Elsevier

Pediatric Neurology

Volume 40, Issue 5, May 2009, Pages 377-379
Pediatric Neurology

Case Report
MRI of Pneumorachis in a Newborn Infant With Open Myelomeningocele

https://doi.org/10.1016/j.pediatrneurol.2008.11.019Get rights and content

Pneumorachis, or air within the spinal canal, is a very rare radiographic finding, frequently of traumatic origin. A newborn infant with an open lumbosacral myelomeningocele presented with severe paraparesis and an unexpected dysfunction of the brainstem with somnolence, irregular respiration, and motor deficits of the upper extremities. Magnetic resonance imaging revealed hypoplasia of the brainstem and cerebellum and low-signal lesions on T2-weighted images at the cervicocranial junction, in the lateral ventricles, and in the spinal canal. Computed tomography confirmed the presence of intracranial and intraspinal air. The air disappeared 2 weeks later according to magnetic resonance imaging, but no clinical improvement occurred, even after 6 months. Pneumorachis and pneumocephalus can occur in newborn infants as an unexpected complication of an open myelomenigocele, with or without signs. The radiographic appearance of intraspinal air on magnetic resonance imaging was not described previously.

Introduction

Pneumorachis, or intraspinal air, is an extremely rare radiographic finding, especially in the pediatric population. Etiologies include trauma (e.g., skull fractures or paranasal sinus and subarachnoid fistula after thoracic or spinal injury), iatrogenic lesions (e.g., after lumbar puncture, thoracotomy, and proctocolectomy), and malignancy with associated therapy [1], [2], [3], [4], [5], [6], [7], [8]. Nontraumatic pneumorachis (e.g., spontaneous, infection-related, or tumor-related) is even more exceptional [1], [4]. Pediatric nontraumatic pneumocephalus, as detected by ultrasound, x-rays of the skull, or cranial computed tomography, has been described mostly as a complication of mechanical ventilation and tension pneumothorax, and very rarely as a complication of a congenital defect of the dura, i.e., open myelomeningocele in newborn infants [9], [10], [11], [12]. Intracranial air is thought to be the result of air entering the (enlarged) central canal through an open myelomeningocele, and ascending into the ventricular system. Air penetration into the spinal canal through a neural tube defect was reported and radiographically confirmed by fluoroscopy and postmortem myelography in only one case report, which concluded that the hydromyelia was probably the major factor permitting the ascent of air [9].

This case report describes a newborn infant with an open myelomeningocele presenting with unexpected somnolence, irregular respiration, and extensive motor deficits. Magnetic resonance imaging revealed a pneumorachis and pneumocephalus, as well as the cerebral malformations associated with myelomeningocele. The radiographic presentation of intraspinal air on magnetic resonance imaging as a complication of an open myelomeningocele was not described previously.

Section snippets

Case Report

A boy, born during week 37 of an uncomplicated pregnancy with maternal use of folic acid, presented at our hospital. No prenatal ultrasound had been performed. An emergency cesarean section was performed because of fetal distress during attempts to rotate the child out of his breech position. The APGAR score was 8 and 10, respectively, after 1 and 5 minutes. Upon physical examination, his birth weight was 2335 g, and an unexpected open lumbosacral myelomeningocele was evident, with leakage of

Discussion

We describe a newborn infant with an open lumbosacral myelomeningocele with severe paraparesis and unexpected dysfunction of the brainstem with somnolence, irregular respiration, dysphagia, and motor deficits of the upper extremities. Magnetic resonance imaging revealed the expected spinal and cerebral malformations, in addition to unexpected pneumorachis and pneumocephalus. We will discuss the clinical signs and underlying pathophysiologic mechanisms.

The brainstem dysfunction was caused by an

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