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American-Eurasian Journal of Scientific Research 2 (1): 60-62, 2007
ISSN 1818-6785
© IDOSI Publications, 2007
Corresponding Author: Dr. T.O. Odebode, P.O. Box 5173, Central Post Office, Ilorin 240001, Kwara State, Nigeria
60
Cervical Myelomeningocele and Hydrocephalus
Without Neurological Deficit: A Case Report
T. Olugbenga Odebode, S. Ukpong Udoffa and A. Donald Nzeh
1 12
Division of Neurological Surgery, Department of Surgery, Department of Radiology,
12
University of Ilorin Teaching Hospital, Ilorin, Nigeria
Abstract: Cervical myelomeningocoele is a rare dysraphic lesion which rarely presents with neurological deficit
in affected infants. We report a 9-day-old female neonate with a posterior midline neck swelling and a large head
noted at birth; that had a mid cervical myelomeningocoele and hydrocephalus without neurological deficit. All
the brain ventricles were dilated on ultrasound and the fluid filled neck swelling showed no evidence of Chiari
malformation or cord tethering. These associations were also absent during spinal exploration, repair of
myelomeningocoele and ventriculo-peritoneal shunting. Except for a shunt revision necessitated by shunt
blockade occurring 12 days after surgery, there has been no neurological deterioration since eighteen months
of initial surgery. We conclude that an infant with cervical myelomeningocoele and hydrocephalus does not
always have a Chiari malformation and could present without neurological deficit. A prolonged follow-up is
however paramount in the event that the child may subsequently develop neurological deterioration.
Key words: Cervical myelomeningocoele hydrocephalus
INTRODUCTION
Myelomningocoele is the commonest congenital
malformation of the central nervous system (CNS)
worldwide [1] and in Nigeria, it is the commonest anomaly
in the north [2] and middle belt [3] but second to
hydrocephalus in the south [4] the lumbo-sacral lesion
being the most common [2-4]. From previous reports;
the cervical type is very rare constituting only 3.0% to
8.0% of spinal bifida cystica [5, 6]. The degree of
neurological deficit in infants with myelomeningocoele
increases the further away the lesion is from the conus.
However, cases of isolated cervical myelomeningocoele
are usually associated with limited or no neurological Fig. 1: A 9-day-old neonate with an oval midline low
deficit, though subsequent neurological deterioration cervical cystic swelling measuring 5 cm x 4 cm x 3
could be fairly common. In this communication, we cm and a large head. The sessile base is covered
report a neonate who presented with cervical with full thickness skin
myelomeningocoele associated with hydrocephalus
but without neurological deficit. child of a family devoid of history of CNS malformation.
CASE REPORT swelling measuring 5 cm x 4 cm x 3 cm in size. Its wide
A 9-day-old female; product of an uneventful term comprised of a tough purplish membrane without an
pregnancy, labour and delivery, had a posterior neck evidence of cerebrospinal fluid leakage (Fig. 1). The
swelling which was first noted at birth. She is the third active and pink neonate had normal growth parameters
There was a round, fluctuant, translucent cystic neck
base was covered with full thickness skin while its dome
Am-Euras. J. Sci. Res., 2 (1): 60-62, 2007
61
Fig. 2: Plain cervical spine radiograph showing a spina simple layered closure was achieved with ease. Twelve
bifida defect and widening of interpedicular days after shunting she developed a catheter blockage
distances of C vertebral bodies necessitating a revision of the shunting procedure.
4-7
including a head circumference of 35 cm. The anterior enjoyed a hitch free clinical status for more than eighteen
fontanel was however full and 4 cm wide in its months.
coronal plane. She had spontaneous movements in all
extremities without focal pyramidal or cranial nerve DISCUSSION
deficit. The anal sphincter tone was normal and there
was spontaneous voiding. She fixed and tracked Myelomeningocoele; the commonest anomaly of the
promptly to moving targets. A plain cervical spine X- cerebrospinal axis worldwide, is a complex malformation of
ray revealed spinal bifida defect and widening of the spinal cord, nerve roots, meninges, vertebral bodies
inter-pedicular distances of the C vertebral bodies and skin resulting from a failure of closure of the neural
4-7
(Fig. 2), while a trans-fontanel ultrasound scan (TFUSS) tube in the developing foetus during the first four weeks
and ultrasound of the neck swelling revealed dilated of gestation. Its distribution along the vertebral column
lateral, third and fourth ventricles without an evidence [6] is as follows: thoracic 5%, thoracolumbar 26%, lumbar
of a Chiari type II or III anomaly. The cost of a cranio- 26%, lumbosacral 10 %; with cervical myelomeningocoele
cervical magnetic resonance imaging or cervical (CMMC) being the least common (3%). In Nigeria, most
metrizamide myelography was not affordable to the reported series of CNS anomalies lacked a cervical
parents. Her packed cell volume was 38% and blood myelomeningocoele [2-4]. The current case is the first
chemistry was normal. cervical type to be reported from our centre.
A ventriculo-peritoneal shunting, using a medium
pressure Chhabra "Slit n spring" hydrocephalus shunt
system (G. Surgiwear; India) and a repair of the
myelomeningocoele were performed. The major findings
at spinal exploration were a myelomeningocoele with a
narrow neck, a dural fistula traceable into the spinal canal
via a C5 lamina defect through which whitish strands of
nerve rootlets connect the dorsal cervical cord to a
placode at the center of the dome. There was no split cord
malformation, syringomyelia or myelomeningocystocoele.
The nerve rootlets were detached bluntly from their
attachment to the placode and dropped into the spinal
canal. The reflected dura was double breasted and a
She was discharged ten days after revision and has
Fig. 3: Right and left parasagittal views of trans-fontanel ultrasound brain scan showing dilated right and left lateral
ventricles due to hydrocephalus
Am-Euras. J. Sci. Res., 2 (1): 60-62, 2007
62
The fact that neurological deficit worsens the higher patient also raised the suspicion of a Chiari type II [10]
a myelomeningocoele lesion is above the conus or III [11] malformation, but this was debunked by
appears to be restricted to patients with lumbo-sacral findings at ultrasound and surgical exploration.
and thoracic dysraphic lesions. Patients with cervical
lesions tend to retain normal neurology as in this CONCLUSION
and some other reported cases [5, 7]. In these patients
the neural nodule most likely constitutes a vertical Cervical myelomeningocoele associated with
outgrowth from a functioning spinal cord in the form hydrocephalus could present without a neurological
of a limited dorsal myeloschisis (LDM) and not a deficit.
terminal placode as it obtains with thoracic and
lumbosacral myelomeningocoele [8]. Hence these REFERENCES
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