative MRI done revealed an cating syringomyelia.3-6 Flail arm syndrome hands with support. He had 5/5 strength in enlargement of the spinal cord from C1-C4 (FAS), also referred to as brachial amy- his bilateral hip extensors and flexors and level. Within the spinal cord is a mass that otrophic diplegia or man-in-barrel syn- 5/5 strength in bilateral knee extensors and appears hypo-intense on T1 and hyper- [Neurology International 2017; 9:7336] [page 71] Case Report intense on T2. These findings are consistent with Chiari malformation type I with prominent syringomyelia (Figure 2A and B). MRI of lumbar vertebral and adjacent structures showed no abnormalities. There is no disc space narrowing, no abnormal vertebral body and no cord stenosis. The patient was the scheduled for suboccipital decompression of the syringomyelia. The patient was on park-bench position after general anesthesia with the surgical incision area marked. A straight median incision was made from the occipital protu- berance up to C3. Subcutaneous tissues and muscle was dissected up the C3. Suboccipital craniectomy and laminectomy of C1 and C2 was done using the high-drill and further exposer with a Kerrison rongeur. Resection of the atlanto-occipital dura was done to free the compression. The dura was peeled off with a Y shaped inci- sion and the arachnoid membrane was intact. After securing total hemostasis, the fascia, muscle, subcutaneous tissues and skin was closed in layers. Estimated blood lost was 50 mL. We noticed during surgery only that there were no obvious abnormalities on the skin and subcutaneous tissues but how- Figure 1. Physical examination showing patient’s upper extremities with severe atrophy of forearm extensors (A) and flexors (B). ever, there was thickening of paraspinal muscle with retracted occipital plane and use thickening of atlanto-occipital fascia. The patient was nursed in the recovery word and B later to neurosurgery word. Post-operative A management was uneventful. His amyotro- phy did not improve much while on admis- sion. He was discharged home two weeks after the operation. Outpatient visits were arranged at 3 months’ interval. MRI done at the 2-year follow up showing the syringomyelia was shrinked significantly (Figure 2C and D). His amyotrophy remained stable over the years afterwards. commercial Discussion Non The precise pathogenic link between syringomyelia and Chiari type 1 malforma- C D tion is still a matter of debate among neuro- surgeons and quite a number of authors have proposed various theories as the for- mation of syrinx.1,3,4 Most of the authors have come to the conclusion that malforma- tion of the mesodermal occipital somite is accountable for the tiny nature of the poste- rior fossa leading to the anomalous CSF flow at the level of the foramen mag- num.1,11,12 Therefore, most disorders origi- nate from the cervical spinal cord and can enlarge superiorly into the brainstem lead- ing to the formation of syringobulbia or inferiorly into the thoracic and sometimes Figure 2. Preoperative magnetic resonance
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