Available via license: CC BY-NC-ND 4.0
Content may be subject to copyright.
Atlantoaxial limited dorsal myeloschisis: A report of two cases and review
of literature
Wai Cheong Soon
*
, Joe M. Das, Azam Baig, Pasquale Gallo, Desiderio Rodrigues
1
,
William B. Lo
1
Department of Neurosurgery, Birmingham Children's Hospital, Steelhouse Ln, Birmingham, B4 6NH, United Kingdom
ARTICLE INFO
Keywords:
Spinal dysraphism
Tethered cord syndrome
Myeloschisis
Cutaneous lesion
Atlantoaxial
ABSTRACT
Introduction: Limited dorsal myeloschisis (LDM) is a rare form of spinal dysraphism that is characterised by a
distinctive fibroneural stalk connecting the spinal cord to the overlying skin lesion. The skin lesions associated
with LDM can appear benign clinically and careful evaluation with an MRI scan is essential for diagnosing LDM
and to differentiate this entity from other forms of spinal dysraphism and benign causes of skin lesions.
Research question: There is a lack of reported atlantoaxial LDM in the literature. We sought to report the clinical
presentation, radiological features and surgical management of the first two reported atlantoaxial LDM.
Material and methods: Clinical findings and radiological images of the two cases of atlantoaxial LDM that un-
derwent surgical intervention at our institution were retrieved from the medical notes, operative records and
imaging system.
Results: Both cases of atlantoaxial LDM (C0-1 and C1-2 respectively) underwent successful resection of the
overlying cutaneous lesions and stalks to release the tethered spinal cords.
Discussion: The surgical management of LDM have been associated with good outcomes and consists of resecting
the fibroneural stalk close to the underlying cord, releasing the tethered spinal cord and removing the overlying
cutaneous lesion.
Conclusion: These are the first two reported cases of atlantoaxial LDM in the literature. We aim to raise awareness
of this pathological entity and highlight the importance of establishing the correct diagnosis to guide definitive
management, and report the favourable neurological outcome in these cases despite the rostral location.
1. Introduction
Limited dorsal myeloschisis (LDM) is a rare form of spinal dysraphism
characterised by a focal midline skin lesion and fibroneural pedicle or
stalk that connects the spinal cord to the overlying skin lesion (Pang
et al., 2013). This distinct clinicopathological entity was first described in
1993 and the persistent fibroneural pedicle has been hypothesised to be
the result of an incomplete dysjunction between cutaneous and neural
ectoderms (Pang et al., 2013). The surgical management of LDM involves
disconnecting the fibroneural stalk that links the spinal cord and the
dorsal cutaneous appendage in addition to untethering the spinal cord
(Batista Cezar-Junior et al., 2020). Limited dorsal myeloschisis in the
cervical region is a less common subgroup of LDM when compared to the
thoracolumbar spine. In this article, we present two cases of LDM
involving the atlantoaxial spine, which has not been reported in the
literature to date.
2. Cases
2.1. Case 1
A 3-year-old girl was referred with a midline ‘tail-like’cutaneous
appendage at the nape of her neck that was present since birth (Fig. 1a).
She was neurologically intact with no other stigmata of congenital
anomaly. She had an MRI scan at the age of 24 months which demon-
strated that the overlying cutaneous appendage was in continuity with
the tethered spinal cord (Fig. 1b and c). She underwent surgical resection
of the LDM and untethering of the spinal cord with continuous intra-
operative neuro-monitoring of motor-evoked and somatosensory-evoked
potentials at the age of 38 months. The fibroneural stalk from the base of
* Corresponding author.
E-mail address: wai.soon@nhs.net (W.C. Soon).
1
Joint senior authors.
Contents lists available at ScienceDirect
Brain and Spine
journal homepage: www.journals.elsevier.com/brain-and-spine
https://doi.org/10.1016/j.bas.2021.100298
Received 10 July 2021; Received in revised form 29 August 2021; Accepted 13 September 2021
Available online 26 September 2021
2772-5294/©2021 The Author(s). Published by Elsevier B.V. on behalf of EUROSPINE, the Spine Society of Europe, EANS, the European Association of Neurosurgical
Societies. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Brain and Spine 1 (2021) 100298
the appendage was followed through to the bony defect of the C2 lamina.
Limited bony decompression was performed around the defect until
normal dura was exposed. The dura was opened, and the fibrous tract
was visualised to extend intradurally (Fig. 1d). The spinal cord was
Fig. 1. a–‘Tail-like’skin appendage on the nape of the neck
Fig. 1b and c –Sagittal and axial MRI scan demonstrating the C1/2 limited dorsal myeloschisis with evidence of tethered cord. White arrow indicates the skin
appendage from which the stalk (black arrow) is going through the soft tissue. Fig. 1d–f–Fibrous stalk connecting the cutaneous appendage to the underlying spinal
cord (white arrow). The visible stalk (black arrow) was disconnected flush to the cord to release the tethered cord below the obex (dotted black arrow).
Fig. 2. a. Soft, pedunculated, transilluminable, mobile cystic swelling on the posterior aspect of the neck at the cranio-cervical junction.
Fig. 2b and c –Sagittal and axial MRI scan demonstrating the C0/1 LDM with associated tethering of the cord. Fig. 2d: and e The overlying cutaneous lesion was
connected to the deeper structures by a fibrolipomatous stalk. The deep portion of the stalk (white arrow) extended into the intradural space. Normal spinal cord
(black arrow) was visualised between the dural opening cranial to the stalk.
W.C. Soon et al. Brain and Spine 1 (2021) 100298
2
tethered 3 mm inferior to the obex (Fig. 1e). The stalk was fully excised
from the cord by sharp dissection and resected along with the cutaneous
appendage (Fig. 1f). The tethered cord was carefully released, and the
dura was closed in a watertight fashion. There were no compromise in
motor-evoked and somatosensory-evoked potentials throughout the
procedure. She remained neurologically well and was discharged on
postoperative day four. Histopathological examination of the excised
skin lesion and stalk was consistent with a benign fibroepithelial polyp
and fibroconnective tissue devoid of acute or chronic inflammation.
2.2. Case 2
A newborn baby boy with normal antenatal scans was noted to have a
42cm, soft, pedunculated, transilluminable, mobile cystic swelling on
the posterior aspect of the neck at the cranio-cervical junction (Fig. 2a).
He was neurologically intact, and the rest of his spine was normal. On day
one of life, an ultrasound of the swelling demonstrated a septated cystic
mass with possible attachments to posterior cervical muscles and no
deeper extension. On day three of life, he underwent an MRI scan which
revealed a C0/1 LDM with associated tethered cord and an overlying sac
measuring 11 27 21mm which contained meninges, CSF and fat
(Fig. 2b and c). He subsequently underwent excision of the LDM and
untethering of the cord on the 6th day of life. Intraoperatively, a fibro-
lipomatous stalk measuring 8mm in diameter that connected the cuta-
neous lesion to the underlying cord was identified. The stalk extended
deep through a midline defect in the ligamentum flavum and dura
(Fig. 2d and e). The dura was opened cranially and caudally to the stalk
that was subsequently divided. The cutaneous lesion, a thin rim of
normal skin and the superficial part of the stalk were removed en bloc.A
near-total resection of the abnormal tissue was performed, with a small
portion that was adhered to the cervico-medullary junction left in situ.
The cervical cord was untethered. The patient had an uneventful post-
operative course and was discharged 2 days later. Histopathological ex-
amination revealed neural tissue surrounded by fibrous tissue and blood
vessels. He remains neurologically well at subsequent follow-ups.
3. Discussion
Limited dorsal myeloschisis (LDM) is a rare form of closed spinal
dysraphism that can be associated with tethered cord, lipoma, lip-
omyelomeningocele, split cord malformation, teratoma and other
congenital anomalies (Donovan and Pedersen, 2005;Park et al., 2005;
Pang et al., 2010;Izci and Kural, 2019). The embryogenesis of LDM has
been proposed to be secondary to incomplete fusion of the neural folds in
primary neurulation resulting in incomplete separation between the
cutaneous and neural ectoderm (Pang et al., 2010). This theory is sup-
ported by Pang et al.’s study whereby all 51 cases in their series had
neural stalk attachment to the cord superior to the conus that forms
during secondary neurulation (Pang et al., 2010). However, in a more
recently published series by Kim et al., the authors reported that 11 out of
28 patients with LDMs had interspinous defect in levels below S1-2,
which is formed during secondary neurulation. Kim et al. hypothesised
that LDMs may occur during early stages of secondary neurulation due to
incomplete dysjunction between the caudal cell mass and the skin or
during later stages of secondary neurulation due to incomplete separa-
tion between the ‘terminal balloon’(CSF-filled dilated caudal end of the
secondary neural tube) and cutaneous ectoderm (Kim et al., 2020;Lee
et al., 2013). LDMs are more commonly seen in the thoracolumbar spine
when compared to the cervical spine (Pang et al., 2013;Izci and Kural,
2019;Chatterjee et al., 2021;Lafitte et al., 2018;Chatterjee and Rao,
2015;Mohindra, 2007;Lee et al., 2017). In Pang et al.’s series, all eleven
cervical LDMs were located in the subaxial C3-7 levels (Pang et al.,
2013). Our cases showed that LDM can occur in the atlantoaxial cervical
spine, which have not been previously reported. In Case 1, there was a
skin appendage, which perhaps be an external feature in its own right,
because it does not fit into the external features of saccular LDMs
described by Pang et al.’s(Pang et al., 2013). Case 2 was a typical
saccular LDM consisting of one myelocystocoele but was unique in that it
was the first C0/1 LDM. Both cases were diagnosed postnatally.
3.1. Proposed embryological explanation for rare occurrence
The reason for the rare occurrence of atlantoaxial LDM is unclear.
While it is likely to be underdiagnosed and underreported because it is
misdiagnosed as other cutaneous lesions, there may be other develop-
mental and embryological explanations. Embryological studies have
shown that human neural tube closure initiates at multiple sites (Nakatsu
et al., 2000). The closure at the occipito-cervical region results from the
joining of the closures from two initiation sites: Site A –cervical level,
Site B –mesencephalic-rhombencephalic boundary (Fig. 3). We postulate
that disruption of neural tube closure/dysjunction at the occipito-cervical
region is less likely than other regions, because if the process fails at one
of the two contributing initiation sites, the other process will still
continue at the other site. However, if a neural tube defect does occur, it
is likely to result in conditions much more severe than LDM, including
spontaneous abortion, as described by Nakatsu et al. as Type III defects
(Nakatsu et al., 2000).
3.2. Histopathological features of LDM
The histopathological examination of LDM stalk revealed glial
fibrillary acid protein (GFAP)-immunopositive neuroglial tissues in 50%
of cases (Morioka et al., 2019). Morioka et al. reported that peripheral
nerve fibres were observed in all of the six fibroneural stalks examined
and numerous melanocytes were found along the long-axis of the stalk in
three out of four patients with dermal melanocytosis (Morioka et al.,
2019). Dorsal root ganglion cells, fibrous bands, fat, skeletal muscles,
blood vessels and dermoid or neuroenteric cysts have been detected
surrounding the stalk and/or within the stalk (Pang et al., 2010). The
presence of dermoid elements has the potential to cause neurological
deterioration with development of dermoid cysts following partial
resection of LDM stalk (Eibach et al., 2017).
Fig. 3. Schematic diagram showing the current model for multi-site neural tube
closure in human embryo. The black line represents the craniocaudal arrange-
ment of the embryo. Open triangles indicate the three initiation sites: A –cervical
level, B –mesencephalic-rhombencephalic boundary, and C –rostral tip of the
neural groove. Arrows indicate the directions of neural tube closure. Dotted circle
indicates the occipito-cervical region where the reported atlantoaxial LDMs are
located. (Adapted from Nakatsu et al. (2000).
W.C. Soon et al. Brain and Spine 1 (2021) 100298
3
3.3. Antenatal radiological differential diagnosis of LDM
The antenatal diagnosis of LDM can often mimic myelomeningocele
(MMC) and it is important to differentiate between these two entities due
to the differences in prognosis (Friszer et al., 2017). In a prospective
prenatal ultrasound study by Friszer et al., the authors found that diag-
nosis of MMC was revised to LDM in 7 out of 29 cases (Friszer et al.,
2017). It is important to note that myelomeningocele is often associated
with cerebral abnormalities such as hydrocephalus or Chiari malforma-
tion (Morais et al., 2020;Russell et al., 2013). It is essential to establish
the correct diagnosis of LDM as missed LDM can lead to delayed neuro-
logical deficits, recurrent tethered cord syndrome and need for further
surgical intervention (Chatterjee and Rao, 2015). LDMs are usually
associated with a better prognosis in comparison to MMC and foetal MRI
should be performed if there is any diagnostic uncertainty (Pang et al.,
2013;Russell et al., 2013;Spoor et al., 2019). On the MRI scan, the
fibroneural stalk or tract can usually be traced from the base of the skin
lesion to the underlying cord (Pang et al., 2010).
3.4. Cutaneous stigmata of LDM
Skin tags are usually benign pedunculated lesions that can be found
anywhere on the body. However, the presence of midline dorsal skin tags
or neuroectodermal appendages is not always benign and should prompt
further investigations to detect the presence of underlying spinal dysra-
phism (Guggisberg et al., 2004). Pang et al. elegantly described that the
skin lesions associated with LDMs can be subdivided into four types
namely saccular, crater, pit and membranous sac with saccular skin le-
sions being most commonly found in cervical LDMs (Pang et al., 2010).
The ‘tail-like’appendage described in Case 1 of our series has not been
included in Pang et al.’s classification. Therefore, we propose an addition
of ‘appendage’to the four external features of the saccular subtype
described by Pang et al.: thick squamous top, thin squamous top, dome
pit, and membranous sac. There are other reported cases of ‘tail-like’
appendages in the literature (Batista Cezar-Junior et al., 2020;Sarukawa
et al., 2019;Gaskill and Marlin, 1989;Cai et al., 2011;Canaz et al., 2018;
Turk et al., 2016;Abe et al., 2020). By the 8th week of intrauterine life,
the tail of the human embryos usually regresses. Failure of regression the
distal remnant of the embryonic tail has been proposed to result in a
persistent ‘human-tail’, more commonly located in the lumbosacral re-
gion (Klinge et al., 2020;Mukhopadhyay et al., 2012). Including our
case, only two cases of ‘tail-like’appendages in the cervical region have
been reported in the literature to date (Mohindra, 2007) and the path-
oembryological explanation for these occurrences are unclear.
3.5. Surgical treatment and neurological outcome
The surgical management of LDM have been associated with good
outcomes and consists of resecting the fibroneural stalk flush/close to the
underlying cord, releasing the tethered spinal cord and removing the
overlying cutaneous lesion (Batista Cezar-Junior et al., 2020;Donovan
and Pedersen, 2005;Pang et al., 2010;Nakatsu et al., 2000;Russell et al.,
2013;Guggisberg et al., 2004;Sarukawa et al., 2019;Gaskill and Marlin,
1989;Cai et al., 2011;Canaz et al., 2018;Turk et al., 2016). It is essential
to open the dura to expose the intradural component of the stalk and
ensure that it is adequately disconnected from the cord. Based on Pang
et al.’s series, seven out of eleven patients with cervical LDM presented
with neurological deficit (Pang et al., 2013), which is in contrast to our
cases of two young children, who were both neurologically intact at
presentation. The difference may be explained by the young age of our
patients (38-months and 6-days-old respectively), which means subtle
neurological deficit or decline were not detected. Without releasing the
tethered cord caused by the fibroneural stalk, Pang et al. reported that
five out of six patients returned with progressive neurological deficits
(Pang et al., 2010). We are in agreement that fibroneural stalk should be
completely excised to minimise the risk of recurrence especially in the
presence of any dermoid elements, although our series of two have not
included cases of inadequate resection, and thus no direct comparison
can be made (Eibach et al., 2017).
The surgical management of LDM at the atlantoaxial spine poses an
additional risk of causing atlantoaxial instability as these levels are more
heavily reliant on the ligaments and facet joints to prevent dislocation
(Yang et al., 2014). Atlantoaxial instability is more common in the pae-
diatric population due to the increased ligamentous laxity, more angled
facet joints and incomplete ossification of the odontoid process when
compared to the adult population (Lustrin et al., 2003). At the level of C1,
the anterior arch usually completes fusion with the posterior neural
arches by 7 years of age. At the level of C2, posterior fusion of the neural
arches occur by 3 years of age and fusion of the neural arches with the
body of the odontoid process occurs between 3 and 6 years of age (Lustrin
et al., 2003). Therefore, careful bony exposure with preservation of the
facet joints and lateral masses should be performed to maintain stability
of the atlantoaxial spine. One should also be mindful of the close prox-
imity of V3 and V4 segments of the vertebral arteries as they pierce the
atlanto-occipital membrane and the dura to avoid vascular injury during
the surgical approach.
Due to the tethering of the cord by the fibroneural stalk, the risk of
neurological deficit increases over time with longitudinal growth of the
spine, even though the change in the relative positions between the
tethered cord and the overlying dura is modest in cervical cases (Pang
et al., 2010). Urgent surgery should be offered in the presence of cere-
brospinal fluid leakage and/or neurological deficit.
4. Conclusion
These are the two first reported cases of atlantoaxial LDM in the
literature. We advocate that all dorsal cervical midline cutaneous lesions
should be investigated further with MRI scan to look for evidence of
spinal dysraphism and tethered cord. The presence of a stalk or tract
between the cutaneous appendage and the spinal cord should be recog-
nised pre-operatively and complete disconnection and resection of the
fibroneural stalk should be performed to optimise surgical outcomes and
prevent delayed neurological deterioration.
Conflict of interest
On behalf of all authors, the corresponding author states that there is
no conflict of interest.
Consent
Verbal parental consents have been obtained for the publication of
this manuscript.
Declaration of competing interests
The authors declare that they have no known competing financial
interests or personal relationships that could have appeared to influence
the work reported in this paper.
Funding
None.
References
Abe, K., Mukae, N., Morioka, T., Shimogawa, T., Suzuki, S.O., Mizoguchi, M., 2020. Tail-
like cutaneous appendage at the upper thoracic region with a continuous stalk of
limited dorsal myeloschisis. Interdiscplinary Neurosurgery 20, 100823.
Batista Cezar-Junior, A., Faquini, I.V., Frank, K., Euripedes Almondes S Lemos, L., de
Carvalho, E.V., Junior, Almeida, N.S., Azevedo-Filho, H.R.C., 2020. Limited dorsal
myeloschisis with a contiguous stalk to human tail-like cutaneous appendage,
W.C. Soon et al. Brain and Spine 1 (2021) 100298
4
associated with a lipoma of conus medullaris: a Case report. Int J Surg Case Rep. 71,
303–306.
Cai, C., Shi, O., Shen, C., 2011. Surgical treatment of a patient with human tail and
multiple abnormalities of the spinal cord and column. Adv Orthop 153797.
Canaz, G., Akkoyun, N., Emel, E., Cevik, O.M., Baydin, S., Gokcedag, A., 2018. A rare case
of "human tail" associated with lipomyelomeningocele and tethered cord. J. Pediatr.
Neurosci. 13 (2), 241–244.
Chatterjee, S., Rao, K.S., 2015. Missed limited dorsal myeloschisis: an unfortunate cause
for recurrent tethered cord syndrome. Childs Nerv Syst 31 (9), 1553–1557.
Chatterjee, S., Rao, K.S.M., Nadkarni, A., 2021. Amniotic band syndrome associated with
limited dorsal myeloschisis: a Case report of an unusual case and review of the
literature. Childs Nerv Syst 37 (2), 707–713.
Donovan, D.J., Pedersen, R.C., 2005. Human tail with noncontiguous intraspinal lipoma
and spinal cord tethering: case report and embryologic discussion. Pediatr.
Neurosurg. 41 (1), 35–40.
Eibach, S., Moes, G., Zovickian, J., Pang, D., 2017. Limited dorsal myeloschisis associated
with dermoid elements. Childs Nerv Syst 33 (1), 55–67.
Friszer, S., Dhombres, F., Morel, B., Zerah, M., Jouannic, J.M., Garel, C., 2017. Limit ed
dorsal myeloschisis: a diagnostic pitfall in the prenatal ultrasound of fetal
dysraphism. Fetal Diagn. Ther. 41 (2), 136–144.
Gaskill, S.J., Marlin, A.E., 1989. Neuroectodermal appendages: the human tail explained.
Pediatr. Neurosci. 15 (2), 95–99.
Guggisberg, D., Hadj-Rabia, S., Viney, C., Bodemer, C., Brunelle, F., Zerah, M., Pierre-
Kahn, A., Prost, Y.D., Hamel-Teillac, D., 2004. Skin markers of occult spinal
dysraphism in Children: A review of 54 cases. Arch. Dermatol. 140 (9), 1109–1115.
Izci, Y., Kural, C., 2019. Limited dorsal myeloschisis with and without type I split cord
malformation: report of 3 cases and surgical nuances. Medicina (Kaunas) 55 (2), 28.
Kim, J.W., Wang, K.C., Chong, S., Kim, S.K., Lee, J.Y., 2020. Limited dorsal myeloschisis:
reconsideration of its embryological origin. Neurosurgery 86 (1), 93–100.
Klinge, P.M., Cho, D., Taylor, H.O., Morrison, C.S., Birgfield, C.B., Sullivan, S.R., 2020.
Human tails: interdisciplinary treatment for dorsal cutaneous appendages and
associated spectrum of spinal dysraphism. Interdisciplinary Neurosurgery 19,
100632.
Lafitte, A.S., Blouet, M., Belloy, F., Borha, A., Benoist, G., 2018. A Case of prenatally
diagnosed limited dorsal myeloschisis with good prognosis. J. Clin. Ultrasound 46
(4), 282–285.
Lee, J.Y., Kim, S.P., Kim, S.W., Park, S.H., Choi, J.W., Phi, J.H., Kim, S.K., Pang, D.,
Wang, K.C., 2013. Pathoembryogenesis of terminal myelocystocele: terminal balloon
in secondary neurulation of the chick embryo. Childs Nerv Syst 29 (9), 1683–1688.
Lee, J.Y., Chong, S., Choi, Y.H., Phi, J.H., Cheon, J.E., Kim, S.K., Park, S.H., Kim, I.O.,
Wang, K.C., 2017. Modification of surgical procedure for "probable" limited dorsal
myeloschisis. J. Neurosurg. Pediatr. 19 (5), 616–619.
Lustrin, E.S., Karakas, S.P., Ortiz, A.O., Cinnamon, J., Castillo, M., Vaheesan, K.,
Brown, J.H., Diamond, A.S., Black, K., Singh, S., 2003. Pediatric cervical spine:
normal anatomy, variants, and trauma. Radiographics 23 (3), 539–560.
Mohindra, S., 2007. The 'human tail' causing tethered cervical cord. Spinal Cord 45 (8),
583–585.
Morais, B.A., Solla, D.J.F., Yamaki, V.N., Ferraciolli, S.F., Alves, C.A.P.F., Cardeal, D.D.,
Matushita, H., Teixeira, M.J., 2020. Brain abnormalities in myelomeningocele
patients. Childs Nerv Syst 36 (7), 1507–1513.
Morioka, T., Suzuki, S.O., Murakami, N., Mukae, N., Shimogawa, T., Haruyama, H.,
Kira, R., Iihara, K., 2019. Surgical histopathology of limited dorsal myeloschisis with
flat skin lesion. Childs Nerv Syst 35 (1), 119–128.
Mukhopadhyay, B., Shukla, R.M., Mukhopadhyay, M., Mandal, K.C., Haldar, P.,
Benare, A., 2012. Spectrum of human tails: a report of six cases. J. Indian Assoc.
Pediatr. Surg. 17 (1), 23–25.
Nakatsu, T., Uwabe, C., Shiota, K., 2000. Neural tube closure in humans initiates at
multiple sites: evidence from human embryos and implications for the pathogenesis
of neural tube defects. Anat. Embryol. 201 (6), 455–466.
Pang, D., Zovickian, J., Oviedo, A., Moes, G.S., 2010. Limited dorsal myeloschisis: a
distinctive clinicopathological entity. Neurosurgery 67 (6), 1555–1579 discussion
1579-80.
Pang, D., Zovickian, J., Wong, S.T., Hou, Y.J., Moes, G.S., 2013. Limited dorsal
myeloschisis: a not-so-rare form of primary neurulation defect. Childs Nerv Syst 9,
1459-84.
Park, S.H., Huh, J.S., Cho, K.H., Shin, Y.S., Kim, S.H., Ahn, Y.H., Cho, K.G., Yoon, S.H.,
2005. Teratoma in human tail lipoma. Pediatr. Neurosurg. 41 (3), 158–161.
Russell, N.E., Chalouhi, G.E., Dirocco, F., Zerah, M., Ville, Y., 2013. Not all large neural
tube defects have a poor prognosis: a Case of prenatally diagnosed limited dorsal
myeloschisis. Ultrasound Obstet. Gynecol. 42 (2), 238–239.
Sarukawa, M., Morioka, T., Murakami, N., Shimogawa, T., Mukae, N., Kuga, N.,
Suzuki, So, Iihara, K., 2019. Human tail-like cutaneous appendage with a contiguous
stalk of limited dorsal myeloschisis. Childs Nerv Syst 35 (6), 973–978.
Spoor, J.K.H., Gadjradj, P.S., Eggink, A.J., DeKoninck, P.L.J., Lutters, B., Scheepe, J.R.,
van Meeteren, J., de Laat, P.C.J., van Veelen, M.L., de Jong, T.H.R., 2019.
Contemporary management and outcome of myelomeningocele: the Rotterdam
experience. Neurosurg. Focus (4), E3, 1;47.
Turk, C.C., Kara, N.N., Bacanli, A., 2016. The human tail: a simple skin appendage or
cutaneous stigma of an anomaly? Turk Neurosurg 26 (1), 140–145.
Yang, S.Y., Boniello, A.J., Poorman, C.E., Chang, A.L., Wang, S., Passias, P.G., 2014.
A review of the diagnosis and treatment of atlantoaxial dislocations. Global Spine J. 4
(3), 197–210.
W.C. Soon et al. Brain and Spine 1 (2021) 100298
5
