Abstract

Background: While it is widely recognized that surgical intervention is crucial at birth, the occurrence of giant myelomeningocele in adulthood is exceedingly uncommon. This case study aims to provide a comprehensive overview of a rare presentation of myelomeningocele accompanied by a tethered cord in an adult patient, highlighting the clinical features, diagnostic imaging, and surgical repair technique employed.

Case Description: This patient is a 58-year-old woman with a history of spinal dysraphism that was diagnosed during childbirth. Due to her family’s low sociocultural status, she did not receive timely treatment. She was referred to our institution because of a persistent increase in volume in the lumbar region, accompanied by a continuous and intense pulsating headache. An magnetic resonance imaging revealed a giant spinal defect caused by lumbar dysraphism. A surgical rotational flap procedure and untethering of the spinal cord using microsurgical techniques were performed. During follow-up, the patient experienced a complete recovery of her clinical symptoms.

Conclusion: Surgical treatment can benefit adult patients with symptomatic myelomeningocele. This would influence the quality of life for the patient. For large lesions in the spine, adequate flap rotation demands knowledge of how to do it.

Keywords: Adulthood, Congenital spine defect, Myelomeningocele, Neuroplastic surgery, Surgical technique

INTRODUCTION

Myelomeningocele is a congenital malformation caused by a neural tube’s failure to closure during early embryonic development.[ 5 ] This condition is characterized by the herniation of the meninges and spinal cord tissue through an associated defect in the bony structures of the spine.[ 6 ] Depending on the level and severity of the spinal defect, patients with myelomeningocele often present with a variety of symptoms, which can include motor impairment, sensory deficits, and various complications affecting the urinary and gastrointestinal systems.[ 10 ]

The incidence of myelomeningocele is noted to be between 2.7 and 3.8 cases/10,000 live births, making it one of the more prevalent forms of neural tube defects in the pediatric population.[ 9 ] Early intervention is crucial for improving outcomes; therefore, affected infants typically undergo surgical correction within the first 72 h of life. This surgical treatment aims not only to close the defect and protect the exposed spinal cord and nerves but also to prevent further neurological damage and enhance the child’s quality of life as they grow. Ongoing management and rehabilitation are important components of care for individuals with myelomeningocele, addressing the numerous challenges that may arise throughout their development. The approach to this pathology in adulthood is limited due to poor clinical neurologic improvement that is reached, and few clinical data are documented in adulthood due to the rare presentation of the pathology.[ 2 , 4 ] Usually managed, conserved, and surgical repair when existing spillage of the cerebrospinal fluid (CSF), or for cosmetic correction purposes, can be indicated as a surgical treatment.[ 1 , 2 ]

Here, we present the atypical case of myelomeningocele accompanied by a tethered cord in adulthood, approached with an advancing flap, emphasizing some steps that could be considered for the neurosurgical trainee to reconstruct spinal dysraphism defects not previously described.

CASE DESCRIPTION

This patient is a 58-year-old woman who presented with a mass in her lower back during childbirth. Unfortunately, she did not receive timely treatment due to a low socioeconomic status. She was referred to our institution for an intermittently increased lumbar mass, accompanied by a continuous, intermittent pulsatile headache. Clinical examination revealed bilateral lower extremities hypotrophy with proximal paresis. Magnetic resonance imaging (MRI) revealed the posterior lumbar defect at the L3 level, with a large sac protrusion accompanied by a tethered cord [ Figure 1 ].

Figure 1:

Lumbosacral spine MRI. (a) MRI-PD, with hyper-intense CSF in the dural sac. L3 defect with dysraphism type myelomeningocele 89 × 66 × 58 mm, accompanied by a tethered cord (b) MRI-T1 with contrast, slightly reinforcing the dural sac. MRI: Magnetic resonance imaging, PD: Proton density, CSF: Cerebrospinal fluid.

Surgical management

A transverse incision in relation to Langerhans lines was performed over the dural sac while circularly preserving the lumbar muscles to obtain sufficient plane dissection for the transposition of the skin flaps, followed by a midline incision of the muscular plane and a circular dissection plane that maintains the dural sac. Preserving the thin muscular layers is primordial for covering the defect and reducing the percentage of CSF leaks. A vertical incision over the dural sac was accomplished [ Figure 2 ]. In this case, the spinal nerves were untethered from the dural sac. Previous electrostimulation was applied over the spinal roots before detaching them. The dural sac was dissected and removed, followed by a running stitch using 4-0 Prolene. Then, a primary closure with apposition of the muscular layers was performed in a usual fashion [ Figure 3 ].

Figure 2:

(a) Large defect, 18 × 12 cm. (b and c) Transverse incision over the lumbar defect, followed by a circumferential dissection and preservation of the lumbar muscular layers, is performed.

Figure 3:

(a) Neural tube defect, arrowhead point neural fiber attached to dural sac. (b) After the release, the fundus of the dural sac is visualized. (c) Dissection is realized between the muscular layers and the dural sac, followed by a primary closure over the dural defect with a continuous running stitch with Prolene 4-0. (d) Opposition of the muscular layers is performed, covering the defect to decrease the rate of CSF leak. CSF: Cerebrospinal fluid.

Flap reconstruction

After repairing the dural defect, the dissection is extended, and the direction and elevation of the fasciocutaneous flap with a rostral and caudal base are measured to maximize and preserve the adequate irrigation supply of the flap’s borders. Advancement of the flap in the caudal-rostral direction is achieved to cover the resultant defect over the midline. A cephalic incision is performed. In this manner, the tissue will be preserved in symmetry, and the fascial, subcutaneous, and cellular flaps will be adequately placed, taking care to avoid too much flap tension. A Penrose drain can be placed at the surgical site to prevent the accumulation of exudative inflammatory response secondary to the subcutaneous dissection and to reduce the infection rate [ Figures 4 and 5 ].

Figure 4:

(a) A midline mark is realized for a more accurate flap precision. (b) Then, an incision is performed. (c) It is important to use continuous flap mobilization to avoid inadequate overcutting of the flaps. (d) An extension at the level of the rostral flaps can be marked and cut to extend the resection of the rostral remnant tissue. A midline stitch can be placed in the midportion of the rostral and caudal flaps.

Figure 5:

(a) After the approximation of both flaps, an additional mark is realized until it is connected to the base of the flap. (b) The base of the rostral flap is cut until we get adequate flap tension (the blue dotted line marks the cuts of the caudal flap that can be removed to re-approximate the rostral and caudal flaps, taking care to follow the symmetry of the flaps. (c) Approximation of the rostral-caudal flap with simple stitches is realized to alleviate some tension of the flaps. Previous Penrose drainage was left. Then, the subdermal approximation is realized with a 3-0 monocrystal with a running stitch (d), and separate simple stitches with nylon 3-0 are placed.

The patient was discharged 72 h after surgery. The patient was followed for 12 months with no clinical evidence of headache [ Figure 6 ].

Figure 6:

(a and b) Sagittal MRI at 6 months with evidence of defect correction, tethered still present. MRI: Magnetic resonance imaging.

DISCUSSION

This study describes a rotation flap technique used on an adult patient with myelomeningocele. The goal is to offer a different visual perspective and enhance neurosurgeons’ overall concept of reconstructing a spinal defect.

The survival of a patient with myelomeningocele following its surgical treatment ranges between 30 and 40 years; therefore, finding an adult with this condition is rare.[ 1 , 2 , 7 , 12 ]

Some authors, Godzik et al.,[ 6 ] present the case of a 62-year-old woman who did not receive surgical repair at birth. The defect caused pain and CSF leakage through the defect; after the defect was repaired, the patient reported improved quality of life.[ 6 ] Özdemir et al.[ 11 ] also illustrate a case of a 41-year-old woman with a myelomeningocele-type defect repaired. They documented a decreased risk of infection and urinary and bladder control, with an overall improvement in her development and daily life.[ 11 ] Our clinical case involves different aspects to consider, such as the socioeconomic aspect, which was the main obstacle to receiving timely care; the emotional aspect is vital because it changes one’s perspective of life and the cognitive and physical aspects of societal development. In the case presented, the patient already exhibited motor compromise and atrophy. Correcting the lumbar defect would improve the patient’s quality of life and alleviate the ongoing pressure during movement and subsequent headaches. This last clinical symptom was not previously commented on in any article.

Due to its low presentation rate, there is no consensus regarding the surgical technique. The pediatric population involves several alternatives, including linear closure, unilateral or bilateral rhomboid flap, and rotation advancement flap. Disadvantages could include the high-tension flap and poor description in technical notes. The choice depends on the size of the lesion, the degree of infiltration into the adjacent tissue, and how proximal the communication of the meningocele with the lumbosacral dura is.[ 3 ] The key point is that an adequate dissection plane must be necessary to preserve the flap supply to enhance the cicatrization, decreasing the rate of infections with poor healing.[ 8 ]

There is no description of surgical repair for spinal defects in adulthood. In addition, some surgeons may not be familiar with this pathology or how to correct the defect, and the neurosurgeon may find it challenging to reconstruct when the overlying skin is abnormal. In this particular case, the patient experienced symptom improvement without complications and was able to lead a socially fulfilling life.

Neurosurgical intervention can benefit elderly patients with myelomeningocele; however, further research is needed. Patients often report a good quality of life. In addition, recent advances in managing adults with myelomeningocele are expected to enhance their life expectancy. Identifying factors that can improve the quality of life for seniors with this condition is crucial, as doing so can lead to better patient longevity and overall care.

CONCLUSION

Myelomeningocele dysraphism is one of the most common conditions resulting from improper closure of the neural tube. It is associated with various complications, such as hydrocephalus and tethered spinal cord, which can lead to neuropsychological delays. Therefore, timely treatment is essential to minimize lasting effects and promote healthy development. Access to neurosurgical care enables us to intervene promptly. The lumbosacral deficit is rarely seen in adulthood, and proper knowledge of operating these patients is mandatory to improve the quality of life. The presence of intractable headaches should be considered as a surgical indication to correct the lumbar defect.

Ethical approval:

Institutional review board approval is not required.

Declaration of patient consent:

Patient’s consent not required as patients identity is not disclosed or compromised. The authors certify that they have obtained institutional medical consent to use the clinical images.

Financial support and sponsorship:

Nil.

Conflicts of interest:

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation:

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

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