Of the infants with spina bifida referred to a neonatal surgical unit over three years and denied early

closure of the myelocele, a significant proportion survived long enough for procedures to drain
hydrocephalus to be needed. The key decision in the management of this condition is not whether
myeloceles should be closed but whether hydrocephalus should be treated.

Embryology

Embrio terdiri dari dua lapisan sel dalam 10 hingga 14 hari pertama, sel epiblas di lapisan atas dan sel
hipoblas di lapisan bawah. Gastrulasi dimulai sekitar hari ke-14 kehamilan. Selama waktu ini, sepanjang
garis primitif (alur yang terletak di sepanjang sepertiga ekor cakram embrionik) beberapa sel epiblas
meluncur sebagian besar lateral dan membentuk dua lapisan baru ventral ke epiblas yang tersisa,
mengubah embrio dari dua lapis menjadi tiga lapis; Sel-sel pertama melalui garis primitif menggantikan
hipoblas asli untuk membentuk endoderm, sementara sel-sel yang bermigrasi sedikit kemudian
menciptakan lapisan tengah baru, mesoderm. Sel-sel yang tersisa di epiblas yang tidak bermigrasi
melalui garis primitif membentuk ektoderm. Selama tahap ini, garis primitif menebal di ujung cephalic
untuk membentuk node Henson. Beberapa sel invaginating bermigrasi sepanjang sumbu kraniocaudal
garis tengah dari garis primitif untuk membentuk proses notochordal.

Pada hari ke 20, ada resorpsi lantai proses notochordal yang mengarah ke transformasi pelat prochordal
menjadi notochord. Sumbu primitif dan kerangka embrio didefinisikan oleh notochord, yang akhirnya
digantikan oleh kolom vertebral. Notochord membentang sepanjang embrio, meluas ke tingkat otak
tengah masa depan yang berakhir di wilayah dorsum sella masa depan. Notochord juga merangsang
ektoderm di atasnya sehingga menimbulkan neuroektoderm dan perkembangan lempeng saraf. Antara
minggu 3 dan 4, "landak sonik" (protein yang dilepaskan oleh notochord) memainkan peran kunci dalam
memulai neurulasi primer dengan menginduksi perkembangan sinyal lempeng saraf neuron motorik.

The neural plate transforms into a neural groove between days 18 and 20, once neural folds meet in the
midline, they undergo closure at day. Two sites of closure of the neural folds and two neuropores are
defined. The open parts of the neural tube are named anterior and posterior neuropores, after the
closure starts. The zippering proceeds caudally until the posterior neuropore is closed. But, in the cranial
region a second site of closure appears in the forebrain.

Lempeng saraf berubah menjadi alur saraf antara hari ke 18 dan 20, begitu lipatan saraf bertemu di garis
tengah, mereka mengalami penutupan pada siang hari. Dua situs penutupan lipatan saraf dan dua
neuropori didefinisikan. Bagian terbuka dari tabung saraf diberi nama neuropori anterior dan posterior,
setelah penutupan dimulai. Ritsleting berlangsung secara kaudal sampai neuropori posterior ditutup.
Tapi, di daerah tengkorak situs penutupan kedua muncul di otak depan

After that, zippering continues in two directions. The anterior neuropore is closed on day 25, the
posterior neuropore completes closure on day twenty-eight.At the same time, giving rise to the neural
crests, the cells at the border zone of neuroectoderm and ectoderm separate. Neural crests then
separate and form the primordial of the ganglia giving rise to the sensory innervations while the adjacent
level of the neural tube (eventually forms the spinal cord), which then produce the motor innervations.
The somite plate forms on either sides of the neural tube, which by the end of fifth week forms 42 pairs
of somites. Subsequently, somites form a central cavity with the inner edge forming the sclerotome,
which migrates to the notochord and forming the vertebral primordial that includes chondroblasts,
osteoblasts, and fibroblasts. Dermomyotomes also arise from the somite that divides into dermatomes
and myotomes, forming the cutaneous derivates and muscles, respectively. Myotomes eventually form
the vertebral muscles. The cranial and caudal halves of each sclerotome fuse to form vertebral bodies
and the intervertebral disk. Notochord regresses to the level of the intervertebral disks and forms the
nucleus pulposus. Primary neurulation completes by the end of fifth week by which the brain and 90% of
the spinal cord have formed. Secondary neurulation occurs during fifth to sixth week, completes by the
end of seventh week giving rise to the remaining 10% of the spinal cord and filum terminale.

myelocele
Herniation of spinal cord tissue and meninges through a defect in the lumbar region of the vertebral
column. The protrusion of the tissue is flush with the level of the skin surface

spinal malformations

spinal malformations, including disorders of

(1) primary or

(2) secondary neurulation and

(3) anomalies of notochordal development.

Primary neurulation disorders can be classified as

(1) open (nonskin covered) spinal dysraphism,

(2) dorsal dermal sinus, and

(3) closed (skin covered) spinal dysraphism.

Most of these malformations are characterized by cutaneous findings including an overlying hairy tuft,
cutaneous hemangiomas, and/or varying degree of abnormal skin pigmentation.

Open spinal dysraphism results in CSF leakage versus closed spinal dysraphism in which ectodermal and
mesodermal tissue (e.g., skin, subcutaneous fat) covering the malformed neural placode prevents CSF
leakage.

Open (Non-skin Nonskin Covered) Spinal Dysraphism Open spinal dysraphism has the most significant
impact on the quality of life for the affected child. They are classified as

1. myelocele (MC) and

2. myelomeningocele (MMC).

MMCs are more frequently seen than MCs. Both MMC and MC result from a disruption of primary
neurulation. A defective or incomplete closure of the neural tube results in a neural placode that is not
separated from the adjacent surface ectoderm. As a result, a flattened midline neural placode is either at
the same level with the adjacent cutaneous surface (MC) or is pushed dorsally through the defect
outside of the level of the open osseous spinal canal (MMC) by the widened anterior subarachnoid space
(Fig 3A– D).
Adjacent bone, muscle, and skin are deficient in various degrees of severity. Open posterior elements
can be best documented on axial views (Fig 3C,D). These malformations most frequently occur at the
lumbar level; however, the thoracic or cervical spinal cord may also be involved. Direct surgical repair is
necessary to prevent further damage or secondary inflammation of the malformed spinal cord, because
the neural placode is directly exposed to the air. In addition, all children with an open spinal dysraphism
have an associated Chiari II malformation (Fig 4A,B).

It is believed that the chronic leakage of CSF during the intrauterine development of the fetal brain
results in an incomplete or deficient expansion of the rhombencephalic vesicle, preventing normal
growth of the skull base and posterior fossa.26 The postnatally observed anatomic features of Chiari II
malformation can at least partially be explained by a too small posterior fossa (Fig 4A) with resultant
upward and downward herniation of cerebellar and brainstem structures (Fig 4B).

Based on this hypothesis, in-utero closure of open spinal dysraphism lesions was shown to diminish
dysfunction in children with Chiari II malformation, including a 50% decrease in shunting for postnatal
hydrocephalus and a significant improvement in neurological function.

Because these lesions are usually recognized on prenatal US or MRI or on physical examination at birth
and due to the increased risk of injury or infection, direct US examination of the cele should be avoided.
However, with the use of a sterile approach MMC can be distinguished from MC as in the second one
there is no widening of the ventrally located CSF space.

Neonatal spinal US should not be limited to the examination of the most obvious area of dysraphism but
should also include examination of the remaining spine looking for associated anomalies, including
hydromyelia, a lipoma, an arachnoid cyst, and coexisting DMM.

US can be used for scanning of the cervical spinal cord and craniocervical junction through a suboccipital
approach to show the posterior fossa changes associated with Chiari II malformation.

Finally, spinal US is useful to detect the postoperative changes and US scanning of the repaired MMC
might be requested looking for secondary postoperative complications (Fig 5A–C) including
rethethering.12
Usha D. Nagaraj, Karin S. Bierbrauer, Charles B. Stevenson, Jose L. Peiro, Foong-Yen Lim, Bin
Zhang, And Beth M. Kline-Fath.American Journal Of Roentgenology 2018 211:6, 1376-1380
neural tube defect, any congenital defect of the brain and spinal cord as a result of
abnormal development of the neural tube (the precursor of the spinal cord) during early
embryonic life, usually accompanied by defects of the vertebral column or skull. In
normal development a plaque of nerve tissue forms along the surface of what will
become the back of the fetus; this tissue folds into a closed tube that develops into the
structures of the central nervous system. Malformations occur because the tube fails to
close properly, because parts of it are missing, or because part of the tube is blocked.

Failure of the tube to close in some degree is the basic defect underlying spina bifida,
meningocele, myelocele, and meningomyelocele. Spina bifida is caused by the failure of
the vertebrae to form over the back of the spinal cord, leaving the nerve unprotected. It
usually occurs in the sacral or lumbar regions at the base of the spine, the final section of
the neural tube to close. All the other neural tube defects in this group are particular
forms of spina bifida.

In more serious forms of spina bifida, part of the spinal cord is left uncovered
by the skin or actually protrudes from the spinal column. In myelocele, the
spinal cord is exposed so that nerve tissue lies exposed on the surface of the
back without even a covering of skin or of the meninges, the membranous
tissue surrounding the brain and spinal cord. Meningocele occurs when these
meninges protrude through the vertebral defect, forming a fluid-filled
sac. Meningomyelocele is a compound defect in which the protruding sac
contains some nervous tissue as well. If any of these defects communicate with
the central canal of the spinal cord, the prefix syringo- is added to the name;
hence, a syringomyelocele is an open defect containing nerve tissue and
opening into the spinal cord.
Children born with the more serious forms of spina bifida experience paralysis in those
parts of the body below the site involved. The legs may be completely paralyzed, and
bladder and bowel functions may be absent or impaired. Spina bifida defects can be
treated surgically early in life; success is greatest with meningocele,
because paraplegia or other disabilities associated with myelocele can complicate
therapy.

Another form of open neural tube defect, encephalocele, occurs when a meningeal sac
containing brain tissue protrudes from the skull. The outlook for affected individuals
depends on the amount of nervous tissue involved.

Britannica, T. Editors of Encyclopaedia (2018, January 29). neural tube

defect. Encyclopedia Britannica. https://www.britannica.com/science/neural-tube-
defect.

ARNOLD CHIARI MALFORMATION

Arnold-Chiari or Chiari malformations describe a group of deformities of the posterior fossa and
hindbrain, which includes the cerebellum, pons, and medulla oblongata. These deformities lead to
problems ranging from cerebellar tonsillar herniation through the foramen magnum to the absence of
the cerebellum, with or without other associated intracranial or extracranial defects such as
hydrocephalus, encephalocele, syrinx, or spinal dysraphism. This activity examines when this condition
should be considered within a differential diagnosis and how to evaluate the patient for it properly. This
activity highlights the role of the interprofessional team in caring for patients with this condition.

Arnold-Chiari, also known as Chiari malformation, is the name given to a group of deformities
of the hindbrain (cerebellum, pons, and medulla oblongata).
Issues range from herniation of the posterior fossa contents outside of the cranial cavity to the
absence of the cerebellum with or without other associated intracranial or extracranial defects
such as hydrocephalus, syrinx, encephalocele, or spinal dysraphism.
For Chiari I malformation, the prognosis is good, but it also depends on the presence of
preexisting neurological deficits. Most patients who have no neurological deficits have an
excellent outcome.
A controversial subject in clinical management entails the diagnosis in the setting of
possible sport-specific participation. Historically, symptomatic and postsurgical Chiari I patients
were advised not to return to contact sports, but recent studies have shown that the risk of
catastrophic injury in this population is low.[40][41] Regardless, a decision should be made on a
case-by-case basis.
Individuals who have chronic weakness or gait problems usually do not improve, and their
prognosis is guarded.

Classification
Chiari malformations are classified based on their morphology and severity of anatomic defects,
typically through imaging (or autopsy).
Chiari I is the least severe and often found incidentally. It is characterized by one or both pointed
(not rounded) cerebellar tonsils that project 5 mm below the foramen magnum, measured by a
line drawn from the basion to the opisthion (McRae Line).
Chiari II consists of brainstem herniation and a towering cerebellum in addition to the herniated
cerebellar tonsils and vermis due to an open distal spinal dysraphism/myelomeningocele.
Chiari III involves herniation of the hindbrain (cerebellum with or without the brainstem) into a
low occipital or high cervical meningoencephalocele.
Chiari IV is now considered obsolete.[4] Prior to becoming an obsolete diagnosis, it was already
a more controversial and rare variant that demonstrated severe cerebellar hypoplasia, similar to
primary cerebellar agenesis. Previously some stated that myelomeningocele could be present,
[5] while others argued that the presence of myelomeningocele should then be classified as a
Chiari II with a vanishing cerebellum.[6]
There are other controversial reported classifications, including Chiari 0, Chiari 1.5, and Chiari
V. Chiari 0 is characterized by syringomyelia without hindbrain herniation while Chiari 1.5 is
felt to be the progression of Chiari I with increased cerebellar tonsillar descent and some
involvement of the brainstem.[7][8] Chiari V, the most severe variant, represents cerebellar
agenesis with occipital lobe descent and herniation through the foramen magnum
Etiology

There are multiple proposed theories, including molecular, hydrodynamic, and mechanical, with
the likelihood that different mechanisms can have the same resulting Chiari malformation.[10]
Reduced volume of the posterior fossa leads to displacement of the cerebellar tonsils through the
foramen magnum in Chiari I malformations. Causes include primary congenital hypoplasia or
secondarily from acquired morphologic changes, such as premature closure of sutures, calvarial
dysplasia, or genetic/syndromic. Mutations on chromosomes 1 and 22 have been identified as
possible causes for hereditary posterior fossa hypoplasia.[11]
McClone and Knepper proposed an open neural tube defect (myelomeningocele) is the
underlying cause of Chiari II malformations.[12] This leads to leakage or redirected flow of
cerebrospinal fluid resulting in a fourth ventricle that is unable to maintain distension. This
continued collapse of the fourth ventricle in utero results in a hypoplastic posterior fossa and
cerebellar tonsillar herniation. This is also the suspected cause of Chiari III as well, though in the
setting of an encephalocele or high cervical myelomeningocele, as opposed to the lumbar or
sacral myelomeningocele in Chiari II. Folate deficiency and methylenetetrahydrofolate reductase
mutations increase the risk of neural tube defects and can thus be an underlying cause of Chiari
II and III.
The etiology of the remaining Chiari variants is still debated and not clearly known. Trauma can
be an etiology of cerebellar tonsillar herniation, though, in the setting of a normal-sized posterior
fossa, a designation of Chiari is not appropriate.
Epidemiology

Chiari I malformation is the most common type and occurs in approximately 0.5 to 3.5% of the
general population with a slight female predominance (1.3:1).[13][14]
Chiari II occurs in 0.44/1000 births without gender predominance but can have a decreased
incidence with folate replacement therapy by the mother in utero.
The remaining Chiari malformations are much rarer. Chiari III is the most common of these
other variants, consisting of 1-4.5% of all Chiari malformations.
Pathophysiology

Neurologic signs and symptoms can arise from 2 mechanisms:

 Direct compression of neurological structures against the surrounding foramen magnum
and spinal canal
 Syringomyelia or syringobulbia development
o The obstruction of cerebrospinal fluid (CSF) outflow eventually results in
syrinx development. 
 o Fluid-filled cavities (syrinx) develop within the spinal cord or brainstem, resulting
in neurologic symptoms as the cavity expands.[15]
In patients with the Chiari I malformation, the bones of the skull base often are underdeveloped,
which results in a reduced volume of the posterior fossa, the volume of which is inadequate to
contain the entire cerebellum; thus, cerebellar tonsils are displaced through the foramen
magnum.
The posterior fossa in Chiari type II malformation is even smaller than in Chiari I malformation.
The cerebrospinal fluid (CSF) cisterns are poorly developed due to lack of fourth ventricular
expansion as a consequence of in-utero derivation of CSF circulation to the neural tube defect,
all of which results in hindbrain structures downward herniating with subsequent compression of
these structures against the foramen magnum.[15]
In both type I and II, there is CSF-flow obstruction by the foramen magnum crowding, and
consequently, hydrocephalus and/or syringomyelia formation are possible over time.
History and physical
In Chiari I malformation, the most common presentation is suboccipital headaches and/or neck
pain (80%). Symptoms are exacerbated when asked to perform the Valsalva maneuver. Other
common presentations include ocular disturbances, otoneurologic symptoms (dizziness, hearing
loss, vertigo), gait ataxia, and generalized fatigue. Although much less common, the literature
reports multiple case studies in which patients have presented with isolated extremity pain or
weakness, one such report including a presentation of unilateral shoulder pain with isolated
muscle weakness presenting to a sports medicine clinic.[16]
Myelopathy classically presents with “dissociated sensory loss” (loss of pain and temperature
sensation, preserved fine touch and proprioception) and motor weakness.[17][18]
Cerebellar signs, including ataxia, dysmetria, and nystagmus, and lower cranial nerve deficits
(IX, X, XI, XII CN) result either from direct compression of the cerebellum or medulla at the
foramen magnum or from syringomyelia or syringobulbia.
Sleep apnea can occur in a patient with Chiari malformation due to a weakness of pharyngeal
muscles elicited by the brainstem, upper spinal cord, or lower cranial nerve compression.
It is not an uncommon scenario to find patients with radiological findings compatible with Chiari
malformation with no clinical manifestations of the disease (incidental Chiari malformation).
Therefore, nonspecific symptoms such as generalized fatigue or classic pattern migraines are not
necessarily related to the Chiari malformation.
The remaining variants (with the exception of Chiari 0 and 1.5) are diagnosed often in utero or at
birth.
Evaluation
Imaging evaluation of Chiari malformations varies, with Chiari I evaluated using magnetic
resonance imaging (MRI) typically in a child or adult, whereas Chiari II to IV are often first
evaluated by ultrasound in utero, with fetal MRI assessment performed for further
characterization.
MRI of the head and cervical spine is the test of choice in evaluating Chiari I. This will
demonstrate cerebellar tonsillar descent greater than 5 mm below the foramen magnum (McRae
line). In addition, a decreased size of the posterior fossa and a syrinx may be seen.
[19] Depending on the extent of the syrinx, the addition of a thoracic and/or lumbar spine MRI
may be needed. In the setting of ventricular dilation, CSF flow (or cine) sequences may be
performed to assess for CSF flow dynamics and evaluate for obstruction at the foramen magnum.
Other useful tests in the management of patients with Chiari I malformation include:
 Myelography: Of special value as an alternative in patients in who an MRI cannot be
obtained.
 CT or x-rays of the neck and head: May reveal common associated bony defects,
particularly of the craniocervical junction relevant for surgical planning, such as basilar
invagination.
Fetal sonography, often during the second-trimester anatomy scan, demonstrates the typical
imaging features of Chiari II and III malformations. One classic imaging finding on ultrasound is
the lemon sign of the anterior frontal calvarium, with loss of the normal convex curvature and
flattening or inward bowing/scalloping that results in a shape similar to a lemon.[20] The banana
sign is another classic sign of Chiari II and distal neural tube defect, this time regarding the
cerebellum, which demonstrates an abnormal morphology with anterior curvature of the
cerebellum and obliteration of the cisterna magna.[21] Chiari III will demonstrate the occipital or
high cervical meningoencephalocele when evaluating the posterior fossa during a prenatal exam.
Fetal MRI can demonstrate the cerebellar hypoplasia/aplasia of Chiari IV and V and further
evaluate the neural tube defects and hindbrain herniation of Chiari II and III. MRI also better
demonstrates the tectal beaking that occurs in Chiari II.
Laboratory studies are not of help in the evaluation of patients with Chiari malformation.
However, laboratory studies are needed during planning for surgery. Routine studies like the
complete blood count (CBC), coagulation profile, electrolyte levels, chest X-ray, and
electrocardiogram (ECG) will suffice.
Treatment / Management
Medical Management
Patients with Chiari malformation and who have no symptoms can be managed medically.
Headaches and neck pain can be treated with muscle relaxants, NSAIDs, and temporary use of a
cervical collar. However, studies show that while a headache and nausea may improve, there will
be no improvement in gait with medical management in many symptomatic patients. Close to
90% of patients with Chiari type I may remain asymptomatic even if they have syringomyelia.
Surgical Management
The main treatment for Chiari malformation is surgical with the goal of re-establishing the CSF
flow across the craniovertebral junction and relieving pressure on the cerebellum and hindbrain
by decompressing the posterior fossa.[22][10]
Surgery is recommended for persistently symptomatic patients and confirmed tonsillar
herniation. In the setting of asymptomatic tonsillar herniation, with or without syrinx,
observation is recommended unless symptoms develop.
Better surgical results are seen when surgery is performed within 2 years of symptoms onset.
Surgical Techniques
The standard surgical technique for Chiari I is a posterior fossa decompression.[23][10][24] This
is obtained by a suboccipital craniectomy enlarging the foramen magnum, often in conjunction
with C1, and possible C2, laminectomy. The dura may or may not be opened, with subsequent
dissection of arachnoid adhesions if present. Depending on the available dural expansion and
size of the posterior fossa, a duraplasty may need to be performed. The dural graft can be an
autograft such as occipital fascia or tensor fascia lata (TFL) tendon, or artificial dura.[25] In the
setting of a syrinx, a shunt can also be placed if decompression alone is not effective. Tonsillar
cauterization may also be performed.
More recently, minimally invasive techniques have been described similar to those used in the
spine. These allow for smaller incisions, less soft tissue damage, less dural manipulation, shorter
hospital stays, faster recovery, and fewer complications.[26][27][28][29][30][31]
Initial surgical correction for Chiari II is the correction of the myelomeningocele, generally in
the first 48 hours. This can also be done in utero through a hysterotomy.[32] Closure of the
spinal dysraphism can be done in a variety of ways, with either primary skin closure,
myocutaneous flap, or fasciocutaneous flap, depending on the severity, involved layers, and the
available adjacent tissue. The vast majority will eventually need a ventricular shunt for CSF
diversion in the setting of hydrocephalus.[33] If needed, a posterior decompression is performed
later to allow suboccipital expansion.
Chiari III follows a similar course to Chiari II. The occipital/high cervical encephalocele is
corrected first, with resection of herniated contents, as these are typically non-viable, followed
by dural closure and a cranioplasty.[34] If the amount of herniated tissue is greater than
intracranial contents, the patient is deemed a nonsurgical candidate. A ventricular shunt is placed
if the patient has concomitant hydrocephalus.
Contraindications to Surgery
Suboccipital decompression is contraindicated when the tonsillar herniation is due to a pathology
other than Chiari malformation. Some examples of this are intracranial hypotension or mass
effect in the posterior fossa due to a mass. 
Differential Diagnosis
 Intracranial hypotension – sagging midbrain may mimic tonsillar or hindbrain herniation.
 Normal variant cerebellar tonsillar ectopia – does not meet the criteria for Chiari
malformation and an incidental finding in an asymptomatic patient.
 Tonsillar herniation from increased intracranial pressure (ICP) – assess for causes of ICP
such as mass effect from neoplasm, hydrocephalus, trauma, or hemorrhage.
Prognosis
Chiari I has a good prognosis, but it also depends on the presence of preexisting neurological
deficits. Most patients who have no neurological deficits have an excellent outcome.[35]
[14] Chiari II has a 3% neonatal in-hospital mortality and 15% 3-year mortality rate. Those that
survive can have increasing motor dysfunction over time. Continued follow-up for shunt
placement evaluation or shunt failure is recommended. Prognosis in the more severe Chiari
variants is poor and often dismal, with early death.
Individuals who have chronic weakness or gait problems usually do not improve, and their
prognosis is guarded.[36]
Complications
 Pseudomeningocele
 CSF leak
 Meningitis
 Wound infection
 Lower brainstem malfunction
 Epidural hematoma
 Apnea
 Vertebral artery injury
Postoperative and Rehabilitation Care
In the postoperative period, monitoring for CSF leak is vital. Some patients may develop a
pseudomeningocele, which may require drainage.
Patients can also be evaluated postoperatively with the Chicago Chiari Outcome Scale for a more
subjective evaluation of postoperative improvement.[37][38][39] This scoring system factors
pain, non-pain symptoms, functionality, and postoperative complications into a 1-4 point scale
for each component. This results in a total score ranging from 4-16, with 4 representing an
incapacitated outcome, while 16 represents an excellent outcome.
Exercise and heavy lifting should not be done for at least 3 to 4 weeks after the procedure.
Most patients require 6 to 8 weeks to recover from surgery and reverse any major neurological
deficit fully. Patients should consider refraining from contact sports following surgery, even after
the surgical site is well healed.
Repeat MRI is necessary to ensure that the syrinx has responded to the treatment.
Hidalgo JA, Tork CA, Varacallo M. Arnold Chiari Malformation. [Updated 2022 Sep 5]. In: StatPearls
[Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from:
https://www.ncbi.nlm.nih.gov/books/NBK431076/

Myelocele
 Etiology: failure of closure of posterior neural tube, schisis =
split
  Imaging: opening directly onto spinal canal, flattened spinal
cord with no covering
 Clinical: overt not skin covered spinal dysraphism, most
severe dysraphism
https://pediatricimaging.org/diseases/myelocele/