Congenital Myelomeningocele and hydrocephalus. A clinical Audit

Abstract

Congenital Myelomeningocele and congenital Hydrocephalus are among the most common pediatric neurosurgery diseases in Libya. This paper elucidates the experience of authors in clinical audit of seventy four cases diagnosed as congenital Myelomeningocele and hydrocephalus, in the period of time (Feb 2017 to Dec 2017). Materials and Methods: This was a prospective study of 74 cases whose data were collected as per the pre‑prepared data sheet in a single tertiary Institute in Tripoli (Tripoli Medical Center) from February 2017 and December 2017.

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108
Abstract
Short Communication
IntRoductIon
Congenital myelomeningocele (MMC) and hydrocephalus
(HCP) are common pediatric neurosurgical conditions in
our country, and it is a major contributor to the local burden
of surgically treatable diseases. MMC is characterized by
a protrusion of the meninges and spinal cord through open
vertebral arches, which results in varying degrees of paralysis,
mental retardation, bowel and bladder dysfunction, as well as
orthopedic disabilities.[1] Signicant health disparities exist
for the treatment of MMC and HCP in developing countries
due to a combination of medical and socioeconomic factors.
This clinical audit reviews 74 cases in single tertiary care
hospital in Tripoli, Libya. It was conducted by collaboration
of neurological surgery and obstetric departments of a tertiary
care hospital in Tripoli (Tripoli Medical Center). Our aim was
to determine the magnitude of cases and an overview of the
current challenges and future directions of neurosurgical care
for children with congenital MMC and HCP in Libya.
MateRIals and Methods
Setting
This was a prospective study of 74 cases whose data were
collected as per the pre‑prepared data sheet in a single tertiary
institute in Tripoli (Tripoli Medical Center) from February
2017 and December 2017.
Background: Congenital Myelomeningocele and congenital Hydrocephalus are among the most common pediatric neurosurgery diseases
in Libya. This paper elucidates the experience of authors in clinical audit of seventy four cases diagnosed as congenital Myelomeningocele
and hydrocephalus, in the period of time (Feb 2017 to Dec 2017). Materials and Methods: This was a prospective study of 74 cases whose
data were collected as per the pre‑prepared data sheet in a single tertiary Institute in Tripoli (Tripoli Medical Center) from February 2017
and December 2017. Results: Management of 74 patients with complete data was analyzed. Of 74 patients, 20 (27%) patients were isolated
MMC, 25 (34%) were isolated HCP, and 29 (39%) occurring in association with MMC. From a total of 74 patients, 34 (45%) were male and
40 (54%) were female. Furthermore, 27 (36%) patients delivered as premature infant and 47 (63%) were full term. The method of delivery
was by cesarean section in 69 (93%) cases and by normal vaginal delivery in 5 cases (1%). The prenatal diagnosis of MMC and HCP using
ultrasound was established in 70 (94%) cases. Head circumference of all cases at the time of delivery was ranged from 33 to 56 cm.The
surgical management of all 74 cases after the diagnosis in our neurosurgical department was as follows: Ventriculoperitoneal (VP) shunts
inserted in 63 cases (85%). MMC repair was performed in 38 cases (51%). Most patients 55 (74%) were discharged routinely after VP shunt
insertion and MMC repair, while 19 (26%) died in hospital. Further, folic acid intake by dose of (0.4 mg) orally was documented in 31 (41%)
pregnant women, and 43 (58%) cases were not taken folic acid. The timing of folic acid intake was after the pregnancy was conrmed in all
cases. Conclusion: This review demonstrates a single‑institute experience and the current challenges in the management of both MMC and
HCP in Libya. The Awareness of the mandatory intake of folic in our society is a national call.
Keywords: Clinical audit, Computer Tomography (CT SCAN), hydrocephalus, myelomeningocele, neural tube defects,
ventriculoperitoneal shunt
Address for correspondence: Dr. Faisal Taleb,
Department of Neurosurgery, Tripoli Medical Center, Tripoli, Libya.
E‑mail: talebfaisal@gmail.com
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DOI:
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How to cite this article: Taleb F, Ajaj S, Abudia S, Albakoush LA.
Congenital myelomeningocele and hydrocephalus: A clinical audit. Libyan
J Med Sci 2018;2:108‑10.
Congenital Myelomeningocele and Hydrocephalus: A Clinical
Audit
Faisal Taleb, Seraj Ajaj, Samira Abudia1, Lali Ali Albakoush1
Departments of Neurosurgery and 1Obstetric and Gynecology, Tripoli Medical Center, Tripoli, Libya
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Taleb, et al.: Congenital myelomeningocele and hydrocephalus
Libyan Journal of Medical Sciences ¦ Volume 2 ¦ Issue 3 ¦ July‑September 2018 109
Data analysis
Data were collected by the authors on this particular topic,
and the information was analyzed in MS Excel. The source
of data was from the prepared data sheet for each patient,
imaging results, and operation notes. The tables and gures
representing the frequency, percentage of the diagnosis, and
ndings were summarized.
Results
Management of 74 patients with complete data was analyzed.
Of 74 patients, 20 (27%) patients were isolated MMC, 25 (34%)
were isolated HCP, and 29 (39%) occurring in association with
MMC. The detailed description of the 49 patients with MMC
is summarized in Table 1.
From a total of 74 patients, 34 (45%) were male and 40 (54%)
were female. Furthermore, 27 (36%) patients delivered as
premature infant and 47 (63%) were full term. The method
of delivery was by cesarean section in 69 (93%) cases and
by normal vaginal delivery in 5 cases (1%). The prenatal
diagnosis of MMC and HCP using ultrasound was established
in 70 (94%) cases. Head circumference of all cases at the time
of delivery was ranged from 33 to 56 cm [Table 2].
The surgical management of all 74 cases after the
diagnosis in our neurosurgical department was as follows:
Ventriculoperitoneal (VP) shunts inserted in 63 cases (85%).
MMC repair was performed in 38 cases (51%). Most patients
55 (74%) were discharged routinely after VP shunt insertion
and MMC repair, while 19 (26%) died in hospital. The timing
of surgical intervention was based on clinical setting and not
by frame period in all cases. Maternal sociodemographic
characteristics in terms of age, parity, and consanguinity to
the spouse are summarized in Table 3. Further, folic acid
intake by dose of (0.4 mg) orally was documented in 31 (41%)
pregnant women, and 43 (58%) cases were not taken folic acid.
The timing of folic acid intake was after the pregnancy was
conrmed in all cases.
dIscussIon
Neural tube defects (NTDs) are a group of congenital disabilities
in which an opening in the spinal cord or brain remains from
early in human development. MMC is by far the most common
type. The pathophysiology of MMC starts during the 3rd week
of pregnancy called gastrulation, in which specialized cells on
the dorsal side of the embryo begin to change shape and form
the neural tube. When the neural tube does not close completely,
an NTD develops. NTD has a range of presentations, from
stillbirth to incidental radiographic ndings of spina bida
occulta. MMC is the most severe form type of spina bida that
results from failure of caudal neurulation during the 4th week
of gestation, which leads to a posterior midline defect with
exposed meninges and dysplastic neural tissue.[2] In our study,
the cases presented with spinal or occipital midline swelling;
some were intact and others were ruptured with cerebrospinal
uid leak. Patients with MMC present primarily with motor
functional decit of lower limbs,[3] 18 cases with paraplegia
were documented in this clinical audit.
The etiology of MMC in the majority of cases is multifactorial,
including genetic, racial, and environmental factors, in which
malnutrition particularly of folic acid intake is documented.[4]
In our study, 58% of mothers were not taken folic acid before
and during pregnancy. Folic acid deciency itself does not
cause NTD. The association seen between reduced NTD
and folic acid supplementation is due to gene–environment
interaction such as vulnerability caused by the C677T
methylenetetrahydrofolate reductase variant. Supplementing
folic acid during pregnancy reduces the prevalence of NTD
by not exposing this, otherwise subclinical mutation to
aggravating conditions.[5] Other potential causes can include
folate antimetabolites (such as methotrexate), mycotoxins
in contaminated corn meal, arsenic, hyperthermia in early
development, and radiation.[6,7] None of our cases were
subjected to those factors.
The intrauterine diagnostic tests for NDT include ultrasound
examination and measurement of maternal serum
alpha‑fetoprotein (MSAFP). Second‑trimester ultrasound is
recommended as the primary screening tool for NTD and
Table 1: Diagnostic profile of 49 cases of
myelomeningocele
Description Frequency (%)
MMC anatomical sites
Occipital/cervical 1 (2)
Thoraco/lumbar 37 (78)
Foot and ankle deformity 32 (65)
Rupture sac 32 (65)
Paraplegia 18 (37)
MMC: Myelomeningocele
Table 2: Distribution of head circumferences of the
audited 74 patients
Head circumference (cm) Frequency (%)
33‑38 34 (46)
39‑44 16 (22)
45‑50 8 (11)
51‑56 2 (3)
Table 3: Maternal sociodemographic characteristics
Sociodemographic characteristics Frequency (%)
Age (years)
20‑30 32 (43)
Above 30 46 (62)
Parity
Primigravida 8 (10)
2nd gravid 20 (27)
3rd gravid or more 46 (62)
Consanguinity 15 (20)
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Taleb, et al.: Congenital myelomeningocele and hydrocephalus
Libyan Journal of Medical Sciences ¦ Volume 2 ¦ Issue 3 ¦ July‑September 2018
110
MSAFP as a secondary screening tool.[8] This is due to increased
safety, increased sensitivity, and decreased false‑positive
rate of ultrasound as compared to MSAFP.[9] Amniotic uid
alpha‑fetoprotein and amniotic uid acetylcholinesterase tests are
also used to conrm if ultrasound screening indicates a positive
risk.[9] In this study, ultrasound examination was established in
70 cases during pregnancy and none had intrauterine uid tested.
HCP also has a high prevalence in our community. It can be
dened as a disturbance of cerebrospinal uid formation, ow,
or absorption, leading to an increase in volume of this uid
in the central nervous system.[10] Congenital HCP refers to
ventriculomegaly that develops in the fetal and infancy periods,
often associated with macrocephaly.[11] Intrauterine diagnosis
of HCP during routine fetal anomaly scan at 18–22 weeks can
be achieved by imaging studies. It is usually dened when the
fetal lateral ventricular diameter is >15 mm.[12] Twenty‑nine
cases were diagnosed as congenital HCP in our review and all
were nonobstructive type.
The main treatment of both congenital MMC and HCP is by
neurosurgical intervention. Since our surgical intervention
was based on clinical sitting, MMC repair was during the
rst few days of life and then followed with insertion of
VP shunts (ultra‑small size and low pressure). Almost all of
our patients with MMC had foot deformity with clubfoot as
dominant type. Therefore, orthopedic management should be
taken along neurosurgical care.
conclusIon
This review demonstrates a single‑institute experience and the
current challenges in the management of both MMC and HCP
in Libya. Further studies in multiple centers are mandatory to
reveal the overall magnitude and outcome of these conditions
in Libya.
Recommendations
1. National awareness of the mandatory intake of folic
acid at 0.4 and 4 mg in low‑risk and high‑risk mothers,
respectively, is a national call
2. More studies are crucial to determine outcome and
possible environmental causes
3. Electronic registry or database is mandatory to facilitate
the future researches
4. Specialized clinics for those cases include neurological
surgery, pediatric orthopedic surgery, and pediatric
urology surgery.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conicts of interest.
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