Cervical Diastematomyelia: A Case Presentation and Systematic Review

Abstract

Diastematomyelia is a rare congenital disorder characterized by the separation of the spinal cord by an osseocartilaginous or fibrous septum. While diastematomyelia has been reported to be more common in the thoracic and lumbar regions, the true incidence of cervical diastematomyelia is currently unknown. In this study, we conducted the most comprehensive systematic review to date of all other case reports of diastematomyelia to better characterize the incidence of cervical diastematomyelia and provide comprehensive statistics on the clinical characteristics of diastematomyelia generally. Ninety-one articles were included in our study, which comprised 252 males (27.9%) and 651 females (72.0%) (and one patient with unspecified gender). In 507 cases, the vertebral level of the diastematomyelia was described, and we recorded those levels as either cervical (n = 8, 1.6%), thoracic (n = 220, 43.4%), lumbar (n = 277, 54.6%), or sacral (n = 2, 0.4%). In 719 cases, the type of diastematomyelia was specified as either Type I (n = 482, 67.0%) or Type II (n = 237, 33.0%). Our study found that diastematomyelia has been reported in the cervical region in only 1.6% of cases, and we provide comprehensive data that this disorder occurs in female-to-male ratio of approximately 2.6:1 and Type I versus Type II diastematomyelia in an estimated ratio of 2:1.

 

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Keywords

diastematomyelia - neurosurgery - spinal cord malformation - spine - systematic review - Ukraine

Introduction

Diastematomyelia (also known as split cord malformation [SCM] or diplomyelia) is a rare congenital disorder characterized by the separation of the spinal cord by an osseocartilaginous or fibrous septum. While the total incidence of spinal dysraphism is estimated to be one to three cases per 1,000 live births,[1] the true incidence of diastematomyelia is unknown, though thought to occur in approximately 5% of congenital spine abnormalities.[2]

SCM is classified into two types: Type I SCM, in which the two hemicords are contained within two dural sacs divided by an osseous or cartilaginous septum, and Type II SCM, in which a single dural tube contains both hemicords separated by a fibrous median septum.[3] Diastematomyelia is usually diagnosed in childhood and associated with other congenital spine deformities in 85% of cases, such as scoliosis, tethered cord, syringomyelia, spina bifida, Chiari 2 malformation, spinal lipoma, or dermoid cyst.[4] Type I diastematomyelia is more frequently associated with other congenital anomalies than Type II, and surgical intervention is commonly indicated for Type I patients due to symptom progression resulting from impingement of the rigid septum on the spinal cord, associated adhesions, and increasing scoliosis.[5] Patients with Type II diastematomyelia usually only require surgery when there is a significant change in scoliosis or neurological function, and symptoms in these patients tend to be milder due to the midline septum being fibrous in composition.[5]

While diastematomyelia has been reported to be more common in the thoracic and lumbar regions, the incidence of cervical diastematomyelia is thought to be extremely rare, with very few cases presented in the medical literature. In this case study, we report a patient who presented to neurosurgery clinic in Lviv Ukraine for upper extremity radicular pain during a US–Ukraine neurosurgery partnership mission (the Co-Pilot Project)[6] and was found to have cervical diastematomyelia on imaging. We also present a thorough systematic review of all other case reports of diastematomyelia in the medical literature in order to better characterize the incidence of cervical diastematomyelia.

 

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Case Presentation

A 31-year-old female presented to the neurosurgery clinic with complaints of intermittent right shoulder pain with radiation down her arm and associated right upper extremity hypoesthesia. The patient noted that she had had these symptoms for many years but was concerned due to increased frequency and migration of the pain from her arm to the dorsal cervical region. The patient denied any medical conditions requiring medication or any family history of connective tissue or neurological diseases. The patient noted a surgical history of spina bifida treatment at 7 months of age (operative details for this surgery were unavailable), requiring 2 to 3 days of postoperative hospitalization and no complications at the time of discharge.

On physical examination, the patient had noted 4/5 right hand grip and 4/5 right arm extension weakness. Lower extremity motor and neurological functions were normal, but the patient noted that her right lower extremity was approximately 3 to 4 cm shorter than her left lower extremity. A small tuft of hair in the patient's dorsal cervical region was observed on examination ([Fig. 1]), from which the patient reported her muscle spasms and pain originated.

Fig. 1 Localized hypertrichosis on the patient's dorsal cervical region overlying the location of the diastematomyelia.

CT myelography was performed and revealed a noncontrast-enhancing bony lesion splitting the spinal cord into two asymmetric hemicords at the C6 to C7 vertebral levels ([Fig. 2]). No other structural anomalies were found on imaging.

Fig. 2 Coronal and axial views of the cervical vertebrae at the level of the diastematomyelia lesion.

Due to the patient's symptoms being well-controlled with occasional use of NSAIDs, surgical intervention was not thought to be warranted at the time of the interview. The patient agreed with the course of action and was counseled to seek physical therapy and follow-up for any progression of her symptoms.

 

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Methods

We conducted a systematic search using the PubMed database for all full-text reports in the English language describing patients with diastematomyelia. Searches were performed for all articles with the term “diastematomyelia” in their title. Studies were included in our review if patients described in the case report or series had a confirmed diagnosis of diastematomyelia on imaging. All relevant studies were reviewed and information related to the number of patient(s) described, their sex and age, the spinal level of the lesion, Type I versus Type II diastematomyelia, patient treatment, and clinical outcome were recorded. The search strategy used for study selection is represented by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses flow diagram in [Fig. 3].

Fig. 3 PRISMA flow diagram describing the search strategy used for study inclusion in this systematic review.

 

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Results

Two-hundred and fourteen results were provided by the PubMed database search. Of those 214 articles, 123 results were excluded due to the full article text not being available (n = 55), the article referring to a prenatal patient for whom a neurological examination was not possible (n = 24), the article not presenting a case report or series (n = 21), the article missing critical details (n = 19), or the article presenting a case of triplomyelia (n = 3) or diastematomyelia in a rachipagus twin (n = 1). Ninety-one studies matching the inclusion criteria described in the Methods were included for data collection and further analysis. These results are shown in [Table 1].

Table 1 Systematic review of cases of diastematomyelia

Author	n	Sex/Age	Spinal level	Type	Treatment	Outcome
Ritchie and Flanagan 1969[11]	8	M: 2, F: 6 1 wk–9 y	T: 4 L: 3 U: 1	II: 8	Surgery: 8	Improvement: 2/8
Huang et al, 2013[12]	156	M: 47, F: 109 Mean = 4.5 y	C: 2 T: 82 L: 72	I: 123 II: 33	Surgery: 121 nonsurgical: 35	Improvement: (I: 96/123), (II: 0/33)
Kachewar and Sankaye 2014[13]	2	M: 2 (17 y and 1 y)	T: 1 L: 1	U	U	U
Sack and Khan 2016[14]	1	F: 1 (29 y)	T: 1	II: 1	U	U
Gbadamosi et al, 2022[15]	1	F: 1 (U)	L: 1	I: 1	Nonsurgical	U
Russell et al, 1990[16]	45	M: 12, F: 33 Mean = 37.8 y	U	U	Surgical: 24 Nonsurgical: 21	Improvement: (surgical: 23/24)
Tizard 1957[17]	1	F: 1 (3 y)	C: 1	U	Nonsurgical	U
Saini and Singh 2009[18]	1	M: 1 (22 d)	L: 1	I: 1	U	U
Maebe et al, 2018[19]	1	F: 1 (72 y)	L: 1	I: 1	Nonsurgical	U
Hao et al, 2022[20]	1	F: 1 (18 y)	L: 1	I: 1	Surgical	Improvement
Bekki et al, 2015[21]	1	F: 1 (14 y)	T: 1	I: 1	Surgical	Improvement
Ge et al, 2020[22]	1	M: 1 (36 y)	L: 1	I: 1	Surgical	Improvement
Albulescu et al, 2016[23]	1	U: 1 (45 y)	L: 1	I: 1	U	U
Cheng et al, 2012[5]	138	M: 34, F: 104 Mean = 15.7 y	U	I: 106 II: 32	Surgical: 112 Nonsurgical: 26	Improvement: (surgical: I: 91/96), (surgical: II: 8/16)
Constantinou 1963[24]	1	F: 1 (23 y)	L: 1	I: 1	Nonsurgical	U
Hamidi and Foladi 2019[25]	1	M: 1 (48 y)	L: 1	I: 1	Nonsurgical	U
Apostolopoulou et al, 2021[26]	1	F: 1 (5 y)	L: 1	I: 1	Surgical	Improvement
Kapsalakis 1964[27]	2	F: 2 (6 y and 5 y)	L: 2	I: 2	Surgical	Improvement: 1/2
Vissarionov et al, 2018[28]	20	M: 8, F: 12 Mean = 9.2 y	T:15 L: 5	U	Surgical: 17 Nonsurgical: 3	Improvement: (surgical: 17/17)
Hood et al, 1980[29]	60	M: 13, F: 47 Mean = 4.7 y	T: 24 L: 36	U	Surgical: 51 Nonsurgical: 9	Improvement: (surgical: 20/51)
Meena et al, 2018[30]	1	F: 1 (15 mo)	L: 1	I: 1	Surgical	No improvement
Lersten et al, 2017[31]	1	F: 1 (50 y)	L: 1	I: 1	Nonsurgical	U
Winter et al, 1974[32]	27	M: 6, F: 21 Mean = 6.5 y	T: 8 L: 19	U	Surgical: 22 Nonsurgical: 5	Improvement: (surgical: 5/19)
Srinivasan et al, 2020[33]	1	F: 1 (55 y)	C: 1	II: 1	Nonsurgical	Improvement
Kim et al, 1994[34]	5	M: 3, F: 2 Mean = 14.8 y	L: 5	I: 5	Surgical: 5	Improvement: 4/5
Singh et al, 2015[35]	1	F: 1 (3 mo)	T: 1	I: 1	Surgical	U
Mamo et al, 2021[36]	1	M: 1 (50 y)	T: 1	I: 1	Nonsurgical	U
McNeil et al, 2018[37]	1	F: 1 (3 y)	L: 1	I: 1	U	U
Hader et al, 1999[38]	1	F: 1 (16 y)	L: 1	I: 1	Surgical	U
Alimli et al, 2015[39]	1	F: 1 (4 y)	T: 1	I: 1	Surgical	U
Huang et al, 2014[40]	82	M: 17, F: 65 Median = 6 y	T: 50 L: 32	I: 82	U	U
Khurram et al, 2021[41]	1	M: 1 (38 y)	L: 1	I: 1	U	U
Shorey 1955[42]	1	M: 1 (12 y)	L: 1	I: 1	Surgical	Improvement
Azimi and Mohammadi 2013[43]	1	M: 1 (53 y)	L: 1	I: 1	Surgical	Improvement
Gavriliu et al, 2014[44]	2	M: 1 (12 y), F: 1 (7 y)	T: 1 L: 1	I: 2	Surgical	Improvement: 1/2
Scotti et al, 1980[8]	21	M: 8, F: 13 Mean = 7.5 y	L: 21	I: 5 II: 15	Surgical: 15 Nonsurgical: 6	U
Kansal et al, 2011[45]	1	M: 1 (1.5 y)	L: 1	I: 1	Surgical	No improvement
English and Malthy 1967[46]	2	M: 1 (48 y), F: 1 (32 y)	L: 2	U	Surgical: 1 Nonsurgical: 1	Improvement: (surgical: 0/1) No improvement: (nonsurgical: U)
Patankar et al 2000[47]	1	F: 1 (5 y)	T: 1	I: 1	Surgical	Improvement
Gan et al 2007[48]	17	M: 8, F: 9 Mean = 3.4 y	T: 5 L: 12	I: 17	Surgical: 17	Improvement: 5/17
Yamanaka et al 2001[49]	1	U: 1 (6 d)	L: 1	I: 1	Surgical	Improvement
Beyerl et al 1985[50]	1	M: 1 (34 y)	C: 1	I: 1	Surgical	Improvement
Sandhu et al, 2021[51]	1	M: 1 (25 y)	T: 1	II: 1	Nonsurgical	Improvement
Chembolli 2015[52]	1	F: 1 (16 y)	T: 1	I: 1	Surgical	Improvement
Elmaci et al 2001[53]	1	M: 1 (42 y)	L:1	I: 1	Surgical	Improvement
Xu et al 2023[54]	1	F: 1 (17 y)	L: 1	I: 1	Surgical	Improvement
Zaleska-Dorobisz et al 2010[55]	1	F: 1 (78 y)	L: 1	I: 1	U	U
Sheehan et al,2002[56]	1	F: 1 (38 y)	T: 1	I: 1	Surgical	Improvement
Kanbur et al 2004[57]	1	M: 1 (12 y)	L: 1	I: 1	Nonsurgical	No improvement
Tubbs et al 2004[58]	1	F: 1 (18 y)	T: 1	I: 1	Surgical	No improvement
Shivapathasundram and Stoodley 2012[59]	1	F: 1 (8 y)	T: 1	I: 1	Surgical	Improvement
Parmar et al 2003[60]	1	F: 1 (34 y)	L: 1	I: 1	Surgical	Improvement
Senkoylu et al 2019[61]	1	F: 1 (4 y)	L: 1	I: 1	Surgical	Improvement
Tsitsopoulos et al 2006[62]	1	F: 1 (44 y)	L: 1	I: 1	Nonsurgical	No improvement
Pettorini et al 2007[63]	1	M: 1 (2 y)	T: 1	I: 1	Surgical	Improvement
Lewandrowski et al 2004[64]	1	F: 1 (44 y)	L: 1	I: 1	Surgical	Improvement
Ross et al 1988[65]	1	M: 1 (63 y)	S: 1	I: 1	Nonsurgical	No improvement
Porensky et al,2007[66]	1	F: 1 (54 y)	T: 1 L: 1	I: 2	Surgical	Improvement
Filippi et al 2010[67]	3	M: 1 (67 y), F: 2 (53 and 49 y)	T: 1 L: 2	I: 1 II: 2	U	U
Senel et al 2008[68]	1	F: 1 (14 mo)	T: 1	I: 1	Surgical	U
Shen et al 2016[69]	214	M: 61, F: 153 Mean = 14.2 y	U	I: 73 II: 141	Surgical: 214	U
Sharma et al 1997[70]	1	M: 1 (15 y), F: 1 (9 y)	T: 1 L: 1	I: 2	Surgical: 2	U
Sgouros 2010[71]	1	F: 1 (3 y)	L: 1	I: 1	Surgical	Improvement
Wenger et al, 2001[72]	1	F: 1 (38 y)	L: 1	II: 1	Nonsurgical	Improvement
Kilickesmez et al, 2004[73]	1	F: 1 (7 y)	L: 1	I: 1	Surgical	Improvement
Kaminker et al 2000[74]	1	M: 1 (38 y)	L: 1	I: 1	Surgical	Improvement
Ak et al, 2014[75]	1	F: 1 (10 y)	L: 1	I: 1	Surgical	Improvement
Ohwada et al, 1989[76]	1	M: 1 (29 y)	C: 1	I: 1	Surgical	Improvement
Morelli and Shalick 2011[77]	1	F: 1 (29 y)	L: 1	I: 1	Surgical	No improvement
Macht et al 2012[78]	1	M: 1 (57 y)	L: 1	I: 1	Nonsurgical	U
Kanagaraju et al 2016[79]	1	F: 1 (15 y)	L: 1	I: 1	Nonsurgical	Improvement
Boussaandani et al 2011[80]	1	F: 1 (33 y)	L: 1	I: 1	Nonsurgical	U
Armstrong et al, 2016[81]	1	F: 1 (49 y)	L: 1	I: 1	Nonsurgical	U
Giordano et al 2016[82]	1	F: 1 (43 y)	T: 1	II: 1	U	U
Sharma et al 2005[83]	1	F: 1 (18 mo)	L: 1	I: 1	Surgical	Improvement
Sedzimir et al, 1973[84]	1	M: 1 (22 mo)	L: 1	I: 1	Surgical	Improvement
Callari and Arrigo 2009[85]	2	F: 2 (80 and 59 y)	L: 2	U	Nonsurgical	Improvement: 2/2
Kramer et al, 2009[86]	1	F: 1 (54 y)	L: 1	I: 1	Surgical	No improvement
Roche and Vignaendra 2006[87]	8	M: 3, F: 5 Mean = 53.8 y	L: 7 S: 1	U	U	U
Uzumcugil et al 2003[88]	18	M: 2, F: 16 Mean = 20 mo	T: 9 L: 9	U	Surgical	U
Burnei et al 2015[89]	1	F: 1 (18 y)	L: 1	I: 1	Surgical	Improvement
Hung et al 2010[90]	1	F: 1 (2 d)	L: 1	I: 1	Surgical	U
Turgut and Doger 2008[91]	1	M: 1 (1 d)	L: 1	I: 1	Surgical	U
Bale 1973[92]	1	F: 1 (6 d)	T: 1	I: 1	Surgical	U
Korinth et al 2004[93]	1	M: 1 (2 y)	C: 1	I: 1	Surgical	No improvement
Mendez et al, 2009[94]	1	F: 1 (88 y)	L: 1	I: 1	Nonsurgical	Improvement
Yamada et al,1996[95]	1	F: 1 (2 y)	L: 1	I: 1	Surgical	Improvement
Lourie and Bierny 1970[96]	1	F: 1 (7 y)	T: 1	I: 1	Surgical	U
Ugarte et al, 1980[97]	2	F: 2 (1 d and 1 d)	T: 2	U	Surgical	Died: 2/2
Okada et al 1986[98]	1	M: 1 (19 y)	C: 1	I: 1	Nonsurgical	U
Azhar et al, 1996[99]	1	M: 1 (35 y)	L: 1	I: 1	Surgical	Improvement

Note: Systematic review of case reports and series describing patients with diastematomyelia. “n” number of patients described in the study, the sex (M = male, F = female, U = unknown) and age of included patients, the spinal location of the lesion (C = cervical, T = thoracic, L = lumbar, U = unknown), Type I versus Type II spinal cord malformation, treatment, and clinical outcome were recorded.

The 91 articles included in our study comprised reports from 904 total patients with a mean age of 23.1 ± 22.0 years. A total of 252 males (27.9%) and 651 females (72.0%) (and one patient with unspecified gender) were included in our study. In 507 cases, the vertebral level of the diastematomyelia was described, and we recorded those levels as either cervical (n = 8, 1.6%), thoracic (n = 220, 43.4%), lumbar (n = 277, 54.6%), or sacral (n = 2, 0.4%). In 719 cases, the type of diastematomyelia was specified as either Type I (n = 482, 67.0%) or Type II (n = 237, 33.0%). Of 529 patients for whom follow-up data were available, 420 patients (79.4%) underwent surgical treatment for diastematomyelia, and 305 of those patients (72.6%) reported improvement in their neurological symptoms postoperatively. These findings are presented in [Table 2].

Table 2 Patient demographics

	Population (n = 904)
Age (mean ± SD)	23.1 ± 22.0
Gender
Male (n, %)	252 (27.9)
Female (n, %)	651 (72.0)
Unspecified (n, %)	1 (0.1)
Vertebral level	507 (56.1)
Cervical (n, %)	8 (1.6)
Thoracic (n, %)	220 (43.4)
Lumbar (n, %)	277 (54.6)
Sacral (n, %)	2 (0.4)
Pang criteria	719 (79.5)
Type I (n, %)	482 (67.0)
Type II (n, %)	237 (33.0)
Surgical treatment	420 (79.4)
Improvement (n, %)	305 (72.6)
No improvement (n, %)	115 (27.4)

Abbreviation: SD, standard deviation.

Note: Patient Demographics. Determinations of clinical improvement following treatment were calculated from the n = 529 patients for whom follow-up data were provided.

 

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Discussion

Consistent with previous reports of diastematomyelia found in the medical literature, the incidence of cervical diastematomyelia was found to be extremely rare, accounting for only 1.6% of all cases of diastematomyelia. The results of our comprehensive review of the literature also found that diastematomyelia has an approximately 2.5:1 predilection for females versus males and occurs as Type I versus Type II SCM in a 2:1 ratio.

The etiology of diastematomyelia is uncertain but thought to be related to abnormalities in the formation of the neural tube during the 4th week of development.[5] Adhesions between ectodermal and endodermal tissues lead to the formation of an accessory neurenteric canal in the midline of the neural tube, which results in the separation of the growing spinal cord into two hemicords as the notochord elongates rostrally.[7] These adhesions simultaneously prevent the complete involution of fibrous septations and developmental fistulas, resulting in the formation of cysts, lipomas, and fistulas[8] and cause disruptions in the associated development of the surrounding vertebrae, accounting for the high proportion of comorbid spinal malformations seen in diastematomyelia patients.[5] The accessory canal then forms the basis for the migration of mesenchymal cells which subsequently develop into the bony or cartilaginous septa seen in Type I SCM patients.[9]

While the majority of patients undergoing surgical treatment for diastematomyelia (72.6%) saw improvements in pain severity, motor function, and/or neurological symptoms, the performance of prophylactic surgery for patients with incidental findings of diastematomyelia (particularly patients with Type I SCM) on imaging is controversial.[8] Surgical removal of an osseous septum can cause damage to the spinal cord, especially in young children, and has been reported to result in postoperative worsening of neurological symptoms in a few cases.[10] Though other studies have shown that postsurgical prognoses for patients with Type I SCM are significantly improved compared to patients with Type II SCM,[5] these qualifications of “improvement” versus “no improvement” compared to preoperative status are frequently complicated in studies by patients who had minimal symptoms prior to surgery (especially in patients with Type II SCM). Additionally, it has been suggested that the symptoms characteristic of diastematomyelia are related to some intrinsic myelodysplasia resulting from abnormal development rather than the presence of a bony spur in itself, as the location of a spur (resulting in the asymmetric compression of one hemicord) is not in itself predictive of the laterality or severity of symptoms.[8] This is also corroborated by reports of patients with Type II SCM who did not have any spur detected at all on imaging but nonetheless complained of significant neurological symptoms.[8]

Limitations of our study include an inability to confirm diagnoses of diastematomyelia from an independent review of imaging in all of our included cases, and the difficulty to adequately distinguish surgical outcomes for patients with Type I versus Type II SCM due to inconsistent reporting of results across studies and the often mixture of these two patient populations in the studies that did report surgical outcomes. The decision for surgical treatment is currently based on symptom severity or when necessary in the context of correcting concurrent spinal deformities. The establishment of clearer guidelines for surgical intervention for diastematomyelia requires further studies and trials beyond the scope of this present review.

 

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Conclusion

Cervical diastematomyelia is an extremely rare condition, accounting for 1.6% of all cases of diastematomyelia. Clinical correlations for establishing more rigorous guidelines related to surgical intervention in cases of diastematomyelia require further studies to clarify best practices.

 

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Conflict of Interest

None declared.

Acknowledgments

We would like to thank Razom for Ukraine for providing the organizational and travel support to make this neurosurgery mission trip to Ukraine possible. We would also like to thank the neurosurgical and operative staff of the Clinical Municipal Communal Emergency Hospital and St. Nicholas Children's Hospital of Lviv for their kindness, bravery, and generosity during our time in Ukraine.

Authors' Contributions

Conceptualization: All authors.

Data curation: J.F.Z.

Formal analysis: J.F.Z.

Funding acquisition: N/A

Investigation: All authors.

Methodology: All authors.

Project administration: J.A.F., O.F., and L.D.T.

Resources: N/A

Supervision: J.A.F., O.F., and L.D.T.

Validation: All authors.

Writing—original draft: J.F.Z., O.S., and O.K.

Writing—reviewing and editing: All authors.

Informed Consent

Written and verbal consents were obtained from all patients or their health care proxies for all aspects related to this report and prior to any procedures which were performed.

Data Sharing

Data supporting the findings of this study will be made available by the corresponding author upon request.

^* Both contributed equally as co-second authors.

• References

• 1 Rossi A. Imaging in Spine and Spinal Cord Developmental Malformations. Clinical Neuroradiology: The ESNR Textbook. Springer; 2018
• 2 Özek MM, Cinalli G, Maixner WJ, Maixner W. Spina Bifida: Management and Outcome. Springer Science & Business Media; 2008
• 3 Pang D, Dias MS, Ahab-Barmada M. Split cord malformation: Part I: a unified theory of embryogenesis for double spinal cord malformations. Neurosurgery 1992; 31 (03) 451-480
• 4 Ross JS, Moore KR. Diagnostic Imaging: Spine. 3rd ed.. Elsevier; 2016
• 5 Cheng B, Li FT, Lin L. Diastematomyelia: a retrospective review of 138 patients. J Bone Joint Surg Br 2012; 94 (03) 365-372
• 6 Tomycz LD, Markosian C, Kurilets Sr I. et al. The Co-Pilot Project: an international neurosurgical collaboration in Ukraine. World Neurosurg 2021; 147: e491-e515
• 7 Hawryluk GWJ, Ruff CA, Fehlings MG. Chapter 1—Development and Maturation of the Spinal Cord: Implications of Molecular and Genetic Defects. vol 109. Spinal Cord Injury. Elsevier; 2012
• 8 Scotti G, Musgrave MA, Harwood-Nash DC, Fitz CR, Chuang SH. Diastematomyelia in children: metrizamide and CT metrizamide myelography. AJR Am J Roentgenol 1980; 135 (06) 1225-1232
• 9 Leung YL, Buxton N. Combined diastematomyelia and hemivertebra: a review of the management at a single centre. J Bone Joint Surg Br 2005; 87 (10) 1380-1384
• 10 Goldberg C, Fenelon G, Blake NS, Dowling F, Regan BF. Diastematomyelia: a critical review of the natural history and treatment. Spine 1984; 9 (04) 367-372
• 11 Ritchie GW, Flanagan MN. Diastematomyelia. Can Med Assoc J 1969; 100 (09) 428-433
• 12 Huang SL, He XJ, Wang KZ, Lan BS. Diastematomyelia: a 35-year experience. Spine 2013; 38 (06) E344-E349
• 13 Kachewar SG, Sankaye SB. Diastematomyelia—a report of two cases. J Clin Diagn Res 2014; 8 (04) RE01-RE02
• 14 Sack AM, Khan TW. Diastematomyelia: split cord malformation. Anesthesiology 2016; 125 (02) 397
• 15 Gbadamosi WA, Daftari A, Szilagyi S. Focal diastematomyelia in an adult: a case report. Cureus 2022; 14 (06) e26081
• 16 Russell NA, Benoit BG, Joaquin AJ. Diastematomyelia in adults. A review. Pediatr Neurosurg 1990-1991 16 (4-5): 252-257
• 17 Tizard JP. Diastematomyelia. Proc R Soc Med 1957; 50 (05) 330
• 18 Saini HS, Singh M. Diastematomyelia. A case report. Neuroradiol J 2010; 23 (01) 126-129
• 19 Maebe H, Viaene A, De Muynck M. Diastematomyelia and late onset presentation: a case report of a 72-year-old woman. Eur J Phys Rehabil Med 2018; 54 (04) 618-621
• 20 Hao S, Yue Z, Yu X. et al. Case report: Type I diastematomyelia with breast abnormalities and clubfoot. Front Surg 2022; 9: 981069
• 21 Bekki H, Morishita Y, Kawano O, Shiba K, Iwamoto Y. Diastematomyelia: a surgical case with long-term follow-up. Asian Spine J 2015; 9 (01) 99-102
• 22 Ge CY, Hao DJ, Shan LQ. Rare bony diastematomyelia associated with intraspinal teratoma. World Neurosurg 2020; 133: 185-187
• 23 Albulescu D, Albu C, Constantin C, Stoica Z, Nicolescu I. Diastematomyelia—imaging findings, case report. Curr Health Sci J 2016; 42 (01) 94-96
• 24 Constantinou E. A case of diastematomyelia. JAMA 1963; 185: 983-984
• 25 Hamidi H, Foladi N. Misdiagnosed adult presentation of diastematomyelia and tethered cord. Radiol Case Rep 2019; 14 (09) 1123-1126
• 26 Apostolopoulou K, Andalib A, Zaki H, deLacy P. Diastematomyelia type I associated with intramedullary lipoma and dermoid cyst. Childs Nerv Syst 2021; 37 (09) 2949-2952
• 27 Kapsalakis Z. Diastematomyelia in two sisters. J Neurosurg 1964; 21: 66-67
• 28 Vissarionov SV, Krutelev NA, Snischuk VP. et al. Diagnosis and treatment of diastematomyelia in children: a perspective cohort study. Spinal Cord Ser Cases 2018; 4: 109
• 29 Hood RW, Riseborough EJ, Nehme AM, Micheli LJ, Strand RD, Neuhauser EB. Diastematomyelia and structural spinal deformities. J Bone Joint Surg Am 1980; 62 (04) 520-528
• 30 Meena RK, Doddamani RS, Sharma R. Contiguous diastematomyelia with lipomyelomeningocele in each hemicord—an exceptional case of spinal dysraphism. World Neurosurg 2019; 123: 103-107
• 31 Lersten M, Duhon B, Laker SR. Diastematomyelia as an incidental finding lumbar on magnetic resonance imaging. PM R 2017; 9 (01) 95-97
• 32 Winter RB, Haven JJ, Moe JH, Lagaard SM. Diastematomyelia and congenital spine deformities. J Bone Joint Surg Am 1974; 56 (01) 27-39
• 33 Srinivasan ES, Mehta VA, Smith GC, Than KD, Terry AR. Klippel-Feil syndrome with cervical diastematomyelia in an adult with extensive cervicothoracic fusions: case report and review of the literature. World Neurosurg 2020; 139: 274-280
• 34 Kim SK, Chung YS, Wang KC, Cho BK, Choi KS, Han DH. Diastematomyelia—clinical manifestation and treatment outcome. J Korean Med Sci 1994; 9 (02) 135-144
• 35 Singh N, Singh DK, Kumar R. Diastematomyelia with hemimyelomeningocele: an exceptional and complex spinal dysraphism. J Pediatr Neurosci 2015; 10 (03) 237-239
• 36 Mamo G, Batra R, Steinig J. A case of diastematomyelia presenting with minimal neurologic deficits in a middle-aged patient. Cureus 2021; 13 (01) e12621
• 37 McNeil AG, Jose S, Rowland-Hill C. Diastematomyelia in a 3-year-old girl. Arch Dis Child 2018; 103 (07) 683-684
• 38 Hader WJ, Steinbok P, Poskitt K, Hendson G. Intramedullary spinal teratoma and diastematomyelia. Case report and review of the literature. Pediatr Neurosurg 1999; 30 (03) 140-145
• 39 Alimli AG, Oztunali C, Boyunaga OL. et al. Diastematomyelia with the owl sign (Type I split cord malformation). Spine J 2015; 15 (10) e17-e19
• 40 Huang SL, He XJ, Xiang L, Yuan GL, Ning N, Lan BS. CT and MRI features of patients with diastematomyelia. Spinal Cord 2014; 52 (09) 689-692
• 41 Khurram R, Ahmadi F, Poonawala R, Yasin AS. Horseshoe adrenal gland associated with type 1 diastematomyelia in an asymptomatic adult. BJR Case Rep 2021; 7 (03) 20200188
• 42 Shorey WD. Diastematomyelia associated with dorsal kyphosis producing paraplegia. J Neurosurg 1955; 12 (03) 300-305
• 43 Azimi P, Mohammadi HR. Diastematomyelia presenting with no pain in a 53-year-old man: a case report. Iran Red Crescent Med J 2013; 15 (06) 522-525
• 44 Gavriliu S, Vlad C, Georgescu I, Burnei G. Diastematomyelia in congenital scoliosis: a report of two cases. Eur Spine J 2014; 23 (Suppl. 02) 262-266
• 45 Kansal R, Mahore A, Kukreja S. Jarcho-Levin syndrome with diastematomyelia: a case report and review of literature. J Pediatr Neurosci 2011; 6 (02) 141-143
• 46 English WJ, Maltby GL. Diastematomyelia in adults. J Neurosurg 1967; 27 (03) 260-264
• 47 Patankar T, Krishnan A, Patkar D, Armao D, Mukherji SK. Diastematomyelia and epidermoid cyst in the hemicord. AJR Am J Roentgenol 2000; 174 (06) 1793-1794
• 48 Gan YC, Sgouros S, Walsh AR, Hockley AD. Diastematomyelia in children: treatment outcome and natural history of associated syringomyelia. Childs Nerv Syst 2007; 23 (05) 515-519
• 49 Yamanaka T, Hashimoto N, Sasajima H, Mineura K. A case of diastematomyelia associated with myeloschisis in a hemicord. Pediatr Neurosurg 2001; 35 (05) 253-256
• 50 Beyerl BD, Ojemann RG, Davis KR, Hedley-Whyte ET, Mayberg MR. Cervical diastematomyelia presenting in adulthood. Case report. J Neurosurg 1985; 62 (03) 449-453
• 51 Sandhu J, Gupta SK, Katha M. An unusual case of faun tail nevus with aplasia cutis, dermo-fascial sinus defect, diastematomyelia, and spinal cord syrinx. Indian J Dermatol 2021; 66 (03) 322-324
• 52 Chembolli L. Faun tail overlying spinal dysraphism (diastematomyelia) at the mid thoracic level: cosmetic improvement achieved with diode laser epilation. Indian J Dermatol 2015; 60 (06) 638
• 53 Elmaci I, Dagcinar A, Ozgen S, Ekinci G, Pamir MN. Diastematomyelia and spinal teratoma in an adult. Case report. Neurosurg Focus 2001; 10 (01) ecp2
• 54 Xu L, Ma C, Shen S, Duan H, Li X. A heterozygous mutation in the ALPL gene in an adolescent with Chiari malformation type I accompanied by scoliosis, tethered cord and diastematomyelia. Acta Neurol Belg 2023; 123 (06) 2387-2389
• 55 Zaleska-Dorobisz U, Bladowska J, Biel A, Pałka LW, Hołownia D. MRI diagnosis of diastematomyelia in a 78-year-old woman: case report and literature review. Pol J Radiol 2010; 75 (02) 82-87
• 56 Sheehan JP, Sheehan JM, Lopes MB, Jane Sr JA. Thoracic diastematomyelia with concurrent intradural epidermoid spinal cord tumor and cervical syrinx in an adult. Case report. J Neurosurg 2002; 97 (2, Suppl) 231-234
• 57 Kanbur NO, Güner P, Derman O, Akalan N, Cila A, Kutluk T. Diastematomyelia: a case with familial aggregation of neural tube defects. ScientificWorldJournal 2004; 4: 847-852
• 58 Tubbs RS, Smyth MD, Dure LS, Oakes WJ. Exclusive lower extremity mirror movements and diastematomyelia. Pediatr Neurosurg 2004; 40 (03) 132-135
• 59 Shivapathasundram G, Stoodley MA. Use of a synthetic dural substitute to prevent ventral retethering in the management of diastematomyelia. J Clin Neurosci 2012; 19 (04) 578-581
• 60 Parmar H, Patkar D, Shah J, Maheshwari M. Diastematomyelia with terminal lipomyelocystocele arising from one hemicord: case report. Clin Imaging 2003; 27 (01) 41-43
• 61 Senkoylu A, Cetinkaya M, Aktas E, Cetin E. Excision and short segment fusion of a double ipsilateral lumbar hemivertebrae associated with a diastematomyelia and fixed pelvic obliquity. Acta Orthop Traumatol Turc 2019; 53 (02) 160-164
• 62 Tsitsopoulos P, Rizos C, Isaakidis D, Liapi G, Zymaris S. Coexistence of spinal intramedullary teratoma and diastematomyelia in an adult. Spinal Cord 2006; 44 (10) 632-635
• 63 Pettorini BL, Massimi L, Cianfoni A, Paternoster G, Tamburini G, Di Rocco C. Thoracic lipomeningocele associated with diastematomyelia, tethered spinal cord, and hydrocephalus. Case report. J Neurosurg 2007; 106 (5, Suppl) 394-397
• 64 Lewandrowski KU, Rachlin JR, Glazer PA. Diastematomyelia presenting as progressive weakness in an adult after spinal fusion for adolescent idiopathic scoliosis. Spine J 2004; 4 (01) 116-119
• 65 Ross GW, Swanson SA, Perentes E, Urich H. Ectopic midline spinal ganglion in diastematomyelia: a study of its connections. J Neurol Neurosurg Psychiatry 1988; 51 (09) 1231-1234
• 66 Porensky P, Muro K, Ganju A. Adult presentation of spinal dysraphism and tandem diastematomyelia. Spine J 2007; 7 (05) 622-626
• 67 Filippi CG, Andrews T, Gonyea JV, Linnell G, Cauley KA. Magnetic resonance diffusion tensor imaging and tractography of the lower spinal cord: application to diastematomyelia and tethered cord. Eur Radiol 2010; 20 (09) 2194-2199
• 68 Senel E, Tiryaki T, Atayurt H, Cansu A, Güç T. Lumbo-costovertebral syndrome with diastematomyelia. Pediatr Int 2008; 50 (04) 600-602
• 69 Shen J, Zhang J, Feng F, Wang Y, Qiu G, Li Z. Corrective surgery for congenital scoliosis associated with split cord malformation: it may be safe to leave diastematomyelia untreated in patients with intact or stable neurological status. J Bone Joint Surg Am 2016; 98 (11) 926-936
• 70 Sharma A, Sharma R, Goyal M, Vashisht S, Berry M. Diastematomyelia associated with intramedullary tumour in a hemicord: a report of two cases. Australas Radiol 1997; 41 (02) 185-187
• 71 Sgouros S. Acquired Chiari I malformation in a child with corrected diastematomyelia disappeared after thickened filum division. Pediatr Neurosurg 2010; 46 (05) 402-405
• 72 Wenger M, Hauswirth CB, Brodhage RP. Undiagnosed adult diastematomyelia associated with neurological symptoms following spinal anaesthesia. Anaesthesia 2001; 56 (08) 764-767
• 73 Kiliçkesmez O, Barut Y, Tasdemiroglu E. Expanding occult intrasacral meningocele associated with diastematomyelia and multiple vertebral anomalies. Case report. J Neurosurg 2004; 101 (1, Suppl) 108-111
• 74 Kaminker R, Fabry J, Midha R, Finkelstein JA. Split cord malformation with diastematomyelia presenting as neurogenic claudication in an adult: a case report. Spine 2000; 25 (17) 2269-2271
• 75 Ak H, Atalay T, Gülşen I. The association of the epidermoid cyst of the filum terminale, intradural spinal lipoma, tethered cord, dermal sinus tract, and type I diastematomyelia in a child. World Neurosurg 2014; 82 (06) e836-e837
• 76 Ohwada T, Okada K, Hayashi H. Thoracic myelopathy caused by cervicothoracic diastematomyelia. A case report. J Bone Joint Surg Am 1989; 71 (02) 296-299
• 77 Morelli C, Schalick III WO. Diastematomyelia presenting in adulthood as back pain. Am J Phys Med Rehabil 2013; 92 (09) 838
• 78 Macht S, Chapot R, Bieniek F, Hänggi D, Turowski B. Unique sacral location of an arteriovenous fistula of the filum terminale associated with diastematomyelia and lowered spinal cords. Neuroradiology 2012; 54 (05) 517-519
• 79 Kanagaraju V, Chhabra HS, Srivastava A. et al. A case of severe and rigid congenital thoracolumbar lordoscoliosis with diastematomyelia presenting with type 2 respiratory failure: managed by staged correction with controlled axial traction. Eur Spine J 2016; 25 (10) 3034-3041
• 80 Boussaadani Soubai R, Tahiri L, Sqalli Houssaini G, Mansouri S, Harzy T. Adult presentation of diastematomyelia: a case report. Joint Bone Spine 2011; 78 (05) 529-530
• 81 Armstrong DJ, McCormick D, O'Longain D. Previously undiagnosed diastematomyelia with bony spur as a cause of back pain in a 49-year-old patient with known psoriatic arthritis. Rheumatology (Oxford) 2017; 56 (02) 238
• 82 Giordano N, Cicone C, Hadilaksono MG, Agarwal S, Kifle G. Poster 375 adult onset lumbar radiculopathy secondary to Type II diastematomyelia: a case report. PM R 2016; 8 (9S): S283
• 83 Sharma MC, Sarat Chandra P, Goel S, Gupta V, Sarkar C. Primary lumbosacral Wilms tumor associated with diastematomyelia and occult spinal dysraphism. A report of a rare case and a short review of literature. Childs Nerv Syst 2005; 21 (03) 240-243
• 84 Sedzimir CB, Roberts JR, Occleshaw JV. Massive diastematomyelia without cutaneous dysraphism. Arch Dis Child 1973; 48 (05) 400-402
• 85 Callari G, Arrigo A. Diastematomyelia in adults. A report of two cases incidentally discovered. Neuroradiol J 2009; 22 (04) 448-451
• 86 Kramer JL, Dvorak M, Curt A. Thoracic disc herniation in a patient with tethered cord and lumbar syringomyelia and diastematomyelia: magnetic resonance imaging and neurophysiological findings. Spine 2009; 34 (14) E484-E487
• 87 Roche J, Vignaendra D. Midline septa in the lumbo-sacral thecal sac: acquired abnormality or developmental anomaly? The equivalent of diastematomyelia occurring below the spinal cord?. Australas Radiol 2006; 50 (06) 553-562
• 88 Uzumcugil A, Cil A, Yazici M. et al. The efficacy of convex hemiepiphysiodesis in patients with iatrogenic posterior element deficiency resulting from diastematomyelia excision. Spine 2003; 28 (08) 799-805
• 89 Burnei G, Gavriliu TS, Vlad C, Japie EM, Ghita RA. L3-L5 teratological spondylolysis with diastematomyelia and L4 radicular syndrome followed by spondyloschisis without myelomeningocele due to somatoarcuate shifting. Spine J 2015; 15 (01) 202-204
• 90 Hung PC, Wang HS, Lui TN, Wong AM. Sonographic findings in a neonate with diastematomyelia and a tethered spinal cord. J Ultrasound Med 2010; 29 (09) 1357-1360
• 91 Turgut M, Döğer FK. A case of diastematomyelia associated with hamartoma masquerading as meningocele in the newborn infant. Pediatr Neurosurg 2008; 44 (01) 85-87
• 92 Bale PM. A congenital intraspinal gastroenterogenous cyst in diastematomyelia. J Neurol Neurosurg Psychiatry 1973; 36 (06) 1011-1017
• 93 Korinth MC, Kapser A, Nolte K, Gilsbach JM. Cervical diastematomyelia associated with an intradural epidermoid cyst between the hemicords and multiple vertebral body anomalies. Pediatr Neurosurg 2004; 40 (05) 253-256
• 94 Méndez JC, Prieto MA, Lanciego C. Percutaneous vertebroplasty in a patient with type I split cord malformation (diastematomyelia). Cardiovasc Intervent Radiol 2009; 32 (03) 608-610
• 95 Yamada S, Mandybur GT, Thompson JR. Dorsal midline proboscis associated with diastematomyelia and tethered cord syndrome. Case report. J Neurosurg 1996; 85 (04) 709-712
• 96 Lourie H, Bierny JP. Diastematomyelia with two spurs and intradural neural crest elements. Case report. J Neurosurg 1970; 32 (02) 248-251
• 97 Ugarte N, Gonzalez-Crussi F, Sotelo-Avila C. Diastematomyelia associated with teratomas. Report of two cases. J Neurosurg 1980; 53 (05) 720-725
• 98 Okada K, Fuji T, Yonenobu K, Ono K. Cervical diastematomyelia with a stable neurological deficit. Report of a case. J Bone Joint Surg Am 1986; 68 (06) 934-937
• 99 Azhar MM, Winter RB, Dunn MB. Congenital spine deformity, congenital stenosis, diastematomyelia, and tight filum terminale in a workmen's compensation patient: a case report. Spine 1996; 21 (06) 770-774

Publication History

Received: 17 October 2023

Accepted: 29 February 2024

Accepted Manuscript online:
04 May 2024

Article published online:
24 May 2024