Content uploaded by Anas Abdallah
Author content
All content in this area was uploaded by Anas Abdallah on Dec 07, 2018
Content may be subject to copyright.
92 Sinir Sistemi Cerrahisi / Cilt 5 / Sayı 3-4, 2015
Congenital Malformation
Meningomyelocele at L5-S1 Level and
Defective Thecal Sac Which is Terminated
Subcutaneously at L3-4 Level: Presentation
of a Rare Case and Surgical Technique
Anas ABDALLAH*, Betül Güler ABDALLAH***,
Erhan EMEL****
*Fulya Orthopaedic and Spine Institute, Istanbul
**Department of Neurosurgery, Kanuni Sultan Süleyman research and Training Hospital, Istanbul
***Department of Neurology, Bakirkoy Research and Training Hospital for Neurology Neurosurgery and
Psychiatry, Istanbul
****Department of Neurosurgery, Bakirkoy Research and Training Hospital for Neurology Neurosurgery and
Psychiatry, Istanbul
Olgu Sunumu
Sinir Sistemi Cerrahisi Derg 5(3-4):92-96, 2015
doi:10.5222/sscd.2015.092
Nutritional, environmental factors and genetic disorders are the main factors that play role in the develop-
ment of neural tube defects (NTDs). In the spinal surgery, the most common congenital lesions presenting
to medical attention are the diverse forms of the spinal dysraphism and caudal spinal anomalies. This re-
port describes a rare case of congenital defective thecal sac that terminated in the skin at L3-4 level of the
child who underwent surgery to repair meningomyelocele in our institute when he was 11- day old. After
the urodynamic study was performed the surgery to repair swelling at L3-4 level had done. The child
followed-up for four years from his birth. The child is doing well, without growth or mental retardation.
Herein, the authors describe the management of multiple congenital malformations in children.
-
rophysiological monitoring, urodynamic study
J Nervous Sys Surgery 2015; 5(3-4):92-96
Beslenme, çevre faktörleri ve genetic hastalıkları, nöral tüp defektleri gelişmesinde önemli oynayan fak-
törlerdir. Spinal cerrahisinde, disrazm ve kaudal spinal anomaliler, konjenital lezyonlar arasında en
sık bebeklere cerrahi bırakan lezyonlardır. Bu makalede, ender olarak 11 günlükken meninjomyelosel
nedeniyle merkezimizde cerrahi olarak tedavi edilen bebekte saptanıp L3-4 seviyesinde ciltaltında biten
konjenital defektif dura sunulmaktadır. Ürodinamik test yapıldıktan sonra L3-4 seviye arasında bulunan
kese müdahele edilip tamir edildi. Çocuk, postoperative olarak 4 yıl takip edildi. Durumu gayet iyidir,
zihinsel ve/veya yapısal gelişim geriliği saptanmamıştır. Bu raporun önemi yazarların keseli olan ve nö-
rolojik olarak sağlıklı çocuklarda decit olmadan nasıl müdahale edilebileceğini anlatmalarıdır.
J Nervous Sys Surgery 2015; 5(3-4):92-96
20.11.2016
Kabul tarihi: 14.09.2017
Uzm. Dr. Anas Abdallah, Fulya Ortopedi ve Omurga Merkezi, Dikilitaş Mah. Ayazmadere Cad. Yeşilçimen Sok. No: 9 K: 3
Beşiktaş / İstanbul
e-mail: abdallahanas@hotmail.com
93
Congenital Malformation Meningomylelocele at L5-S1 Level and Defective Thecal Sac Which is Terminated Subcutaneously at
L3-4 Level: Presentation of a Rare Case and Surgical Technique
Sinir Sistemi Cerrahisi / Cilt 5 / Sayı 3-4, 2015
The incidence of neural tube defects (NTDs)
is 0.17–12 per 1000 live births worldwide
(1-3). Meningomyelocele is one of the com-
mon spinal dysraphic lesions whose incidence
had been reduced in developed countries due
to supplementation of folic acid during preg-
nancy and prenatal diagnosis of dysraphic
malformations almost leading to termination
of the pregnancy. Meningomyelocele is gen-
erally associated with several malformations
such as tethered cord, split cord malformation,
diastematomyelia and vertebral fusion anoma-
lies such as spina bida occulta, block verte-
bra or buttery-shaped vertebrae.
Several factors may play role in developing
NTDs such as nutritional, environmental, ge-
netic syndromes and mutations in multiple
genes (2). However, folic acid deciency in
pregnancy is still the proven etiology. NTDs
can be divided into two main subgroups ac-
cording to the involved sites; cranial dysra-
phism and spinal dysraphism. On the other
hand, some authors classied NTDs as open
or closed NTD.
The consensus among most neurosurgeons is
that the child who was born with meningomy-
elocele should be closed promptly. Several cen-
tres in USA and Europe started to treat infants
during their intrauterine life (1,4). Herein, the
authors describe the management of children
involved with multiple spinal malformations
after presenting surgical intervention to treat
a rare case of meningomyelocele, spina bida
occulta and spinal cutaneous swelling.
A 20-month-old male child was born with the
meningomyelocele. At the 11th day of his life,
he had been treated by repairing the thecal sac
and closing the skin at the L5-S1 level. He had a
swelling sac which was covered with hair patch
at L3-4 level. To avoid the complication may be
occurred, the swelling at L3-4 had not been in-
cluded in the surgical procedure. It was thought
that swelling contains bers of the medullary
conus, therefore, neurosurgical team decided to
perform urodynamic test to explore the contents
of this cystic swelling then plan a second surgery
when the child would be 18 months of age. Af-
ter the rst surgery, the child called to monthly
control visits.
On his 18-month control child was neurologi-
cally intact. Excluding the hypertrichosis (tuft
of hair) and swelling on his back, his physical
examination was unremarkable. The neurosurgi-
cal team decided to perform urodynamic study
(because of the controversies about use of MRI
in children the authors did not perform MRI).
The urodynamic study demonstrated residue in
the bladder after urination (70 cc). As is well
known radiography ca not conrm the presence
of a neural structure (if any) in the swelling at
L3-4 .
Under general anesthesia and intraoperative
neurophysiological monitoring the paramedian
vertical midline (sh- mouth shaped) incision
between L1 to L5 was performed. To avoid cut-
ting or incising any neural structure contained
in this swelling the spinous process of L2 was
identied, bilateral paraspinal muscles were dis-
sected and spinous process of L2 was split using
a ne-tip blade. The spina bida occulta of L3-
L4, and diastematomyelia at L3 level were seen,
while the thecal sac was terminated in the skin at
L3-4 level (Figure 1). Duraplasty was performed
(Figure 2). L2, 3 and 4 laminoplastİes were per-
formed using non-absorbable 2/0 sutures. The
fascia and skin were sutured appropriately to
their anatomic layers. Postoperative course was
94
A. Abdallah, A. Gökçadağ, B. G. Abdallah, E. Emel
Sinir Sistemi Cerrahisi / Cilt 5 / Sayı 3-4, 2015
uneventful, no complication was detected, and
the child was discharged after 3 days.
The child were followed-up for four years from
his birth. He is developing well, without dem-
onstrating growth or mental retardation. On his
postoperative 30th month visit, the urodynamic
study showed a slight decrease in residual vol-
ume ( 60 cc which is at age-matched upper limit
of normal physiologic volume ). Yearly control
visits were recommended.
Meningomyelocele is the most common con-
genital anomaly of the central nervous system.
It accounts for 98% of open spinal dysraphisms
(5). It is caused by defective neural tube. It is as-
sociated with other spinal cord anomalies. A hair
tuft on our patient can be distinctive nding on
physical examination. The malformations in our
child were meningomyelocele, diastematomy-
elia, spina bida occult, and spinal cutaneous
swelling (cyst) between L3 and L4, however, his
systematic and neurologic examinations were
unremarkable.
Spina bida is an incomplete fusion of halves
of the ventral arches resulting in midline defect
usually in lumbosacral region. Its manifestations
vary, but generally the small bones (vertebrae)
that make up the spine do not complete their
developmental process, and gaps may be seen
between them. The term spina bida refers to
the defective fusion of posterior spinal bony ele-
ments in open dysraphism. Myelomeningoceles
and myeloceles are characterized by exposure
of the placode through a midline defect in the
back. In myelomeningoceles, expansion of the
underlying subarachnoid space results in eleva-
tion of the placode above the cutaneous surface
(open spina bida with dorsal cyst), whereas in
myeloceles, the placode is ush with the cutane-
ous surface (6).
Open spinal dysraphism originates from defec-
tive closure of the primary neural tube, which
leads to the persistence of a segment of non-
neurulated placode. Generally majority of cases
are located at the lumbosacral level, with the
placode being the conus (5,6). Since neurulation
does not occur, the cutaneous ectoderm does not
detach from the neural ectoderm and remains in
a lateral position. This results in a midline skin
defect. Therefore, the external surface of the pla-
code is directly visible on inspection (6).
In utero surgical repair has been applied in sev-
Figure 1. Photo had been taken through operation showing the
subcutaneous tissue in the caudal part of the dura. There was no
Figure 2. Duraplasty was performed after explore the spinal
cord.
95
Congenital Malformation Meningomylelocele at L5-S1 Level and Defective Thecal Sac Which is Terminated Subcutaneously at
L3-4 Level: Presentation of a Rare Case and Surgical Technique
Sinir Sistemi Cerrahisi / Cilt 5 / Sayı 3-4, 2015
eral centers in the USA and Europe for many
years (1,4) for the management of myelomenin-
gocele. In utero surgical repair was evaluated in
a controlled trial and short-term benets for the
newborn, including 50% reduction in the need
for hydrocephalus shunting and signicant im-
provement in spinal neurological function was
demonstratred (1).
Our patient had been treated surgically by repair
of his thecal sac at L5-S1 level. When we reop-
erated child we had seen that the laminae were
intact without prior surgical interventişon. The
neurosurgical team suggests that laminoplasty
approach should be used in such pediatric pa-
tients. In pediatric patients we prefer to incise
the cartilage of spinous processes by surgical
blade, then use small retractors to expose the
surgical eld. After performing all surgical in-
tervention we ought to close right and left sides
of laminae using a strong non-absorbable sutures
to perform laminoplasty. Laminoplasty and neu-
rophysiological monitoring is essential in child
who will undergo spinal surgery. Further inves-
tigations such as urodynamic studies have to be
performed in children with meningomyelocele
especially those with hair tufts.
In the management of children affected by my-
elomeningocele associated with other spinal
malformations, rst meningomyelocele should
be closed promptly to protect child from men-
ingitis that may be associated with open NTDs.
The authors here suggest to wait 18 months to
perform urodynamic test. The urologists com-
ments of urodynamic test did not guide the
neurosurgical team to decide if there wasa neu-
ral structure involved or not. Therefore, in sec-
ond surgery surgeons did not incise the swell-
ing directly but passed close the swelling using
midline sh- mouth shaped incision to avoid
injuring the neural bers may pass through the
cyst (swelling). The surgeons started dissection
above L2 lamina (the intact lamina of swelling
superior edge). We noticed that the thecal sac
was terminated in subcutaneous layer (Figure 2).
Dissection of the dural sac had been completed.
Then the spinal cord and diastematomyelia were
explored to ensure inexistence of split cord mal-
formation . After duraplasty achieved using 5/0
absorbable (vicryl) sutures, intact L2 and defec-
tive L3 and 4 were repaired with laminoplasty
using non-absorbable sutures.
LLaminoplasty and neurophysiological monitor-
ing is essential in child who will undergo spinal
surgery. The management for spinal cutaneous
swelling (cyst) that has no risk of infection or
meningitis in newborns rst, urodynamic test
should be performed at age of 18 months. If this
study did not give details about the structures in
the cyst surgeon has to avoid cutting or incising
the swelling directly so as to save neural struc-
tures that may be contained in the cyst.
Competing Interest
The authors declare that they have no competing
interests. All authors certify that they have NO
afliations with or involvement in any organiza-
tion or entity with any nancial interest or non-
nancial interest in the subject matter or materi-
als discussed in this manuscript.
Patient Consent
Written informed consent was obtained from the
patient’s parents for publication of this case re-
port and accompanying images. A copy of the
written consent is available for review by the
Editor-in-Chief of this journal.
There are no nancial disclosures or conicts of
interest.
96
A. Abdallah, A. Gökçadağ, B. G. Abdallah, E. Emel
Sinir Sistemi Cerrahisi / Cilt 5 / Sayı 3-4, 2015
-
A randomized trial of
prenatal versus postnatal repair of myelomeningocele.
N Engl J Med 2011;364(11):993-1004.
https://doi.org/10.1056/NEJMoa1014379
2. Alfarra HY, Alfarra SR, Sadiq MF. Neural tube de-
fects between folate metabolism and genetics. Indian J
Hum Genet 2011;17(3):126-31.
https://doi.org/10.4103/0971-6866.92082
Spina bida outcome: a 25-year prospective. Pe-
diatr Neurosurg 2009;34:114-20.
https://doi.org/10.1159/000056005
Neural tube defects:
recent advances, unsolved questions, and controversies.
Lancet Neurol 2013;12(8):799-810.
https://doi.org/10.1016/S1474-4422(13)70110-8
Congenital anomalies
of the central nervous system. In: Kliegman RM, Beh-
rman RE, Jenson HB, Stanton BF, (eds). Nelson Text-
book of Pediatrics. 18th ed. Philadelphia, Pa: Saunders
Elsevier; 2007: [chap 592]
6. Paladini D. Diagnosis of spina bida and other dys-
raphic states in the fetus. In Özek MM, Cinalli G, Maix-
ner WJ (eds). The Spina Bida: Management and Out-
come. Springer-Verlag Italia, 2008; 75-94.
https://doi.org/10.1007/978-88-470-0651-5_6
