Postnatal myelomeningocele closure in smallest reported neonate: illustrative case

Momin M Mohis Department of Neurological Surgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin; and

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Kevin Cordeiro Department of Neurological Surgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin; and

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Sarah Larson Department of Neurological Surgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin; and

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Catharine Garland Division of Plastic Surgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin

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James A Stadler III Department of Neurological Surgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin; and

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BACKGROUND

Myelomeningocele (MMC) is the most serious form of spina bifida, a congenital defect in neural tube development. Defect closure in a patient with an extremely low birth weight presents unique challenges and risks; lower birth weight is associated with multiple organ system concerns, homeostasis is difficult, and local tissue is underdeveloped. To the authors’ knowledge, the present case is the lowest reported weight (490 g) for a neonate with postnatal MMC repair.

OBSERVATIONS

A preterm male with a prenatally diagnosed lumbosacral MMC and associated Chiari malformation type II was born at 23 weeks 1 day to a 29-year-old mother, gravidity 6 parity 4. The patient was medically stabilized and underwent MMC closure on day of life 5. His weight was 490 g at the time of this repair, and he did not have any surgical complications. At age 16 months, he underwent endoscopic third ventriculostomy with choroid plexus cauterization; he has not required any further hydrocephalus treatments since the last follow-up at 30 months of age.

LESSONS

To the authors’ knowledge, this case is the lowest birth weight ex utero MMC closure reported in the literature. Challenges of prematurity and size required appropriate preoperative stabilization, careful hemostasis and temperature regulation, and meticulous surgical technique.

ABBREVIATIONS

MMC = myelomeningocele; NICU = neonatal intensive care unit; OR = operating room

BACKGROUND

Myelomeningocele (MMC) is the most serious form of spina bifida, a congenital defect in neural tube development. Defect closure in a patient with an extremely low birth weight presents unique challenges and risks; lower birth weight is associated with multiple organ system concerns, homeostasis is difficult, and local tissue is underdeveloped. To the authors’ knowledge, the present case is the lowest reported weight (490 g) for a neonate with postnatal MMC repair.

OBSERVATIONS

A preterm male with a prenatally diagnosed lumbosacral MMC and associated Chiari malformation type II was born at 23 weeks 1 day to a 29-year-old mother, gravidity 6 parity 4. The patient was medically stabilized and underwent MMC closure on day of life 5. His weight was 490 g at the time of this repair, and he did not have any surgical complications. At age 16 months, he underwent endoscopic third ventriculostomy with choroid plexus cauterization; he has not required any further hydrocephalus treatments since the last follow-up at 30 months of age.

LESSONS

To the authors’ knowledge, this case is the lowest birth weight ex utero MMC closure reported in the literature. Challenges of prematurity and size required appropriate preoperative stabilization, careful hemostasis and temperature regulation, and meticulous surgical technique.

ABBREVIATIONS

MMC = myelomeningocele; NICU = neonatal intensive care unit; OR = operating room

Myelomeningocele (MMC) is the most common open neural tube defect, resulting from failure of primary neurulation. Neurological deficits vary, depending on the location of the lesion and extent of neurological damage, but they range from complete lower-extremity paralysis to isolated urinary incontinence.1 Damage to the spinal cord and peripheral nerves usually presents at birth and is irreversible despite early repair.2 Historically, standard management was postnatal surgical closure of the lesion. However, the 2011 Management of Myelomeningocele Study demonstrated improved outcomes for children with defects closed prenatally compared with postnatally.2 The risks of fetal MMC surgery include higher rates of preterm birth and a variety of intraoperative and postoperative complications to the mother and fetus, including chorioamniotic separation, placental abruption, pulmonary edema, and uterine dehiscence at the hysterotomy site in current and future pregnancies.2 In postnatal cases, there is controversy over the optimal timing of MMC lesion repair, but the literature suggests it should be closed within 24–48 hours to minimize the risk of infection.1,3 Early intervention is not associated with improvement in neurological function.1 Performing an operation on a patient with an extremely low birth weight presents unique challenges and risks; among these, a lower birth weight is associated with increased mortality.4

To our knowledge, the present case is the lowest reported weight (490 g) for a neonate with postnatal MMC repair. A review of the literature showed a case report of myeloschisis repair in a neonate weighing 599 g and an MMC repair in a neonate weighing 570 g.5,6 Aside from demonstrating technical feasibility, these cases add to the larger discussion regarding optimal closure timing together with a complex balancing of surgical risks in a fragile population.

Illustrative Case

A preterm male with a prenatally diagnosed L5–S1 MMC and associated Chiari malformation type II was born at 23 weeks 1 day to a 29-year-old mother, gravidity 6 parity 4, because of preterm labor. His birth weight was 570 g, and his weight the morning of surgery was 490 g. The MMC was covered with gauze and protected per our institutional protocol, and he was started on prophylactic ampicillin and cefepime. Intended closure of the MMC was planned as a combined case between the plastic and neurological surgery teams, but he was initially too unstable for MMC repair, preventing intervention within the generally accepted guidelines of 24–48 hours after birth. The planned MMC repair was delayed to allow safer transport of the patient to a neurosurgical center, respiratory optimization, and management of a spontaneous ileal perforation, which required separate placement of a peritoneal drain.

Exquisite care was taken by the anesthesia team throughout the perioperative course, given the patient’s prematurity and low birth weight. This included close temperature monitoring with appropriate prewarming of the operating room (OR) to prevent hypothermia, glucose monitoring to prevent both hypo- and hyperglycemia, and volume status monitoring in the event blood products were indicated. Additionally, anesthetic and analgesic agents were carefully titrated to prevent tachycardia or hypertension.

On day of life 5 (Fig. 1), the patient was brought into the OR. Given the patient’s small size, careful hemostasis was maintained throughout the operation. Only 2 mL of blood were lost during the neurosurgical portion of the case and 5 mL during the plastic surgery closure. Plastic surgery assistance was needed once placode and dural closures were achieved. Notably, the muscular fascia was released from the iliac wings on each side, allowing mobilization of skin and muscle medially to achieve final closure. The total length of the defect after closure was 3 cm, and the patient was transferred back to the neonatal intensive care unit (NICU) intubated in stable condition for further care. The patient was continued on prophylactic ampicillin and cefepime, and serial cranial imaging showed a small focus of hemorrhage within the left choroid plexus but no significant intraventricular hemorrhage or hydrocephalus. He was discharged back to the referring institution 1 month after birth for ongoing care related to prematurity, weighing 690 g (Fig. 2). During routine follow-up at age 15 months, his head circumference was noted to be deviating significantly from the expected growth curve, but he was otherwise clinically stable. At 16 months, he developed increased secretions, increased pooling of secretions, and enlarged ventricles. He subsequently underwent endoscopic third ventriculostomy with choroid plexus cauterization to treat his hydrocephalus. We performed this procedure in the hope of potentially avoiding a future shunt placement.

FIG. 1
FIG. 1

Preoperative MMC 5 days after birth.

FIG. 2
FIG. 2

Postoperative MMC 24 days after birth.

At his most recent neurosurgery follow-up at 30 months of age, he was eating without difficulty, walking without support, and achieving age-appropriate growth milestones. His imaging demonstrated a stably dilated ventricular system. He continues to be seen in follow-up by our multidisciplinary spina bifida team, which includes pediatric urology for his neurogenic bladder and bowel and pediatric orthopedics for his abnormal gait.

Patient Informed Consent

The necessary patient informed consent was obtained in this study.

Discussion

Observations

This report describes an ex utero MMC closure in an extremely low birth weight infant (490 g), the smallest and youngest reported neonate to undergo this procedure, to our knowledge. For comparison, the literature puts the average weight of a patient undergoing ex utero MMC closure at approximately 3 kg.7,8 Performing an operation on an extremely low birth weight neonate presents many risks and challenges, regardless of the type of surgery. The surgical technique is complicated by diminutive anatomical structures; meticulous hemostasis is essential; and maintenance of physiological conditions for the patient (temperature, hemodynamics) requires specialized anesthesiology care. Further risk was compounded in this case because of the inability to operate within 72 hours of delivery and medical concerns associated with prematurity.8,9 Cases of extreme prematurity often present with a variety of problems requiring immediate attention, delaying MMC closure.10 In our case, closure needed to wait until the infant was stabilized sufficiently to transport, and intervention was further delayed due to respiratory concerns and a spontaneous bowel perforation. Although the Spina Bifida Association recommends surgical repair within 0–2 days after birth, our patient’s clinical instability required delayed repair at 6 days after birth.11 The goal of early closure is to reduce the risk of infection such as meningitis and to prevent nerve damage.12 We do not believe that this delay in closure adversely affected the patient’s neurosurgical outcome. He never showed signs of infection and was ultimately able to be discharged back to the referring institution after 1 month with no significant complications related to the MMC closure.

Our patient’s MMC closure, although postnatal, occurred at the same gestational age as a typical prenatal closure.2 Closure at this early gestational age may, in part, account for his overall positive outcome. In particular, our patient has not required a shunt and is ambulating independently. Furthermore, post-closure magnetic resonance imaging did demonstrate good hindbrain herniation reversal. Despite this, our patient still developed progressive hydrocephalus by age 15 months. Although uncommon, this occurrence has been previously documented.13 Our patient underwent endoscopic third ventriculostomy with choroid plexus cauterization. The goal with this procedure was to prevent the need for a shunt, because shunting in spina bifida has been shown to be inversely associated with ambulatory status.14 At age 30 months, he had not required any further treatments for hydrocephalus and had been attaining age-appropriate milestones.

With increased public awareness of folate supplementation during pregnancy, the rate of neural tube defects has declined since the 1990s.10,15 Given the proven benefits, in utero MMC repair is increasingly used when clinically appropriate.16 However, some patients require ex utero repair in the setting of medical complexity due to extreme prematurity, and particular care must be taken to promote positive outcomes in these patients.

With this case, we hope to illuminate potential areas of concern when managing an extremely low birth weight neonate with MMC and show that successful closure is possible in this patient population.

Lessons

This case stresses the importance of working in tandem with the NICU to manage risks and complications related to extreme prematurity and concurrent extreme low birth weight to ensure optimal patient stability for timely MMC closure. Improving patient outcomes requires not only excellent short-term surgical and nonsurgical care but also comprehensive longitudinal care from a multidisciplinary team. In our case, the patient continues to be seen in follow-up in our spina bifida clinic, which includes pediatric urology, for his neurogenic bowel and bladder, and pediatric orthopedics, for his abnormal gait. For children requiring MMC closure while facing considerable risks from prematurity, careful and individualized multidisciplinary care is critical to promoting positive outcomes.

Author Contributions

Conception and design: Stadler, Cordeiro, Garland. Acquisition of data: Stadler. Analysis and interpretation of data: Stadler, Cordeiro, Garland. Drafting the article: Mohis, Cordeiro. Critically revising the article: Stadler, Mohis, Cordeiro, Larson. Reviewed submitted version of manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Stadler. Administrative/technical/material support: Stadler. Study supervision: Stadler.

References

  • 1

    Greenberg MS Handbook of Neurosurgery. 9th ed. Thieme; 2020.

  • 2

    Adzick NS, Thom EA, Spong CY, et al. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 2011;364(11):9931004.

  • 3

    Beier AD, Nikas DC, Assassi N, et al. Congress of Neurological Surgeons systematic review and evidence-based guideline on closure of myelomeningocele within 48 hours to decrease infection risk. Neurosurgery. 2019;85(3):E412E413.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4

    Wong LYC, Paulozzi LJ Survival of infants with spina bifida: a population study, 1979-94. Paediatr Perinat Epidemiol. 2001;15(4):374378.

  • 5

    Oi S, Miwa T, Kobayashi M, Ida H Myeloschisis repair in a premature neonate with a birth weight of 599 g. Pediatr Neurosurg. 2011;47(5):379382.

  • 6

    Ucak M Myelomeningocele closure by unilateral lumbar artery perforator flap: experience with thirty-eight patients. Microsurgery. 2018;38(7):752757.

  • 7

    Mayer S, Weisser M, Till H, Gräfe G, Geyer C Congenital myelomeningocele - do we have to change our management? Cerebrospinal Fluid Res. 2010;7(1):17.

  • 8

    Bulbul A, Can E, Bulbul LG, Cömert S, Nuhoglu A Clinical characteristics of neonatal meningomyelocele cases and effect of operation time on mortality and morbidity. Pediatr Neurosurg. 2010;46(3):199204.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    Alnaami IM, Alayad EG Review on myelomeningocele management and its current status in Saudi Arabia. Neurosciences (Riyadh). 2019;24(1):510.

  • 10

    Platt MJ Outcomes in preterm infants. Public Health. 2014;128(5):399403.

  • 11

    Kancherla V, Ma C, Grant G, et al. Factors associated with timeliness of surgical repair among infants with myelomeningocele: California Perinatal Quality Care Collaborative, 2006 to 2011. Am J Perinatol. 2020;37(12):12341242.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Attenello FJ, Tuchman A, Christian EA, et al. Infection rate correlated with time to repair of open neural tube defects (myelomeningoceles): an institutional and national study. Childs Nerv Syst. 2016;32(9):16751681.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 13

    Lu VM, Snyder KA, Ibirogba ER, Ruano R, Daniels DJ, Ahn ES Progressive hydrocephalus despite early complete reversal of hindbrain herniation after prenatal open myelomeningocele repair. Neurosurg Focus. 2019;47(4):E13.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 14

    Dicianno BE, Karmarkar A, Houtrow A, et al. Factors associated with mobility outcomes in a national spina bifida patient registry. Am J Phys Med Rehabil. 2015;94(12):10151025.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Kancherla V Neural tube defects: a review of global prevalence, causes, and primary prevention. Childs Nerv Syst. 2023;39(7):17031710.

  • 16

    Moldenhauer JS, Adzick NS Fetal surgery for myelomeningocele: after the Management of Myelomeningocele Study (MOMS). Semin Fetal Neonatal Med. 2017;22(6):360366.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • Collapse
  • Expand
  • 1

    Greenberg MS Handbook of Neurosurgery. 9th ed. Thieme; 2020.

  • 2

    Adzick NS, Thom EA, Spong CY, et al. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 2011;364(11):9931004.

  • 3

    Beier AD, Nikas DC, Assassi N, et al. Congress of Neurological Surgeons systematic review and evidence-based guideline on closure of myelomeningocele within 48 hours to decrease infection risk. Neurosurgery. 2019;85(3):E412E413.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4

    Wong LYC, Paulozzi LJ Survival of infants with spina bifida: a population study, 1979-94. Paediatr Perinat Epidemiol. 2001;15(4):374378.

  • 5

    Oi S, Miwa T, Kobayashi M, Ida H Myeloschisis repair in a premature neonate with a birth weight of 599 g. Pediatr Neurosurg. 2011;47(5):379382.

  • 6

    Ucak M Myelomeningocele closure by unilateral lumbar artery perforator flap: experience with thirty-eight patients. Microsurgery. 2018;38(7):752757.

  • 7

    Mayer S, Weisser M, Till H, Gräfe G, Geyer C Congenital myelomeningocele - do we have to change our management? Cerebrospinal Fluid Res. 2010;7(1):17.

  • 8

    Bulbul A, Can E, Bulbul LG, Cömert S, Nuhoglu A Clinical characteristics of neonatal meningomyelocele cases and effect of operation time on mortality and morbidity. Pediatr Neurosurg. 2010;46(3):199204.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    Alnaami IM, Alayad EG Review on myelomeningocele management and its current status in Saudi Arabia. Neurosciences (Riyadh). 2019;24(1):510.

  • 10

    Platt MJ Outcomes in preterm infants. Public Health. 2014;128(5):399403.

  • 11

    Kancherla V, Ma C, Grant G, et al. Factors associated with timeliness of surgical repair among infants with myelomeningocele: California Perinatal Quality Care Collaborative, 2006 to 2011. Am J Perinatol. 2020;37(12):12341242.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Attenello FJ, Tuchman A, Christian EA, et al. Infection rate correlated with time to repair of open neural tube defects (myelomeningoceles): an institutional and national study. Childs Nerv Syst. 2016;32(9):16751681.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 13

    Lu VM, Snyder KA, Ibirogba ER, Ruano R, Daniels DJ, Ahn ES Progressive hydrocephalus despite early complete reversal of hindbrain herniation after prenatal open myelomeningocele repair. Neurosurg Focus. 2019;47(4):E13.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 14

    Dicianno BE, Karmarkar A, Houtrow A, et al. Factors associated with mobility outcomes in a national spina bifida patient registry. Am J Phys Med Rehabil. 2015;94(12):10151025.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Kancherla V Neural tube defects: a review of global prevalence, causes, and primary prevention. Childs Nerv Syst. 2023;39(7):17031710.

  • 16

    Moldenhauer JS, Adzick NS Fetal surgery for myelomeningocele: after the Management of Myelomeningocele Study (MOMS). Semin Fetal Neonatal Med. 2017;22(6):360366.

    • PubMed
    • Search Google Scholar
    • Export Citation

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