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Anna-Felicitas Gebert, Matthias Schulz, Karin Schwarz and Ulrich-Wilhelm Thomale

OBJECTIVE

The use of adjustable differential pressure valves with gravity-assisted units in shunt therapy of children with hydrocephalus was reported to be feasible and promising as a way to avoid chronic overdrainage. In this single-center study, the authors' experiences in infants, who have higher rates of shunt complications, are presented.

METHODS

All data were collected from a cohort of infants (93 patients [37 girls and 56 boys], less than 1 year of age [mean age 4.1 ± 3.1 months]) who received their first adjustable pressure hydrocephalus shunt as either a primary or secondary implant between May 2007 and April 2012. Rates of valve and shunt failure were recorded for a total of 85 months until the end of the observation period in May 2014.

RESULTS

During a follow-up of 54.2 ± 15.9 months (range 26–85 months), the Kaplan-Meier rate of shunt survival was 69.2% at 1 year and 34.1% at 85 months; the Kaplan-Meier rate of valve survival was 77.8% at 1 year and 56% at 85 months. Survival rates of the shunt were significantly inferior if the patients had previous shunt surgery. During follow-up, 44 valves were exchanged in cases of infection (n = 19), occlusion (n = 14), dysfunction of the adjustment unit (n = 10), or to change the gravitational unit (n = 1).

CONCLUSIONS

Although a higher shunt complication rate is observed in infant populations compared with older children, reasonable survival rates demonstrate the feasibility of using this sophisticated valve technology. The gravitational unit of this valve is well tolerated and its adjustability offers the flexible application of opening pressure in an unpredictable cohort of patients. This may adequately address overdrainage-related complications from early in treatment.

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Matthias Schulz, Christoph Bührer, Anja Pohl-Schickinger, Hannes Haberl and Ulrich-Wilhelm Thomale

Object

Neonatal intraventricular hemorrhage (IVH) may evolve into posthemorrhagic hydrocephalus and cause neurodevelopmental impairment. In this study, an endoscopic surgical approach directed toward the removal of intraventricular hematoma was evaluated for its safety and efficacy.

Methods

Between August 2010 and December 2012 (29 months), 19 neonates with posthemorrhagic hydrocephalus underwent neuroendoscopic lavage for removal of intraventricular blood remnants. During a similar length of time (29 months) from March 2008 to July 2010, 10 neonates were treated conventionally, initially using temporary CSF diversion via lumbar punctures, a ventricular access device, or an external ventricular drain. Complications and shunt dependency rates were evaluated retrospectively.

Results

The patient groups did not differ regarding gestational age and birth weight. In the endoscopy group, no relevant procedure-related complications were observed. After the endoscopic lavage, 11 (58%) of 19 patients required a later shunt insertion, as compared with 100% of infants treated conventionally (p < 0.05). Endoscopic lavage was associated with fewer numbers of overall necessary procedures (median 2 vs 3.5 per patient, respectively; p = 0.08), significantly fewer infections (2 vs 5 patients, respectively; p < 0.05), or supratentorial multiloculated hydrocephalus (0 vs 4 patients, respectively; p < 0.01).

Conclusions

Within the presented setup the authors could demonstrate the feasibility and safety of neuroendoscopic lavage for the treatment of posthemorrhagic hydrocephalus in neonates with IVH. The nominally improved results warrant further verification in a multicenter, prospective study.

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Matthias Schulz, Georg Bohner, Hannah Knaus, Hannes Haberl and Ulrich-Wilhelm Thomale

Object

Multiloculated hydrocephalus remains a challenging condition to treat in the pediatric hydrocephalic population. In a retrospective study, the authors reviewed their experience with navigated endoscopy to treat multiloculated hydrocephalus in children.

Methods

Between April 2004 and September 2008, navigated endoscopic procedures were performed in 16 children with multiloculated hydrocephalus (median age 8 months, mean age 16.1 ± 23.3 months). In all patients preoperative MR imaging was used for planning entry sites and trajectories of the endoscopic approach for cyst perforation and catheter positioning. Intraoperatively, a rigid endoscope was tracked by the navigation system. For all children the total number of operative procedures, navigated endoscopic procedures, implanted ventricular catheters, and drained compartments were recorded. In addition, postoperative complications and radiological follow-up data were analyzed.

Results

In 16 children, a total of 91 procedures were performed to treat multiloculated hydrocephalus, including 29 navigated endoscopic surgeries. Finally, 21 navigated procedures involved 1 ventricular catheter and 8 involved 2 catheters for CSF diversion via the shunt. The average number of drained compartments in a shunt was 3.6 ± 1.7 (range 2–9 compartments). In 9 patients (56%) a navigated endoscopic procedure constituted the last procedure within the follow-up period. One additional surgery was necessary in 3 patients (19%) after navigated endoscopy, and in 4 patients (25%) 2 further procedures were necessary after navigated endoscopy. Serial follow-up MR imaging demonstrated evidence of sufficient CSF diversion in all patients.

Conclusions

Navigated endoscopic surgery is a safe and effective treatment option for multiloculated hydrocephalus. The combination of the endoscopic approach and neuronavigation further refines preoperative planning and intraoperative orientation. The aim of treatment is to drain as many compartments as possible and as soon as possible, thereby establishing sufficient CSF drainage with few ventricular catheters in single shunt systems. Close clinical and radiological follow-up is mandatory because multiple revisions are likely.

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Martin Misch, Ulrich-Wilhelm Thomale, Rolf Keitzer and Peter Vajkoczy

Tuberculosis-related vasculopathy and resulting ischemic events are a common finding after tuberculous meningitis (TBM) in children. The authors report on a case of recurring ischemic episodes that were refractory to repetitive endovascular procedures (transluminal angioplasty, stent insertion, in-stent vessel dilation). After failure of endovascular treatment, extracranial-intracranial (EC-IC) bypass surgery was performed to prevent further ischemia.

This 5-year-old girl presented with a series of ischemic events in the left middle cerebral artery (MCA) territory 12 months after her presentation with TBM. Angiography studies showed a 50% lumen reduction of the left MCA. After failure of antithrombocyte aggregation and repeated endovascular procedures to prevent new ischemia, EC-IC bypass surgery was performed. The left superficial temporal artery–MCA bypass was found to be capable of preventing new ischemic events in the 21-month follow-up period, despite a near-total MCA occlusion on follow-up conventional and MR angiography studies. Bypass surgery seems to be a reliable tool to augment cerebral blood flow in tuberculosis-related stenoocclusive changes of cerebral vasculature after the failure of endovascular procedures.

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Florian Stockhammer, Ulrich-Wilhelm Thomale, Michail Plotkin, Christian Hartmann and Andreas von Deimling

Object

Oligodendroglial tumors harboring combined 1p and 19q loss (1p/19q LOH) are characterized by a favorable prognosis and response to chemotherapy and radiotherapy, but detection of 1p/19q LOH relies on postoperative procedures. The authors investigated the potential of fluorine-18–labeled fluorodeoxyglucose (FDG) uptake in positron emission tomography (PET) to predict 1p/19q LOH preoperatively in tumors whose appearance on initial magnetic resonance images was consistent with that of low-grade glioma.

Methods

The study population comprised 25 patients who had undergone preoperative FDG-PET followed by tumor resection. Neuronavigation ensured a precise match of FDG uptake wi th the site of biopsy. All tumor specimens were graded according to the World Health Organization (WHO) classification system. Microsatellite analysis was used to identify 1p/19q LOH.

In this series, 16 of 25 gliomas corresponded to WHO Grade II. In eight of these 16, 1p/19q LOH was detected. Raised glucose utilization within the tumor was seen in the six of eight WHO Grade II gliomas with 1p/19q LOH and in none of the WHO Grade II gliomas without this genetic alteration (p = 0.003).

Conclusions

These findings demonstrate the potential of FDG-PET to predict 1p/19q LOH in WHO Grade II gliomas.

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Ulrich-Wilhelm Thomale and Matthias Schulz

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Stefan-Nikolaus Kroppenstedt, Michael Kern, Ulrich-Wilhelm Thomale, Gerd-Helge Schneider, Wolfgang Reinhardt Lanksch and Andreas Wilhelm Unterberg

Object. Although it is generally acknowledged that a sufficient cerebral perfusion pressure (CPP) is necessary for treatment of severe head injury, the optimum CPP is still a subject of debate. The purpose of this study was to investigate the effect of various levels of blood pressure and, thereby, CPP on posttraumatic contusion volume.

Methods. The left hemispheres of 60 rats were subjected to controlled cortical impact injury (CCII). In one group of animals the mean arterial blood pressure (MABP) was lowered for 30 minutes to 80, 70, 60, 50, or 40 mm Hg 4 hours after contusion by using hypobaric hypotension. In another group of animals the MABP was elevated for 3 hours to 120 or 140 mm Hg 4 hours after contusion by administering dopamine. The MABP was not changed in respective control groups. Intracranial pressure (ICP) was monitored with an ICP microsensor. The rats were killed 28 hours after trauma occurred and contusion volume was assessed using hematoxylin and eosin—stained coronal slices. No significant change in contusion volume was caused by a decrease in MABP from 94 to 80 mm Hg (ICP 12 ± 1 mm Hg), but a reduction of MABP to 70 mm Hg (ICP 9 ± 1 mm Hg) significantly increased the contusion volume (p < 0.05). A further reduction of MABP led to an even more enlarged contusion volume. Although an elevation of MABP to 120 mm Hg (ICP 16 ± 2 mm Hg) did not significantly affect contusion volume, there was a significant increase in the contusion volume at 140 mm Hg MABP (p < 0.05; ICP 18 ± 1 mm Hg).

Conclusions. Under these experimental conditions, CPP should be kept within 70 to 105 mm Hg to minimize posttraumatic contusion volume. A CPP of 60 mm Hg and lower as well as a CPP of 120 mm Hg and higher should be considered detrimental.

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Sebastian A. Ahmadi, Frank K. H. van Landeghem, Cristiane Blechschmidt, Kerstin Lieber, Ernst-Johannes Haberl and Ulrich-Wilhelm Thomale

Intracranial osteolipomas and chondromas are rare benign tumors. Forty-five chondromas, mostly supratentorial, have been reported in the literature since 1981, with origins most commonly in the sellar regions. Twenty-one osteolipomas have been described to date, usually located near the tuber cinereum or the corpus callosum. The authors present a case of an osteochondrolipoma arising from the tentorium diagnosed in a pediatric patient at the age of 9 years. The case and treatment are discussed, and a review of the literature is provided.

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Patrick Cramer, Ulrich-Wilhelm Thomale, Ali Fuat Okuducu, Arne J. Lemke, Florian Stockhammer and Christian Woiciechowsky

✓ The authors report the case of a 23-year-old man who presented with a C1–3 spinal mass. Following intraspinal decompression the tumor was histologically classified as an atypical meningioma (World Health Organization grade II). Two further surgical interventions resulted in almost total removal of the meningioma. In addition, radiotherapy was performed. During the 1.5-year follow-up period the diagnostic examinations identified a local tumor recurrence, an intraspinal C-6 metastasis, and a segmental instability with anterior C2–3 slippage and C3–4 kyphosis. The tumor was resected and occipitocervical stabilization was performed. Histological examination showed no change in malignancy. Despite additional hydroxyurea-based chemotherapy, the patient presented 4 months later with a hemiparesis and a massive recurrence of the tumor mass involving the posterior fossa and the upper thoracic spine. Because there were no further therapeutical options, the patient died. The authors discuss more aggressive therapeutic options in addition to surgery in patients with metastatic atypical meningiomas. The results in the reported case indicate that meningiomas associated with cerebrospinal fluid metastasis may represent a higher grade of malignancy.

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Vivek A. Mehta, Chetan Bettegowda, Sebastian A. Ahmadi, Petra Berenberg, Ulrich-Wilhelm Thomale, Ernst-Johannes Haberl, George I. Jallo and Edward S. Ahn

Object

Symptom response to spinal cord untethering, and the impact of duraplasty and scoliosis on retethering, are poorly understood in tethering after myelomeningocele (MMC) repair. In this retrospective study, the authors examined the outcomes of children who developed first-time spinal cord tethering following MMC repair. The response of symptoms to untethering and the role of duraplasty and scoliosis in retethering are explored.

Methods

The authors performed a review of 54 children with first-time symptomatic spinal cord tethering following MMC repair to determine the impact of untethering on symptoms, the impact of dural repair type on retethering, and the role of scoliosis on the prevalence and time to retethering.

Results

The average patient age was 10.3 ± 4.9 years, and 44% were males. The most common presenting symptoms of tethered cord syndrome were urinary (87%), motor (80%), gait (78%), and sensory (61%) dysfunction. The average postoperative time to symptom improvement was 2.02 months for sensory symptoms, 3.21 months for pain, 3.50 months for urinary symptoms, and 4.48 months for motor symptoms, with sensory improvement occurring significantly earlier than motor improvement (p = 0.02). At last follow-up (an average of 47 months), motor symptoms were improved in 26%, maintained in 62%, and worsened in 11%; for sensory symptoms, these rates were 26%, 71%, and 3%, respectively; for pain, 28%, 65%, and 7%, respectively; and for urinary symptoms, 17%, 76%, and 7%, respectively. There was no difference in symptom response with type of dural repair (primary closure vs duraplasty). Symptomatic retethering occurred in 17 (31%) of 54 patients, but duration of symptoms, age at surgery, and type of dural repair were not associated with retethering. Scoliosis was not associated with an increased prevalence of retethering, but was associated with significantly earlier retethering (32.5 vs 61.1 months; p = 0.042) in patients who underwent additional untethering operations.

Conclusions

Symptomatic retethering is a common event after MMC repair. In the authors' experience, sensory improvements occur sooner than motor improvements following initial untethering. Symptom response rates were not altered by type of dural closure. Scoliosis was associated with significantly earlier retethering and should be kept in mind when caring for individuals who have had previous MMC repair.