Case Report

Neurenteric cyst presenting with stridor and dysphagia: case report and literature review

Scottish Medical Journal 2015, Vol. 60(1) e4–e7 ! The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0036933014563239 scm.sagepub.com

V Visvanathan1, J Crowther2 and William Taylor3

Abstract Objective: Neurenteric cysts are rare lesions that account for 0.7–1.3% of all spinal cord tumours. We report the first ever case of a neurenteric cyst presenting with stridor and dysphagia. A literature review on the presentation and management of these lesions is also included. Methods: A MEDLINE search of articles using the terms ‘neurenteric cyst’, ‘intraspinal cyst’, ‘enterogenous cyst’, ‘intramedullary cyst’ along with diagnosis, presentation and management was performed. Suitable references from these articles were also reviewed. Results: All published evidence on neurenteric cysts are either case series or case reports (level IV/V) with the largest case series reporting 23 patients from a single institution. Conclusion: Neurenteric cysts are rare spinal cord lesions that usually present with focal neurological signs and managed within neurosurgical units. This is the first reported case of a neurenteric cyst presenting with upper aerodigestive tract symptoms warranting specialist ear, nose and throat input.

Keywords Neurenteric cyst, intramedullary cyst, spinal cord cysts

Introduction Spinal neurenteric cysts are rare benign lesions that represent 16% of cysts in the central nervous system and 0.3–0.5% of spinal tumours.1,2 Neurenteric cysts can either be intramedullary or extrameduallary in location. Most published cases document patients presenting with pain and focal neurological signs. We report the first ever case of a neurenteric cyst presenting with symptoms involving the upper aerodigestive tract.

Case report A 48-year-old man presented with a 10-month history of an enlarging left neck swelling and a 2-month history of dyspnoea, dysphagia to solids and restricted neck movements. He had no other ear, nose and throat (ENT) symptoms. Past medical history was significant for hypothyroidism for which he was on thyroxine. He was a smoker and consumed a moderate amount of alcohol. Clinically the patient was noted to have a

mild degree of stridor, and neck examination revealed a large cystic lesion (approximately 8 cm) extending from level 2 to level 4 on the left side. The rest of the examination including flexible nasendoscopy was unremarkable.

Investigations The patient underwent an ultrasound-guided aspiration of the cyst which revealed mucinous contents. Computed tomography (CT) imaging of the neck and chest (Figure 1) revealed a large cystic structure centred

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Specialist Trainee ENT/Head and Neck Surgery, Southern General Hospital, UK 2 Consultant ENT/Skull Base Surgeon, Southern General Hospital, UK 3 Consultant Neurosurgeon, Southern General Hospital, UK Corresponding author: V Visvanathan, Specialist trainee ENT/Head and Neck Surgery, Southern General Hospital, Glasgow G51 4TF, Scotland. Email: [email protected]

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Figure 1. CT axial slice at level of thyroid cartilage demonstrating cyst in prevertebral space.

within the lower neck and extending down into the upper mediastinum measuring 10  8 cm. It was overlying the vertebral bodies C2 to C7. Compression of the trachea was noted. The mass itself was also seen extending into the prevertebral space through a gap in vertebral body C2 and appeared to be in continuity with the spinal canal. An MRI scan of the neck (Figure 2) apart from this large cystic lesion further identified a small 1-cm cyst immediately anterior to the vertebral body extending through the defect in C2 vertebra. Whilst this small cystic lesion demonstrated cerebrospinal fluid (CSF) signal intensity consistent with a meningocele, the large 10-cm mass had a predominantly fluid signal.

Differential diagnosis A diagnosis of neuroenteric cyst was made based on the imaging. Differential diagnosis of a large cervical cyst causing airway compromise includes lesions arising within the thyroid, branchial cyst or a cervical lymph node.

Treatment options Due to the presence of airway compromise, the most appropriate management plan was excision of the cyst under general anaesthesia.

Surgical technique A left transcervical incision was made. Subplatysmal flaps were raised and the sternomastoid retracted laterally. Dissection was close to the cyst and the cyst was freed from the prevertebral fasica. The neck of the cyst was identified, ligated and excised in total. A drain was placed postoperatively and the patient discharged home two days later.

Figure 2. T2-weighted MRI coronal slice showing hyperintense cystic mass.

Recovery and follow-up The patient was discharged home two days later and follow-up at 6 months revealed no evidence of recurrence. Patient remains under close follow-up.

Discussion Spinal neurentic cysts are rare congenital lesions that are usually present in the second and third decades of life with a slight male preponderance. They are commonly seen in the cervicothoracic region.3 They occur due to incomplete separation of the embryonic notochodal plate and endoderm during the third week of embryogenesis. The nomenclature of these lesions has evolved from their first description by Kubie and Fulton in 19284 as teratomatous cysts later modified by Puusepp in 19345 as intestinomas to the now widely accepted neurenteric cyst by Holcomb and Matson in 1954.6 Histologically Wilkins and Odom classified neurenteric cyst into three variants.7 Type A cysts comprise ciliated or nonciliated cuboidal/columnar cells resting on basement membrane composed of type-4 collagen. Type-B cysts consist of all the elements of type-A cysts along with bone, fat, cartilage and/or lymphatic tissue. Type-C cysts in addition to type-A contents contain ependymal or glial tissue. Another classification system based on clinical and prognostic features was proposed by Gimeno8 (Table 1). To our knowledge, this is the first reported case of a neurenteric cyst presenting with stridor and dysphagia. Clinical symptoms are usually secondary to meningeal

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Scottish Medical Journal 60(1)

Table 1. Classification of spinal neurenteric cysts. Type of cyst

Pathology

Prognosis

Congenital dorsal enteric fistula

Poor prognosis, often incompatible with life

Congenital dorsal enteric diverticulum Congenital dorsal enteric cyst (a) Prevertebral (b) Postvertebral

Fistulous communication between gut and skin in midline traversing the spinal column and cord Persistence of ventral part of neurenteric canal Ventral and dorsal portion of canal obliterate with remnant tissue forming a cyst Associated with vertebral anomalies

Congenital dorsal enteric sinus

Ventral portion of canal obliterates with persistence of only the dorsal portion

irritation and include pain, and motor or sensory deficits depending on the level involved. Myelopathic symptoms are usually seen with lesions involving the cervicothoracic region, while radicular symptoms are seen in lesions affecting the cervical and lumbar region. Newborns and infants can also present with signs of meningitis, incontinence, chronic pyrexia and paraplegia.9,10 Our patient did not have neurological signs. Neurenteric cysts have also been reported with other malformations such as spinal dysraphism, spina bifida and Kippel–Fiel syndrome.11 There are also reported associations with gastrointestinal, renal, cardiac and cutaneous abnormalities.12 Cross-sectional imaging is paramount in the workup of these patients. CT and MRI imaging are complementary. CT imaging is useful to identify bony abnormalities that are associated with these lesions, whereas MRI is superior in studying neural anatomy. Typical features of neurenteric cyst on imaging are a lobulated homogenous mass without an associated mural nodule.13 Like our case, the classic MRI feature is isointensity on T-1 weighted imaging and hyperintensity on T-2 weighted images. However these radiological findings are not entirely specific. Muzumdar et al. described a cyst with a granulomatous-type presentation with peripheral enhancement whilst in a study of 18 patients,14 Kimura et al. in their study of 18 patients reported only two cases showing the typical isointensity on T-1 and hyperintensity on T-2 weighted images.15 Complete surgical excision with avoidance of neurovascular injury is the treatment of choice for neurenteric cysts within the spinal canal. Various approaches described in the literature include anterior, posterior and lateral techniques. The posterior approach is favoured as it is associated with fewer complications. Although complete excision is the norm, this may not be possible in all cases. Factors contributing to difficult excision include limited access, bony abnormalities,

Asymptomatic Intraspinal and postvertebral types are often symptomatic The prevertebral type can extend into the mediastinum Can cause spinal compression and meningitis

adhesions to the spinal cord or intramedullary cysts.15,16 An attempt at complete resection of intramedullary neurenteric cysts carries a risk of permanent neurological injury. Hence in such cases, a planned subtotal excision may be appropriate as documented in some case series.1,17,18 Cyst recurrence is a recognised complication and ranges between 0% and 37%. Chavda et al. in the longest follow-up period at 30 years report a 37% recurrence rate (two patients) among eight patients.19 Unsurprisingly, recurrences were observed in patients who underwent partial excision. In the largest series of 23 patients, Garg et al. reported recurrence in five patients (21%).16 Other modalities of treatment include simple aspiration, fenestration of the cyst and/or marsupialisation. Although these methods may bring about symptomatic relief, these are temporary measures. Certainly our patient demonstrated an improvement in stridor following needle aspiration.

Conclusion . Neurenteric cysts are rare lesions and usually present with focal neurological signs. . This is the first case report of a neurenteric cyst presenting with features of stridor and dysphagia requiring specialist ENT and neurosurgical input. . MRI and CT imaging are crucial to aid diagnosis and plan appropriate treatment. . Complete surgical excision is the treatment of choice, however, in intramedullary lesions and those with neuronal adhesions, partial excision may be appropriate to avoid long-term neurological sequel. Declaration of conflicting interests None declared.

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Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

Authors’ contribution 1. Mr V Visvanathan (Manuscript preparation, literature review)

2. Mr W Taylor (Manuscript review)

3. Mr J Crowther (Case concept, manuscript review)

References 1. Lippman CR, Arginteanu M, Purohit D, et al. Intramedullary neurenteric cysts of the spine. J Neurosurg (Spine 2) 2001; 94: 305–309. 2. Palma L and Lorenzo Di. Spinal endodermal cysts without vertebral or congenital abnormalities. Acta Neurochir (Wien) 1976; 33: 283–300. 3. Arai Y, Yamauchi Y, Tsuji T, et al. Spinal neurenteric cyst. Report of two cases and review of forty one cases reported in Japan. Spine 1992; 17: 1421–1424. 4. Kubie LS and Fulton JF. A clinical and pathological study of two teratomatous cysts of the spinal cord containing mucus and ciliated cells. Surg Gynec Obstet 1928; 47: 297–311. 5. Agnoli AL, Laun A and Schonmayr R. Enterogenous intrapinal cyst. J Neurosurg 1984; 61: 834–840. 6. Paleologos TS, Thom M and Thomas DGT. Spinal neurenteric cysts without associated malformations. Are they the same as those presenting in spinal dysraphism? Br J Neurosurg 2000; 14: 185–194. 7. Wilkens RH and Odom GL. Spinal intradural cysts. In: Vinkin PJ and Bruyn GW (eds) Tumours of the spine and spinal cord, Part II. Handbook of clinical neurology. Vol. 20, North Holland: Amsterdam, 1976, pp.55–102.

8. Gimeno A. Arachnoid, neurenteric and other cysts. In: Vinken PJ and Bruyn GW (eds) Congenital malformations of the spine and spinal cord. Handbook of clinical neurology. Vol. 32, Amsterdam: North Holland Publishing, 1978, pp.421–446. 9. Shenoy SN and Raja a. Spinal neurenteric cyst. Report of 4 cases and review of the literature. Pediatr Neurosurg 2004; 40: 284–292. 10. Rizk T, Lahoud GA, Maarrawi J, et al. Acute paraplegia revealing an intraspinal neurenteric cyst in a child. Childs Nerv Syst 2001; 17: 754–757. 11. Mann KS, Khosla VK, Gulati DR, et al. Spinal neurenteric cyst. Association with vertebral anomalies, diastematomyelia, dorsal fistula and lipoma. Surg Neurol 1984; 21: 358–362. 12. de Oliveira RS, Cinalli G, Roujeau T, et al. Neurenteric cysts in children: 16 consecutive cases and report of the literature. J Neurosurg 2005; 103: 512–523. 13. Brooks BS, Duvall ER, El Gammal T, et al. Neuroimaging features of neurenteric cysts. Analysis of nine cases and review of the literature. AJNR Am J Neuroradiol 1993; 14: 735–746. 14. Muzumdar D, Bhatt Y and Sheth J. Intramedullary cervical neurenteric cyst mimicking an abscess. Pediatr Neurosurg 2008; 44: 55–61. 15. Kimura H, Nagatomi A, Ochi M, et al. Intracranial neurenteric cyst with recurrence and extensive craniospinal dissemination. Acta Neurochir (Wien) 2006; 148: 347–352. 16. Garg N, Sampath S, Yasha TC, et al. Is total excision of spinal neurenteric cysts possible? Br J Neurosurg 2008; 22: 241–251. 17. Cai C, Shen C, Yang W, et al. Intraspinal neurenteric cysts in children. Can J Neurol Sci 2008; 35: 609–615. 18. Rotondo M, D’Avanzo R, Natale M, et al. Intramedullary cysts of the spine. Report of three cases. J Neurosurg Spine 2005; 2: 372–376. 19. Chavda SV, Davies AM and Cassar-Pullicino VN. Enterogenous cysts of the central nervous system: a report of eight cases. Clin Radiol 1985; 36: 245–251.

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Neurenteric cyst presenting with stridor and dysphagia: case report and literature review.

Neurenteric cysts are rare lesions that account for 0.7-1.3% of all spinal cord tumours. We report the first ever case of a neurenteric cyst presentin...
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