|Year : 2018 | Volume
| Issue : 3 | Page : 108-110
Congenital myelomeningocele and hydrocephalus: A clinical audit
Faisal Taleb1, Seraj Ajaj1, Samira Abudia2, Lali Ali Albakoush2
1 Department of Neurosurgery, Tripoli Medical Center, Tripoli, Libya
2 Department of Obstetric and Gynecology, Tripoli Medical Center, Tripoli, Libya
|Date of Web Publication||4-Oct-2018|
Dr. Faisal Taleb
Department of Neurosurgery, Tripoli Medical Center, Tripoli
Source of Support: None, Conflict of Interest: None
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 confirmed 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
|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
| 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. Significant 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|| |
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.
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 figures representing the frequency, percentage of the diagnosis, and findings 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 confirmed 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 findings of spina bifida occulta. MMC is the most severe form type of spina bifida 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. In our study, the cases presented with spinal or occipital midline swelling; some were intact and others were ruptured with cerebrospinal fluid leak. Patients with MMC present primarily with motor functional deficit of lower limbs, 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. In our study, 58% of mothers were not taken folic acid before and during pregnancy. Folic acid deficiency 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. Other potential causes can include folate antimetabolites (such as methotrexate), mycotoxins in contaminated corn meal, arsenic, hyperthermia in early development, and radiation., 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 MSAFP as a secondary screening tool. This is due to increased safety, increased sensitivity, and decreased false-positive rate of ultrasound as compared to MSAFP. Amniotic fluid alpha-fetoprotein and amniotic fluid acetylcholinesterase tests are also used to confirm if ultrasound screening indicates a positive risk. In this study, ultrasound examination was established in 70 cases during pregnancy and none had intrauterine fluid tested.
HCP also has a high prevalence in our community. It can be defined as a disturbance of cerebrospinal fluid formation, flow, or absorption, leading to an increase in volume of this fluid in the central nervous system. Congenital HCP refers to ventriculomegaly that develops in the fetal and infancy periods, often associated with macrocephaly. Intrauterine diagnosis of HCP during routine fetal anomaly scan at 18–22 weeks can be achieved by imaging studies. It is usually defined when the fetal lateral ventricular diameter is >15 mm. 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 first 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.
- 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
- More studies are crucial to determine outcome and possible environmental causes
- Electronic registry or database is mandatory to facilitate the future researches
- Specialized clinics for those cases include neurological surgery, pediatric orthopedic surgery, and pediatric urology surgery.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Adzick NS. Fetal myelomeningocele: Natural history, pathophysiology, and in-utero intervention. Semin Fetal Neonatal Med 2010;15:9-14.
Bruner JP, Tulipan N, Paschall RL, Boehm FH, Walsh WF, Silva SR, et al.
Fetal surgery for myelomeningocele and the incidence of shunt-dependent hydrocephalus. JAMA 1999;282:1819-25.
Vinck A, Nijhuis-van der Sanden MW, Roeleveld NJ, Mullaart RA, Rotteveel JJ, Maassen BA, et al.
Motor profile and cognitive functioning in children with spina bifida. Eur J Paediatr Neurol 2010;14:86-92.
Centers for Disease Control and Prevention (CDC). Spina bifida and anencephaly before and after folic acid mandate – United States, 1995-1996 and 1999-2000. MMWR Morb Mortal Wkly Rep 2004;53:362-5.
Yan L, Zhao L, Long Y, Zou P, Ji G, Gu A, et al.
Association of the maternal MTHFR C677T polymorphism with susceptibility to neural tube defects in offsprings: Evidence from 25 case-control studies. PLoS One 2012;7:e41689.
Suarez L, Brender JD, Langlois PH, Zhan FB, Moody K. “Pregnant”. Ann Epidemiol 2007;17:772-7.
Zhou FC, Fang Y, Goodlett C. Peptidergic agonists of activity-dependent neurotrophic factor protect against prenatal alcohol-induced neural tube defects and serotonin neuron loss. Alcohol Clin Exp Res 2008;32:1361-71.
Wilson RD; Sogc Genetics Committee, Special contributor. Prenatal screening, diagnosis, and pregnancy management of fetal neural tube defects. J Obstet Gynaecol Can 2014;36:927-39.
Milunsky A, Alpert E. Results and benefits of a maternal serum alpha-fetoprotein screening program. JAMA 1984;252:1438-42.
Rekate HL. A contemporary definition and classification of hydrocephalus. Semin Pediatr Neurol 2009;16:9-15.
Garne E, Loane M, Addor MC, Boyd PA, Barisic I, Dolk H, et al.
Congenital hydrocephalus – Prevalence, prenatal diagnosis and outcome of pregnancy in four European regions. Eur J Paediatr Neurol 2010;14:150-5.
Wyldes M, Watkinson M. Isolated mild fetal ventriculomegaly. Arch Dis Child Fetal Neonatal Ed 2004;89:F9-13.
[Table 1], [Table 2], [Table 3]