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A Case of Junctional Neural Tube Defect Associated with a Lipoma of the Filum Terminale: a New Subtype of Junctional Neural Tube Defect?

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A Case of Junctional Neural Tube Defect Associated with a Lipoma of the Filum Terminale: a New Subtype of Junctional Neural Tube Defect? CASE REPORT J Neurosurg Pediatr 21:601–605, 2018 A case of junctional neural tube defect associated with a lipoma of the filum terminale: a new subtype of junctional neural tube defect? Simona Mihaela Florea, MD,1 Alice Faure, MD,2 Hervé Brunel, MD,3 Nadine Girard, MD, PhD,3 and Didier Scavarda, MD1 Departments of 1Pediatric Neurosurgery, 2Pediatric Surgery, and 3Neuroradiology, Hôpital Timone Enfants, Marseille, France The embryological development of the central nervous system takes place during the neurulation process, which in- cludes primary and secondary neurulation. A new form of dysraphism, named junctional neural tube defect (JNTD), was recently reported, with only 4 cases described in the literature. The authors report a fifth case of JNTD. This 5-year-old boy, who had been operated on during his 1st month of life for a uretero-rectal fistula, was referred for evaluation of possible spinal dysraphism. He had urinary incontinence, clubfeet, and a history of delayed walking ability. MRI showed a spinal cord divided in two, with an upper segment ending at the T-11 level and a lower segment at the L5–S1 level, with a thickened filum terminale. The JNTDs represent a recently classified dysraphism caused by an error during junctional neurulation. The authors suggest that their patient should be included in this category as the fifth case reported in the literature and note that this would be the first reported case of JNTD in association with a lipomatous filum terminale. https://thejns.org/doi/abs/10.3171/2018.1.PEDS17492 KEYWORDS junctional neurulation; junctional neural tube defect; spina bifida; dysraphism; spine; congenital HE central nervous system and vertebrae are formed or lipomas of the filum terminale.16 When there are altera- during the neurulation process that occurs early in tions present in both the primary and secondary neurula- the embryonic life and is responsible for the trans- tion we can find mixed dysraphisms that present with ele- Tformation of the flat neural plate into the neural tube (NT). ments from the 2 forms of neural tube defects (NTDs). Primary neurulation allows the formation of the brain and Recent papers have brought into discussion a new class the spinal cord down to the junction between the S-1 and of spinal dysraphisms that result from disturbances that S-2 segments, whereas secondary neurulation is respon- appear in the short period between the end of primary sible for the formation of the spinal cord segments distal to neurulation, marked by closure of the caudal neuropore, the S1–2 junction. and the beginning of secondary neurulation, consisting of Alteration of primary neurulation leads to malforma- the formation of the caudal cell mass.9,10,20,21 The phase be- tions of the spine and spinal cord known as open neural tween the end of primary neurulation and the beginning tube defects (ONTDs) in their most severe form or limited of secondary neurulation was termed junctional neurula- dorsal myeloschisis (LDM) in the lesser.6,18,19 tion,4 and it is thought to be responsible for maintaining a When the secondary neurulation process is impaired, continuity between the fully formed primary NT and the the result is either a defect of formation that leads to an secondary NT that begins to form. The alteration of this absent conus and a short spinal cord, a condition known transitional process leads to the apparition of a new dysra- as caudal agenesis;15 or a defect during its regression as we phism called junctional neural tube defect (JNTD).4,5,20,22 can see in terminal myelocystocele, ventriculus terminalis, In this paper, we report another case that we believe ABBREVIATIONS JNTD = junctional neural tube defect; LDM = limited dorsal myeloschisis; MET = mesenchymal-epithelial transition; NSB = node-streak border; NT = neural tube; NTD = NT defect; ONTD = open NTD. SUBMITTED August 30, 2017. ACCEPTED January 3, 2018. INCLUDE WHEN CITING Published online March 30, 2018; DOI: 10.3171/2018.1.PEDS17492. ©AANS 2018, except where prohibited by US copyright law J Neurosurg Pediatr Volume 21 • June 2018 601 Unauthenticated | Downloaded 09/25/21 11:19 PM UTC S. M. Florea et al. FIG. 3. Axial T1-weighted MR image obtained at the S-3 level, showing the lipomatous filum terminale black( arrow). context of overall urinary incontinence. He had bilateral clubfeet for which he had undergone multiple interven- tions as well as orthopedic treatment such as immobiliza- tion in plaster splints. Examination of his lower extremities showed weakness of multiple muscles and atrophy. He had FIG. 1. Sagittal T2-weighted MR images of the thoracic-lumbar spine acquired the ability to walk with some delay, at about 2 showing the 2 segments of the spinal cord and the tissue band that con- years old, most probably related to the clubfeet. The patient nects them. The upper segment ends at the T11–12 level (white arrow), exhibited no cutaneous signs of occult spina bifida. whereas the lower segment is located at the L5–S1 level (gray arrow). Imaging can be included in this new class of dysraphisms described An MRI examination of the spinal cord was performed as JNTDs and discuss implications for the embryological (Figs. 1 and 2). This study showed a spinal cord divided in processes that lead to NTDs. two, with the upper segment ending abruptly at the T11–12 level and the lower segment located at the L5–S1 level and ending with a filum that was slightly thicker than normal, Case Report suggesting a slightly lipomatous nature (Fig. 3). The 2 seg- History and Presentation ments were connected by a 9.5-cm-long tract. The maxi- This 5-year-old boy was referred to our service for eval- mum thickness of the long tract connecting the 2 parts of uation of possible dysraphism. He had multiple malforma- the dysraphic spinal was 3 mm. A partial sacral agenesis tions. He had undergone surgical repair of a ureteral-rectal was observed, as well as other vertebral anomalies in the fistula at 1 month of age, and he had an anteriorly dis- cervical and thoracic regions. placed anus and partial sacral agenesis. He suffered from a hypertonic anal sphincter and was treated for constipa- Operation and Postoperative Course tion on a chronic basis. Urodynamic testing showed that Given the continence disturbances and the MRI ap- his bladder had a tendency for urinary retention, within the pearance of the filum, we suspected that the patient had a FIG. 2. Axial T2-weighted MR images. A: Image obtained at the T-11 level, showing the upper segment of the spinal cord (ar- row). B: Image obtained at the L-2 level, showing the band (arrow) that connects the 2 segments of the spinal cord. C: Image obtained at the L-5 level, showing the lower segment of the spinal cord (arrow). 602 J Neurosurg Pediatr Volume 21 • June 2018 Unauthenticated | Downloaded 09/25/21 11:19 PM UTC S. M. Florea et al. severe forms of ONTDs such as anencephaly, encephalo- celes, or myelomeningoceles to less severe malformations, known as LDMs.6,18,19 Secondary neurulation results in the formation of the secondary NT and it is essentially different from the pri- mary neurulation. During this phase, the caudal cell mass passes through a process called mesenchymal-epithelial transition (MET)2,3,7,8,11,13,24,27,28 and is eventually respon- sible for the formation of the spinal cord segment from S-2 distally, together with the filum terminale, but also for the development of most of the hind gut, lower genitourinary tract, and sacrococcygeal osseous elements.9,10,12,13 Impair- ment of this process results in caudal agenesis or regression syndromes, which are frequently associated with visceral and vertebral malformation due to the common origin of these entities.5,10,11,14–17,21,31 Recently a third category of NTDs, named junctional neural tube defects (JNTDs), has been proposed. These defects that are thought to originate from alterations of the transitional process between primary neurulation, marked by the closing of the posterior neuropore and the begin- ning of secondary neurulation.7 In their 2014 article, Dady et al.4 described the mechanisms by which the continuity FIG. 4. Sagittal T2-weighted images obtained 3 months after surgery. of the 2 segments of the spinal cord resulting from the well-known neurulation processes is maintained. Accord- ing to their work, the cells located between the Hensen’s tethered cord and proposed surgical sectioning of the lipo- node and the cranial end of the primitive streak, which matous filum. Intraoperatively we found a thickened filum they named “node-streak border” (NSB) are influenced in terminale, with a macroscopically lipomatous aspect, but their activity by the expression of the SOX-2 protein. Dady we observed that there was no tension in the filum during and colleagues demonstrated that the SOX-2–positive cells its manipulation. Despite the lack of tension, we decided to located dorsally will move dorsolaterally and be part of perform the sectioning as planned and analyze the resect- ed tissue microscopically, given that the appearance was the primary neural tube, while the cells that become SOX- compatible with a filum terminale lipoma. At 3 months af- 2 positive during the MET process will migrate caudally ter the surgery, there was no improvement concerning the and participate at the formation of the secondary NT, dur- urinary troubles, and there was no change on MRI with ing secondary neurulation. The SOX-2 positivity of the regard to the position of the lower segment of the spinal NSB cells maintains a connection between the dorsolateral cord (Fig.
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