The male child was born in an external hospital in the 23rd week of pregnancy (weight at birth 565 g, length 32 cm, head circumference 21 cm) and suffered from PHH after prolonged prenatal cardiorespiratory resuscitation. A Rickham-reservoir had initially been implanted and was regularly punctured every 2–3 days; a VPS was implanted at the age of 6 months. The shunt had to be revised due to infection at the age of 7 months, was reimplanted at 8 months and again explanted due to infection at 9 months. Contamination with Klebsiella pneumonia (3MDRGN) was treated with intravenous (i.v.) application of Fosfomycin and Meropenem. MR-imaging showed growing ventricular spaces and multiple septation as well as unspecific sedimentation in the posterior part of the ventricle system. The child was thereafter transferred to our university hospital for treatment of multiloculated hydrocephalus and suspected chronic ventricular infection.
Additionally, the ileum had to be partially resected at an age of 1 month due to a volvulus. The stoma was replaced 4 weeks later. Laparotomy and revision had to be performed at an age of 6 months and resulted in a chronic hernia.
Clinical presentation: Stable cardiorespiratoric state, awake, positive sundown phenomenon. No faucial reflex. Does not reach, no visual fixation. Basal uncoordinated symmetric movement of all 4 limbs. Babinski sign bilaterally positive. Plagiocephalic configuration, wounds closed and dry. Fontanel minimally distended. Rickham-reservoir frontal left, no signs of local infection.
Course of treament: Contaminated foreign material was removed, followed by explorative NEL and implantation of an antibacterial EVD in a first step. The procedure revealed multiple septations and cysts in the enlarged ventricular system with optically distinct compartments without CSF communication. We opened as many cysts and septs as possible by coagulation and resection with endoscopic scissors. Furthermore, extensive lavage of the ventricular system was performed. CSF samples were obtained and showed no contamination. Due to earlier detection of Klebsiella pneumoniae (3MDRGN) we continued antibiotic therapy with Vancomycin and Meropenem i.v. for 21 days. Follow-up CSF samples remained sterile.
An individual trial of intraventricular application with Actilyse (rtPA) over 5 days was initiated 3 times per day with 0.5 mg in 3 ml NaCl. Corresponding CSF parameter follow-up (especially decrease of CSF protein concentration) and volumetric analysis are presented in Fig. 1. Ultrasound and MRI imaging 8 days after surgery disclosed multiple remaining septations and cysts (Fig. 2). MRI-follow up at day 15 after surgery showed a decrease in ventricular size and precipitation of protein while external CSF space was increasing (Fig. 3). There were clear signs of ventriculitis. We continued antibiotic treatment and removed the external ventricular drainage. Ventricle size and precipitation were monitored via ultrasound. Ultrasound at day 28 and day 36 after surgery showed constant ventricular size and decrease in external CSF space (Fig. 3A, B).
During treatment course, the child experienced seizures of increasing frequency that led to feeding insufficiency and were initially treated with phenobarbital and midazolam. Due to insufficient results, both drugs were tapered and new treatment with vigabatrin initiated. With the seizure frequency significantly lowered, the child was again orally feedable. Despite persistent percentile-running head perimeter and unchanged ventricular size, the child was discharged into neurological rehabilitation after 35 days of in-hospital treatment. It was readmitted two months later due to severe fever, sepsis, dehydration and ongoing convulsive seizures. Additional to anticonvulsive and antipyretic therapy, we performed MRI-scans (Fig. 3C-F). Due to signs of ventriculitis, hypoxic and post-ischemic lesions in mesencephalon, pons and peduncula, best medical treatment, in consensus with the parents, was initiated.
The patient was transferred to our facility on 62nd postnatal day. The child had been prematurely born (30 + 3 week of pregnancy, birth weight 1520 g) and suffered from IVH at day 3 after birth and PHH. An EVD was established and, after failed EVD weaning, a VPS had been implanted 6 weeks after birth.
Clinical presentation: Awake, stable cardiorespiratoric state, hypotrophic (48.5 cm length (P0.1 / SDS − 3.31, 2.77 kg weight (P0.1 / SDS − 3.07). Screaming loudly, moves all 4 limbs. Fontanel at level, soft. VPS palpable, no signs of infection. No meningism, no deficit of cranial nerves, pupils isocor, pupillary light reflex intact.
Course of treatment: Initial ultrasound (Fig. 5) showed post hemorrhagic hydrocephalus under drainage with external ventricular catheter (not shown). There were no signs of acute hydrocephalus. Ultrasound was regularly undertaken with stable ventricle size. The child was readmitted 3 months after birth with septic constellation, high fever and progressing apathy.
The VPS valve was initially punctured to complete infective diagnostics. CSF was dense and highly suspicious for ventriculitis, microbiological analysis later verified S. agalactiae contamination. We indicated immediate VPS explantation and EVD implantation with an antibacterial catheter. Gentamicin and vancomycin were regularly installed via EVD. CSF was rinsed with NaCl via the EVD. Protein levels persisted and made VPS implantation impossible. We therefore installed Alteplase (0.5 mg, once daily for 7 days) via EVD. After CSF was repeatedly sterile, EVD was explanted. Although there was no neurological deterioration, CSF leakage through the wound and increase in ventricle size in an MRI (Fig. 6A, B) at 3 months after birth made revision necessary. CSF still showed protein precipitation, which made repeated drainage via EVD necessary. CSF protein levels remained elevated throughout the course of treatment, so we rinsed the EVD with NaCl repeatedly (Fig. 4). There were no signs of repeated infection. Due the repeated NaCl instillation, protein levels finally came down and made VPS implantation possible. The child was submitted in stable neurological and overall condition. Ultrasound showed decreased ventricle size and regular position of the catheter (Fig. 6C, D).