Open Access
CC BY-NC-ND 4.0 · Asian J Neurosurg
DOI: 10.1055/s-0045-1813647
Case Report

Pediatric Neurenteric Cysts of the Spine: A Case Report of Two Cases with Review of Literature

Authors

  • Sana Ahuja

    1   Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
  • Dipanker Singh Mankotia

    2   Department of Neurosurgery, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
  • Sufian Zaheer

    1   Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
 

Abstract

Neurenteric cysts are rare, benign congenital lesions of endodermal origin that primarily affect the spinal cord. Their clinical and radiological presentations can vary significantly, especially in pediatric patients, complicating diagnosis and management. We report two pediatric cases of spinal neurenteric cysts and review the relevant literature. An 11-month-old infant presented with progressive lower limb weakness. Magnetic resonance imaging (MRI) revealed a nonenhancing intradural cystic lesion at the thoracic level. A 14-year-old female reported neck pain and upper limb paresthesia; cervical spine MRI showed a well-demarcated T2-hyperintense cystic lesion. Both patients underwent complete surgical excision. Histopathology confirmed neurenteric cysts, showing pseudostratified or columnar epithelium with interspersed goblet cells. Postoperative recovery was uneventful, with no recurrence at 6-month and 1-year follow-up, respectively. These cases underscore the variability in presentation and spinal level involvement of neurenteric cysts in children. MRI plays a central role in preoperative assessment, though definitive diagnosis relies on histopathology. Complete surgical excision remains the treatment of choice, offering favorable outcomes with low recurrence risk. The age extremes and differing anatomical locations in our cases reflect the broad clinical spectrum seen in pediatric patients. Spinal neurenteric cysts should be considered in children with unexplained spinal cord-related symptoms. Early imaging, accurate histological diagnosis, and total surgical excision are critical for optimal outcomes. Increased clinical awareness and case reporting will aid in improving recognition and management of this rare spinal lesion.


Introduction

Pediatric neurenteric cysts of the spine represent a rare but clinically significant anomaly of the central nervous system (CNS). These cysts arise from remnants of the neurenteric canal, a structure present during embryonic development that normally disappears as the fetal spine matures. During embryonic development, the neurenteric canal connects the amniotic cavity to the primitive gut. Normally, it regresses as the spine forms, but in some cases, remnants persist and form cysts within the spinal canal leading to a spectrum of neurological symptoms and complications in affected children. These cysts typically contain fluid and may be lined with gastrointestinal or respiratory epithelium. The prevalence of spinal neurenteric cysts is extremely low with only a few cases, constituting less than 1.3% of all spinal cord tumors. To date, approximately 300 cases of neurenteric cysts have been reported in the literature, with pediatric cases representing a minority.[1]

Understanding the pathogenesis, clinical presentation, diagnostic modalities, and management strategies for pediatric neurenteric cysts is crucial for pediatricians, neurosurgeons, and other health care providers involved in the care of children with spinal abnormalities. Clinical presentation varies widely and can include symptoms such as back pain, sensory deficits, motor weakness, or bowel and bladder dysfunction. The severity of symptoms depends on the cyst's size, location, and effects on surrounding neural structures.[2]

Diagnosis often relies on imaging studies, with magnetic resonance imaging (MRI) being the modality of choice. MRI can accurately depict the cyst's size, location, and its relationship with adjacent neural elements, guiding treatment planning. Management of pediatric neurenteric cysts typically involves surgical intervention to decompress neural structures and remove the cyst. However, the surgical approach may vary based on factors such as cyst size, location, and associated neurological deficits.[1] [2]

Given the potential for neurological impairment and complications, timely diagnosis and appropriate management are paramount in optimizing outcomes for affected children. Multidisciplinary collaboration among pediatricians, neurosurgeons, radiologists, and other specialists is essential in providing comprehensive care for these patients. This study was conducted at a tertiary care teaching hospital in India, focusing on the clinical presentation, radiological features, surgical findings, and histopathology of two pediatric spinal neurenteric cysts along with a review of the literature reported in line with SCARE (Surgical CAse REport) guidelines.[3] The objective was to highlight the variable presentations of pediatric neurenteric cysts and emphasize the importance of early recognition and complete surgical excision.


Case Presentation

All cases of pediatric neurenteric cysts of the spine, confirmed histologically and treated surgically within a 1-year period from October 2022 to October 2023, were retrospectively reviewed. The patient's age, clinical presentation, radiological features, and operative and histopathological findings were evaluated. To minimize interoperator variation, histopathology slides underwent independent review by two pathologists (S.A. and S.Z.). Data analysis was conducted using descriptive statistics.

  • Inclusion criteria: Pediatric patients (≤ 18 years) with histopathologically confirmed neurenteric cysts of the spine who underwent surgical excision from year 2000 to 2023.

  • Exclusion criteria: Patients older than 18 years, those with incomplete clinical or radiological data, and patients with spinal cystic lesions of nonneurenteric origin. PRISMA (Preferred Reporting Items for Systematic reviews and Meta-Analyses) diagram outlining the selection process for the literature review is provided in [Fig. 1].

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Fig. 1 Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) diagram.

For the literature review, a systematic electronic search was conducted using the key term “pediatric neurenteric cysts of the spine” across multiple research databases including PubMed Central (PMC), Embase, Scopus, and Google Scholar. In PubMed Medline, a combination of Boolean operators with keywords “(neurenteric cysts AND spine AND pediatric)” was utilized for the search.

The present study included two cases of pediatric neurenteric cysts of the spine which were operated on in the neurosurgery department in the last year. One case was an 11-month-old male with a thoracic (T1–T3) neurenteric cyst, and the other was a 14-year-old female with a cervical (C4–C6) neurenteric cyst. This report delineates the pertinent physical examination and other salient clinical findings. They experienced a range of clinical symptoms including chronic headaches, vomiting, and motor and sensory deficits. The duration of these symptoms varied from 30 days to 2 years. MRI revealed intradural extramedullary cystic lesions in both patients. Both underwent surgical excision with favorable outcomes. Histopathology confirmed the diagnosis of neurenteric cyst, lined by columnar epithelium ([Table 1], [Figs. 2] and [3]).

Table 1

Summary of clinicopathological and radiological details of cases

Case

Age/Sex

Clinical presentation

Location

Clinical duration

Radiological findings

Gross and histopathological findings

Surgical excision

Follow-up

1

11 months/M

Decreased movement of bilateral lower limbs

Thoracic spine (T1-T3)

1 months

MRI- Well-defined intradural extramedullary cystic lesion in thoracic spine. The cyst was hypointense on T1 and hyperintense on T2-weighted images, with no postcontrast enhancement, consistent with their fluid-filled nature

Already cut open cystic structure

M/E- Cyst lined by cuboidal to columnar epithelium with goblet cells

Complete excision of cyst

Patient remained asymptomatic, with no evidence of clinical recurrence over 6 months

2

14/ F

Headache, vomiting

Cervical spine (C4-C6)

2 years

MRI- Well-defined cystic lesion centered in the cervical spine. The cyst was hypointense on T1 and hyperintense on T2-weighted images, with no postcontrast enhancement, consistent with their fluid-filled nature

Already cut open cystic structure

M/E- Cyst lined by pseudostratified ciliated epithelium

Complete excision of cyst

Patient remained asymptomatic, with no evidence of clinical recurrence over 1 year

Abbreviations: F, female; M, male; MRI, magnetic resonance imaging.


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Fig. 2 Neurenteric cyst in the thoracic spine in an 11-month-old child. (A) Magnetic resonance imaging (T1) exhibiting a well-defined intradural extramedullary cystic lesion in the thoracic spine. (B) Intraoperative image of the cystic mass. (C, D) Hematoxylin and eosin-stained sections exhibited a cyst lined by cuboidal to columnar epithelium with goblet cells [40×, 100×].
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Fig. 3 Neurenteric cyst in the cervical spine in a 14-year-old female. (A) Magnetic resonance imaging (T1) exhibiting a well-defined cystic lesion centered in the cervical spine. (B) Intraoperative image of the cystic mass centered in the cervical spine. (C, D) Hematoxylin and eosin-stained sections exhibited a cyst lined by pseudostratified ciliated epithelium [40×, 100×].

Discussion

Pediatric neurenteric cysts of the spine represent a rare but clinically significant anomaly within the CNS. As highlighted in the cases presented and supported by the literature review, these cysts can manifest across a broad spectrum of ages, ranging from infancy to adolescence, with variable clinical presentations and radiological features.[4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15] [16] The two cases described in this study further underscore the diverse clinical scenarios encountered in pediatric patients with neurenteric cysts, emphasizing the importance of multidisciplinary collaboration in their management.

The clinical presentation of pediatric neurenteric cysts can be nonspecific and variable, mirroring the heterogeneity of spinal cord pathology. Symptoms may range from chronic headache and vomiting to motor and sensory deficits, reflecting the cyst's size, location, and effects on adjacent neural structures. Radiological imaging, particularly MRI, plays a pivotal role in the diagnostic workup, providing detailed anatomical information and guiding treatment planning. The characteristic radiological features include well-defined intradural extramedullary cystic lesions with variable signal intensities, depending on the cyst's contents and associated hemorrhage or calcification.[1] [2]

Histopathological examination remains the gold standard for confirming the diagnosis of neurenteric cysts and distinguishing them from other cystic lesions of the spine. The differential diagnosis includes arachnoid cysts, epidermoid cysts, dermoid cysts, teratomas, and cystic tumors such as astrocytomas or ependymomas, all of which may mimic neurenteric cysts on imaging. Arachnoid cysts are usually cerebrospinal fluid-filled with arachnoid lining, epidermoid cysts contain keratinized squamous epithelium, dermoid cysts often harbor skin appendages such as hair or sebaceous material, while teratomas exhibit tissues from multiple germ layers. Intramedullary tumors like ependymomas or astrocytomas may also present with cystic changes, further complicating diagnosis.[9] [10] [11] [12] [13] [14] In such scenarios, histopathological examination is indispensable, as neurenteric cysts uniquely demonstrate a lining of gastrointestinal or respiratory-type epithelium, typically pseudostratified ciliated or cuboidal cells with goblet cells. These histological features corroborate the embryological origin of neurenteric cysts from remnants of the neurenteric canal, providing insights into their pathogenesis. Radiological imaging, particularly MRI, remains central in preoperative planning, though it cannot reliably differentiate among these entities.

The presented cases share similarities with previously reported cases in terms of clinical presentation, radiological features, and histopathological findings, particularly the presence of a pseudostratified or columnar epithelium with goblet cells. However, one distinguishing aspect was the location: case 1 involved the thoracic spine in an infant, while case 2 presented in the cervical region in an adolescent, reflecting the broad anatomic and age spectrum seen in the literature. The surgical outcome in both cases also mirrors previously reported favorable prognoses following total cyst excision. Across the literature, cases have been reported spanning a wide age range, with varying clinical symptoms and radiological features ([Table 2]). Surgical intervention, typically involving complete cyst excision, has been associated with favorable outcomes and low recurrence rates, as evidenced by the follow-up data provided in the literature.[4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15] [16]

Table 2

Clinicopathological and radiological details of previously reported cases

Authors

Age/Gender

Symptoms

Duration of symptoms

Location and size of cyst

Radiological findings

Histopathologic features

Surgical excision

Follow-up

Kim et al (2021)[4]

16 years/F

Slowly progressive headache

14 × 16 × 20 mm, premedullary cistern

MRI: A well-defined, intradural extramedullary cystic lesion in the premedullary cistern

Nonciliated, mucin-producing columnar and cuboid epithelial cells with isolated goblet cells

Far-lateral transcondylar approach with cyst puncture and complete resection of cyst

Uneventful, no evidence of any recurrence

Liu et al (2020)[5]

2 years/F

Right upper abdominal pain and sudden onset of lower limb paralysis

Sudden onset

2.5 cm

C6-T2

MRI: A meningeal cyst at the anterior spinal canal, C6 through T2 level

A fibrous wall lined with cuboid wall and columnar cells, some with cytoplasmic mucin

Laminectomy of T1-T2 and cyst excision

Uneventful, no evidence of any recurrence

Haque et al (2020)[6]

11 years/M

Gradually progressive neck pain and quadriparesis

1 year

4 × 2.5 cm

Cervical spine

MRI: A well-circumscribed, ventrally placed intradural, extramedullary cystic lesion extending from the pontomedullary junction down to C2 measuring 4 cm vertically and 2.5 cm anteroposteriorly

Neurenteric cyst

Suboccipital craniotomy followed by cyst excision

Uneventful, no evidence of any recurrence

Vasani et al (2019)[7]

9 month/M

Sudden onset of decreased movement in both lower limbs

2 weeks

C4-D1

D4-D6

MRI: One lesion at C4–D1 level (intradural-extramedullary location, cystic) compressing the spinal cord anteriorly. The second lesion was at D4–D6 level (intradural-extramedullary location, cystic) and pushed the spinal cord posteriorly

Dorsal lesion: A fibrocollagenous uni-/bilayered cyst wall lined with low columnar, flattened epithelial cells Cervical lesion: cuboidal to short columnar epithelium lining the fibrocollagenous cyst wall. No goblet cell seen

Laminectomy and decompression of dorsal lesion followed by cervical lesion 6 weeks later

Uneventful, no evidence of any recurrence

Lai et al (2018)[8]

5 years/M

Progressive weakness

3 years

2.1 × 2.5 × 4. cm

C3

MRI: A well circumscribed, dorsal intradural, extramedullary cystic lesion extending from the level of the upper medulla to C3

A cyst wall lined by columnar and pseudostratified epithelium with cilia and occasional goblet cells

C1 and partial C2 laminectomy and cyst excision

Uneventful, no evidence of any recurrence

Lan et al (2018)[9]

4 years/M

Cervical lordosis and bilateral lower extremity pain

5 months

T12-L1

MRI: A cystic lesion at T12-L1 with tethering of spinal cord and cervical lordosis

A benign cystic lesion lined by mucinous columnar epithelium

T12-L2 laminectomy and complete cyst excision

Uneventful, no evidence of any recurrence

Yuce et al (2015)[10]

1 year/M

Lower extremity weakness

T3-T4

MRI: An intradural extramedullary cystic mass at the T3–T4 level with posterior mediastinal component

Neurenteric cyst

Excision

Uneventful, no evidence of any recurrence

Choi et al (2015)[11]

15 years/M

Progressive neck and both shoulders pain which was not responding to medical treatment

2 months

2.7 × 1.6 × 4.8 cm

C1-C3

MRI: A well-delineated intradural extramedullary lesion located ventrally to the cord, extending from the C1 to the C3 level

A ciliated pseudostratified columnar epithelium containing mucinous contents

C1, C2, C3 hemilaminectomy followed by cyst excision

Uneventful, no evidence of any recurrence

Cai et al (2008)[12]

13 months/M

Decreased movement of lower limbs

6 months

L3-L4

MRI: Intradural extramedullary oval cyst, isointense to CSF

Columnar to cuboidal; mucin-producing cell rich lining

Posterior approach, Laminectomy L3-L4 and complete excision

Uneventful, no evidence of any recurrence

4.5 years/F

Neck tilt to left and neck pain

1 month

C2-C3

MRI: Intradural extramedullary round cyst, isointense to CSF

Columnar, ciliated; mucin-producing cell rich lining

Lateral cervical approach and complete excision

Uneventful, no evidence of any recurrence

9 months/F

Neck stiffness and quadriplegia

15 days

C3-C4

MRI: Intradural extramedullary round cyst, hyperintense to CSF

Columnar, ciliated; mucin-producing cell rich; focal chronic inflammation in stroma

Posterior approach, laminectomy C2-C4 and complete excision

Uneventful, no evidence of any recurrence

3 years/F

Paraplegia and back pain

20 days

C7-T1

MRI: Intramedullary round cyst, isointense to CSF

Cuboidal; mucin-producing cell poor; focal chronic inflammation in stroma

Posterior approach, laminectomy C6-T1 and incomplete excision

Uneventful, no evidence of any recurrence

10 years/M

Weakness of both lower limbs

1 month

C6-T2

MRI: Intradural extramedullary oval cyst, hyperintense to CSF

Columnar to cuboidal, ciliated; mucin-producing cell poor

Posterior approach, laminectomy C5-T2 and incomplete excision

Uneventful, no evidence of any recurrence

2 years/M

Neck pain and quadriplegia

2 months

C2-C6

MRI: Intradural extramedullary oval cyst, isointense to CSF

Cuboidal; mucin-producing cell rich

Posterior approach, laminectomy C2-C5 and complete excision

Uneventful, no evidence of any recurrence

5 years/M

Asymmetric weakness of lower limbs

21 days

T7

MRI: Intradural extramedullary round cyst, isointense to CSF

Columnar, ciliated; mucin-producing cell rich

Posterior approach, laminectomy C2-C5 and complete excision

Uneventful, no evidence of any recurrence

Sreedhar et al (2006)[13]

1 year 4 months/F

Deviation of the neck toward the left

15 days

8.4 × 4.3 × 6.7 cm

MRI: A large multiloculated cystic lesion in the pre- and paravertebral regions extending from C5-D8 level. It was hypointense on T1W1 and hyper intense on T2W1

A cyst lined by ciliated, tall columnar epithelium leading to transitional to squamous epithelium

Surgical excision in two stages (cervical component followed by thoracic component)

Uneventful, no evidence of any recurrence

Shenoy and Raja (2004)[14]

4 years/M

Progressive weakness in all limbs

2 weeks

C2-C3

MRI: A well-defined ventrally situated hypointense lesion opposite C2–C3 on the T1-weighted image causing flattening of the spinal cord

Cuboidal to columnar lining cells with ciliated and mucin-secreting cells

C2–C4 laminectomy followed by complete excision of cyst

Uneventful, no evidence of any recurrence

3 years/M

Neck pain, weakness of lower limbs

10 days

C7-T1

MRI: A well-defined, ventrally situated hypointense lesion on the T1-weighted image, causing compression of the spinal cord

C6–T3 laminectomy followed by complete excision of cyst

No significant neurological improvement

16 year/F

Neck pain

2 months

C3-C4

Myelogram: A widening of the cord shadow opposite C3–C4 level

C3–C4 laminectomy followed by complete excision of cyst

Uneventful, no evidence of any recurrence

5 years/ F

Sphincter incontinence and progressive difficulty in walking following a fall

3 months

T6-T8

MRI: A large dorsally placed well-defined hyperintense cystic lesion compressing the spinal cord both on the T1- and T2-weighted images

Laminectomy followed by excision

Recurrence at 3 years followed by reexcision

Kumar et al (2001)[15]

17 years/M

Painless progressive swelling in the lumbosacral region with weakness of lower limbs

6 months

L5

MRI: A brightly hyperintense and having a higher signal intensity than CSF on T2-weighted and hypointense on T1-weighted images, was seen at the level of L-5

Neurenteric cyst

Laminectomy L3-L5 with cyst excision

Uneventful, no evidence of any recurrence

18 years/M

Dribbling of urine since birth

18 years

L5 to sacral canal

MRI: A well-defined intradural homogeneous cystic lesion isointense to CSF on all sequences extending from L-5 down into the sacral canal

Neurenteric cyst

Laminectomy L5-S3 with cyst excision

Uneventful, no evidence of any recurrence

5 years/F

History of constipation and difficulty in passing urine (hesitancy and increased frequency and progressive paraparesis)

2 years

T3-T7

MRI: A large intradural extramedullary oval, cystic lesion extending from T-3 to T-7, which was hypointense on T1-weighted and hyperintense on T2-weighted images

Neurenteric cyst

Dorsal laminectomy from T-3 to T-7 with cyst excision

Uneventful, no evidence of any recurrence

Kadhim et al (2000)[16]

10 days/F

Continuous fever. At 8 weeks presented with sudden onset hypertonia and bilateral ankle clonus, decreased movements in the lower limbs and left arm, and urinary retention

10 days

T6-T9

Doppler ultrasonography of the spinal canal revealed an anterior cystic mass, and extending until the central canal of the spinal cord in an area displaying a laminated appearance

A mass of smooth muscles entrapping cavities lined by an epithelium of digestive and respiratory type

Thoracic laminectomy at T6-T9 followed by partial resection

At 18 months, fever, paraplegia, and neurologic bladder persisted whereas motor function of the left arm improved

Abbreviations: CSF, cerebrospinal fluid; F, female; M, male; MRI, magnetic resonance imaging.


Despite advancements in diagnostic and therapeutic modalities, several challenges persist in the management of pediatric neurenteric cysts. These include preoperative diagnosis, risk stratification, and long-term outcomes assessment. Future research endeavors should focus on elucidating the molecular mechanisms underlying cyst formation, refining diagnostic algorithms, and exploring minimally invasive treatment modalities to minimize surgical morbidity. The limitations of this study include the small sample size and the retrospective design, which limit the generalizability of findings. Furthermore, the short follow-up duration may not fully capture the risk of late recurrence. Larger, multicenter studies with long-term follow-up are needed to validate these findings and optimize management protocols.

Pediatric neurenteric cysts of the spine represent a rare but clinically significant pathological entity with diverse clinical presentations and therapeutic implications. Despite advancements in diagnostic and therapeutic modalities, several challenges persist in the management of pediatric neurenteric cysts. These include preoperative diagnosis, risk stratification, and long-term outcomes assessment. Future research endeavors should focus on elucidating the molecular mechanisms underlying cyst formation, refining diagnostic algorithms, and exploring minimally invasive treatment modalities to minimize surgical morbidity. Multicenter collaborative studies are warranted to establish consensus guidelines and optimize treatment strategies, ultimately improving outcomes for affected children. Comprehensive evaluation and multidisciplinary collaboration of medical specialities are needed for optimizing outcomes for affected patients.



Conflict of Interest

None declared.

Authors' Contributions

All authors contributed to the study conception and design. Material preparation, data collection, and analysis were performed by S.A. and S.Z. The first draft of the manuscript was written by S.A. and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.


Ethical Approval

The study was done in accordance with the Declaration of Helsinki.



Address for correspondence

Sufian Zaheer, MD
Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital
New Delhi 110029
India   

Publication History

Article published online:
24 November 2025

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Zoom
Fig. 1 Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) diagram.
Zoom
Fig. 2 Neurenteric cyst in the thoracic spine in an 11-month-old child. (A) Magnetic resonance imaging (T1) exhibiting a well-defined intradural extramedullary cystic lesion in the thoracic spine. (B) Intraoperative image of the cystic mass. (C, D) Hematoxylin and eosin-stained sections exhibited a cyst lined by cuboidal to columnar epithelium with goblet cells [40×, 100×].
Zoom
Fig. 3 Neurenteric cyst in the cervical spine in a 14-year-old female. (A) Magnetic resonance imaging (T1) exhibiting a well-defined cystic lesion centered in the cervical spine. (B) Intraoperative image of the cystic mass centered in the cervical spine. (C, D) Hematoxylin and eosin-stained sections exhibited a cyst lined by pseudostratified ciliated epithelium [40×, 100×].