Neuropathology Evaluation of in Utero Correction of Myelomeningocele and Complications of Late-Onset GBS Infection

Abstract

Background: Myelomeningocele (MMC) causes significant morbidity and mortality. Efforts have been directed to correct this defect in utero. The neuropathology literature on antenatally repaired MMC and associated complications in humans is limited. Case report: A 12-day-old female, who underwent prenatal MMC repair via a two-layer closure (dural replacement patch, primary skin closure), was born at 34 weeks’ gestation. Her group B streptococcus positive mother received appropriate antepartum prophylactic antibiotics. She remained stable until day 11 of life when she underwent rapid clinical deterioration. Despite aggressive intervention, she expired on day 12. Review of placental pathology showed maternal and fetal inflammatory response. Autopsy revealed Gram-positive cocci and inflammation within the basilar leptomeninges and lumbosacral region. Neural and dermal elements were present within the MMC repair. Conclusion: This case documents integration of the dermal matrix patch to neural elements, adhering the spinal cord to scar tissue, the clinical implications of which remain unclear.

Keywords:

• spina bifida
• myelomeningocele
• dural patch
• fetal surgery
• neuropathology

Acknowledgments

We wish to acknowledge the family of the decedent for graciously allowing the pathologic and histologic studies. We want to acknowledge Moe Takeda, DO, Chris Lephong, DO, and Kurshad Cheeniba, MD for their performance of autopsy.

Author contributions

Neuropathology: SE and LJS; Fetal and neurosurgery: JC, DL and RHC; and clinical team: TW and AVS. The first draft of the manuscript was written by Drs. Edminster and Szymanski and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Ethics approval and consent to participate

This case study does not meet the definition of human subjects research and no ethical approval is required. Written informed consent was obtained from the parent to perform the full autopsy.

Consent for publication

The decedent’s parent has consented to the submission of the case report to the journal.

Availability of data and material

Not applicable.

Disclosure statement

The authors declare that no competing interests exist.

Funding

The author(s) reported there is no funding associated with the work featured in this article.