Double Lumbar Localization of Myelomeningocele: Case Report

Introduction: Myelomeningocele (MMC) is a malformation resulting from the neural tube’s failure to close during embryonic development, and the majority of the cases of neural tube defects (NTDs) were prevalent as single location lesions along the spine; however, multiple NTDs (MNTDs) are a very rare condition. Only a few cases of MNTDs were found in the literature. Case Presentation: We report the case of a 2-month-old male infant prenatally diagnosed with MMC, presented with two unconnected lumbar and lumbosacral epidermal, soft, dome-shaped swellings located on both sides of the midline (paravertebral) covered by intact skin. MRI revealed double MMC at the level of L4-L5, with spinal nerve roots. The patient underwent surgical repair of the defects by replacing the spinal cord and its nerve roots inside the thecal sac and recreating a covering layer around the neural structures to resemble thecal sac. The outcome was favorable, and postoperative head CT scan did not show any complication. Conclusion: Our case report is considered the first from Algeria to report the condition and the first to report the occurrence of double lesions in the same spine region. MMC can be associated with neurological deficits or other congenital anomalies, thus it is necessary to thoroughly examine such patients. However, there was no antenatal folic acid deficiency in our case. We recommend antenatal care with adequate folic acid supplementation given that its deficiency during pregnancy is considered a ubiquitous risk factor for the condition. The optimal timing for surgery of MMC cases is 8 ± 5 days. Prenatal intrauterine repair of the condition provides favorable outcomes but carries high fetal and maternal risks. Surgical repair should include the sac removal, the reconstruction of the placode, and the closure of the overlying meninges. With early diagnosis and proper repair of such cases, MMC has good prognosis and favorable outcomes.

presented with two unconnected lumbar and lumbosacral epidermal, soft, dome-shaped swellings located on both sides of the midline (paravertebral) covered by intact skin. MRI revealed double MMC at the level of L4-L5, with spinal nerve roots. The patient underwent surgical repair of the defects by replacing the spinal cord and its nerve roots inside the thecal sac and recreating a covering layer around the neural structures to resemble thecal sac. The outcome was favorable, and postoperative head CT scan did not show any complication. Conclusion: Our case report is considered the first from Algeria to report the condition and the first to report the occurrence of double lesions in the same spine region. MMC can be associated with neurological deficits or other congenital anomalies, thus it is necessary to thoroughly examine such patients. However, there was no antenatal folic acid deficiency in our case. We recommend antenatal care with adequate folic acid supplementation given that its deficiency during pregnancy is considered a ubiquitous risk factor for the condition. The optimal timing for surgery of MMC cases is 8 ± 5 days. Prenatal intrauterine repair of the condition provides favorable outcomes but carries high fetal and maternal risks. Surgical repair should

Introduction
Spina bifida, also known as spinal dysraphia, is a congenital anomaly resulting from the neural tube's failure to close during embryonic development. There is a general assumption that neural tube defects (NTDs) prevalence in Africa is comparatively higher than in developed countries, which is estimated to reach 3.33 events per 1,000 observations in Africa and 0.94 per 1,000 in Europe [1,2]. Among the NTDs, spina bifida rate was the highest by an estimated 2.05 per 1,000 [1]. As Blencowe et al. [3] more than 75% of children born with NTDs die before 5 years, while in Algeria 54.4% of the cases died in utero [4]. There are two main dominant types of this condition: spina bifida oculta and spina bifida aperta. Aperta is the open form with high risks of spinal cord exposure to the environment, while oculta is the closed form with no spinal cord exposure [5]. There is no certainty regarding the etiology behind these malformations, but genetic, nutritional, and environmental factors are thought to play a significant role. In addition, a proper understanding of this etiology will play a significant role in reducing its prevalence. Interestingly, the observed prevalence of spina bifida varies globally and is influenced mainly by differences in surveillance methods, prenatal diagnosis, elective termination policies, and folic acid fortification of staple foods in a given country or region [6]. In the same context, the majority of the cases of NTDs were prevalent as single location lesions along the spine, mostly the lumbosacral spine (about 30-50%) [7]. However, very few cases of multiple location NTDs were reported in the literature, where NTDs were observed in more than one level along the vertebral column of an infant. Here, we present the case of an otherwise well, healthy male newborn presenting with double myelomeningocele (MMC), a type of open-form spinal dysgraphia. We will also discuss the timely, and planned surgical intervention as well as the postoperative outcome and prognosis.

Case Report
A 2-month-old male infant was presented to the department of neurosurgery at the University Hospital of Tlemcen for investigation of MMC in the lumbosacral area. The full-term patient was born vaginally with no other congenital defects and no family history of developmental or congenital anomalies.
His birth weight was 2,500 g. His mother did not take any medications and received appropriate antenatal care with folic acid supplementation. The neural defect was revealed during the antenatal follow-up by ultrasonography screening.
On clinical examination, the patient was vitally stable with no fever, the head circumference was average, and his fontanelle was flat and soft. Examination of the spine revealed normal alignment, and no abnormal curvature such as scoliosis. Primitive reflexes were present, and examination of the motor system showed a good tone without any motor-sensory deficit. He had two unconnected lumbar and lumbosacral epidermal, soft, dome-shaped swellings located on both sides of the midline (paravertebral) covered by intact skin, except at the base and between the two swellings where it was thinner without any other abnormality. The larger of the two swellings measured 41 mm in length and 31 mm in width and was in the lumbar region. The other swelling 35 mm in length and 23 mm in width was in the lumbosacral region (shown in Fig. 1a, b). The sacs were not erythematous, and there was no liquid leakage. Imaging of the spine by magnetic resonance imaging (MRI) revealed double MMC at the level of L4-L5, full of cerebrospinal fluid, with two nerve roots (shown in Fig. 2). The laboratory investigations did not reveal any particular abnormality.
The patient subsequently underwent surgical exploration and repair. The exploration found two spinal nerve roots at the level of the lumbar sac and one root at the level of the lumbosacral sac. Intraoperatively, the spinal cord and its nerve roots were put back inside the thecal sac which was reconstructed around the neural tissue using the surrounding soft tissue. This was followed by watertight closure of the reconstructed thecal sac to prevent cerebrospinal fluid leakage. Finally, the fascia and skin were closed in multiple layers (shown in Fig. 3).
The patient remained hospitalized for 4 days and showed favorable signs. The nonabsorbable skin stitches were removed on day 12, and a head CT imaging done after a month of the operation did not show any hydrocephalus (shown in Fig. 4).

Discussion
Spina bifida is a type of NTDs that occurs due to failure of complete fusion of the spinal neural tube. This process takes place in the first month of intrauterine life [6]. MMC is a subtype of open-form spina bifida [6]. It is one of the most common CNS congenital anomalies [3,8]. Its prevalence is significantly more common in developing countries, specifically India and African countries [9]. The condition is highly prevalent in Algeria, with the highest prevalence rate in Africa (75.0, 95% CI: 64.98, 85.02 per 10,000 live births), as per Oumer et al. [10]. The condition is mainly reported as a single defect within the spine. Multiple neural tube defects (MNTDs) are a very rare condition. Only a few cases of MNTDs were found in the literature. In their case series and literature review, Deora et al. [9] presented all the previous studies (case reports, case series, or retrospective studies) that reported MNTDs. To date, only about 60 cases of MNTDs, including those reported in Deora et al. study, have been reported in the literature.
Despite the high incidence of this congenital anomaly in Algeria, our case report is considered the first literature report from Algeria to report the condition. Most of the cases found in the literature were reported from India. Shiferaw et al. [11] reported a similar case to ours from Adis Ababa, Ethiopia.
MNTD is the presence of more than one defect within the child's spine. Most of the cases reported in literature presented with double lesions [9], which is consistent with the presentation of our patient. Our 2month male infant had two separate lesions in the lumbar and lumbosacral regions almost parallel to each other on both sides of the midline. Our case is the first to report the occurrence of double lesions in the same spine region. Most previously reported cases were cervicothoracic and lumbosacral MMCs [9,11].In addition, only a few cases were demonstrated in literature to have paravertebral MMC, most of them were associated with scoliosis [12,13], while our case has normal has spine alignment. Although MNTDs mostly present as two separate lesions, Srinivas et al., 2008 [14] revealed the occurrence of triple MMCs (cervicothoracic, lower thoracic, and lumbar) in one of the 2 cases reported in their study. Tekkök, 2005 also discussed a similar condition of triple NTD, but it consisted of double MMC and an encephalocele [15].
Although our case had appropriate antenatal care with adequate folic acid supplementation, folic acid deficiency during pregnancy is considered a ubiquitous risk factor for the condition [16]. Therefore, folic acid supplementation, especially in low-income countries, has become necessary to reduce the condition's prevalence in such countries [17]. Other risk factors for the condition may include environmental, genetic, and maternal diseases or medications. Some drugs (e.g., anticonvulsant valproate or carbamazepine, warfarin, retinoic acid) can precipitate into MMC [18,19].
The exact pathophysiology of the occurrence of this condition remains controversial. Many theories have explained the sequence that ends with the presence of MNTDs. Van Allen et al. first hypothesized the closure of the neural at multiple stages [20,21]. They defined five sites along the neural tube where the fusion process occurs (shown in Fig. 5). This process occurs in a definite order. It's initiated at site 4 (caudal rhombencephalon), followed by site 3 (stomatodeum) and site 2 (junction between prosencephalon and mesencephalon), then site 1 (mid-cervical level) and site 5 (caudal end of neural tube) [20]. MMC mainly results from closure defects at sites 1 and 5 [9]. Van Allen and his colleagues' theory were criticized for the uncommon incidence of MNTDs. In   [22,23]. A more acceptable theory than Van Allen et al. was proposed by Mahalik et al. [24] (the re-zippering initiation model). They theorized that the fusion process of the neural tube folds occurs segmentally (each segment independent of the next one). The interaction mediates this process between Sonic hedgehog and Wnt (Sonic hedgehog antagonist) depending on stimulation from the adjacent notochord. They discussed the reinitiation of the zipper process whenever interrupted. Thus, this theory can explain both single and multiple NTDs occurrence.
Our patient had no neurological deficits regarding the motor, sensory and autonomic functions. However, MMC can be associated with poor neurological functions in the form of paraparesis, poor sensory perception, and urinary or fecal incontinence [9]. Hence, proper neurological assessment of such cases is crucial and plays a vital role in improving the patient's outcomes. In the same context, our patient was free of any associated congenital anomalies. Elarjani et al. [25] reported the association of hydrocephalus and scoliosis with double upper thoracic and thoracolumbar MMC; in addition, 29.8% of NTD  Double Myelomeningocele: A Case Report from Algeria cases were associated with hydrocephalus in Algeria as Rintoul et al. [26].
The patient in this case was operated at 2 months of age. However, it is recommended to perform surgery as soon as possible, particularly in patients diagnosed prenatally, the optimal time for surgery of MMC cases is known to be within the first 5 days as per Oncel et al. [27]. The earlier the operation timing, the less the hospital stay and antibiotic therapy [27]. Mattogno et al. [28] reported that the surgical repair of MMC during the first 48 h greatly reduces the risk of infection in these patients. The MOMs trial demonstrated favorable outcomes of prenatal intrauterine repair of MMC [29]. The delay in performing the operation for our patient was due to the hospital's distance from the patient's home and the increase in the cost of transportation, with no nearby healthcare facility dealing with such cases.
The main aims of the surgical repair of MMC include the removal of the malformed sac, the reestablishment of the normal CSF environment around the malformed spinal cord and thus restoring the normal functions of the placode, in addition to protecting it from infection by recreating barrier with the exterior. These goals can be obtained through the microscopic reconstruction of the placode with multilayer closure of the covering meninges [30].
The favorable postoperative outcomes of our patient emphasize the good prognosis of such cases with minimal or no preoperative deficits, as reported in the literature [9,11,31]. However, postoperative follow-up by examination and imaging is a must to avoid complications or occult deficits in early infancy.
accordance with the World Medical Association Declaration of Helsinki. Written informed consent was obtained from the patient's parents for publication of the details of their medical case and any accompanying images.