CASE 13871 Published on 10.08.2016

Rare case of fatal ventriculitis in a term neonate following myelomeningocele repair


Paediatric radiology

Case Type

Clinical Cases


Aleksandra N. Belova, Katy L. Ruch, Geoffrey McWilliams, Thomas Sanchez

UC Davis medical center,
University of California;
Suite 3100 4860 Y St
95835 Sacramento, USA;

2 weeks, male

Area of Interest Neuroradiology brain, Paediatric, Head and neck ; Imaging Technique CT, Ultrasound
Clinical History
2-week-old infant with repaired sacral myelomeningocele presented to the Emergency Department in septic shock. Head ultrasound showed new hydrocephalus. A ventricular drain was placed which drained purulent secretions. CSF and blood cultures were positive for E.coli. Patient progressed to brain death clinically, supported by CT angiography.
Imaging Findings
Initial head ultrasound prior to myelomeningocele repair was normal (Fig. 1). Follow-up ultrasound on day 17 of life when the patient presented with septic shock at the ED showed a non-communicating hydrocephalus with dilated lateral ventricles filled with debris (Fig. 2 and 3). Contrast CT of the head was performed to rule out intracranial complication of ventriculitis and showed normal enhancement of the intracranial vessels (Fig. 4). There was significant deterioration of the patient neurologically and a subsequent CT angiographic evaluation of the head was performed on day 19 of life (Fig. 5). There was diffuse hypoattenuation of the entire supratentorial brain secondary to global oedema and hypoxia with relative sparing of the structures of the posterior fossa constituting the “white cerebellum sign” also known as "reversal sign". In addition, there was no significant contrast enhancement of circle of Willis and the major intracranial cerebral vessels.
Myelomeningocele is the most frequent form of neural tube defect encountered in newborns with a reported worldwide incidence ranging from 0.17 to 6.39 in every 1000 live births [1-2]. This is characterized by protrusion of a CSF filled sac containing meninges and neural elements through an osseous defect in the spinal column. Hydrocephalus is present in 65-85% of myelomeningocele cases, majority of which will require surgical management most often via ventriculo-peritoneal (VP) shunting [2]. Most CNS infection in myelomeningocele patients are acquired after VP shunt placement. Review of the literature revealed a paucity of data regarding cases of infectious meningitis/ventriculitis following myelomeningocele repair in the absence of a concomitant VP shunt. Retrospective studies have not reported any incidence of CNS infection for those who did not undergo VP shunting [1-2]. The current case report is particularly unique for several reasons. First the patient did not have hydrocephalus at the time of myelomeningocele repair and hence did not undergo VP shunt placement. Second, he did well post-operatively without any signs of surgical site infection and presented 2 weeks later with new onset hydrocephalus and severe gram negative CNS infection.

In the first few weeks of life, ultrasound is usually the first imaging performed to evaluate for signs of infections such as abnormal extra-axial fluid collection or intraventricular debris. Ventriculitis from the underlying inflammatory reaction is seen as thickening of the subependymal lining of the ventricles. Meningitis, cerebritis, subdural empyema and abscess are however better evaluated with CT or MRI. Severe and progressive inflammation can lead to vasculitis and global infarction.
In conclusion, ultrasound can initially be used to evaluate clinical suspicion of intracranial infections in the perinatal period. However, MRI or CT will be necessary to evaluate complications as well as for prognostication.
Differential Diagnosis List
Ventriculitis and septic shock progressing to end organ failure and brain death
Intraventricular haemorrhage
Final Diagnosis
Ventriculitis and septic shock progressing to end organ failure and brain death
Case information
DOI: 10.1594/EURORAD/CASE.13871
ISSN: 1563-4086