**3. Results**

Five pediatric patients aged 0–19 years presented with clinical signs and symptoms of increased ICP.

They had small ventricles, no venous outflow obstruction, high opening pressure on LP and normal CSF composition.

They were all males, four were in the first decade (age: 1,3,4,5 and 18 years). All had bilateral papilledema, headache and visual disturbances in spite of medical treatment (Acetazolamide, corticiosteroids, furosemide). They all had normal weight for age. The results are summarized in **Table 1**.


#### **Table 1.**

*Clinical details.*

#### **3.1 Case 1**

*The first patient* was a 18 year-old boy. In spite of medical treatment and LP every 4 weeks, the headache was troublesome and his vision was partly lost (0,5) during six months in spite of the repeated taps. A lumboperitoneal shunt (mini Holter high valve) was therefore implanted in January 1984. The symptoms resolved and his vision improved but not completely during the next year. After many years, in 2003, he once more experienced headache and blurred vision. There was no papilledema, but high opening pressure on LP. The LPshunt was found blocked and after revision he once more was symptom free.

In 2013 another episode of clinical shunt failure took place, and the symptoms again responded favorably after implantation of a VP shunt. Today, with 36 years follow-up, he is in full-time work as a diary worker.

## **3.2 Case 2**

*The second patient* was a 4 year-old boy presenting with VIth nerve palsy, bilateral papilledema, headache and visual affection in spite of treatment with furosemide for some weeks. After implantation of a cisterno-atrial shunt (mini-Holter high valve), the symptoms resolved (slight binasal visual defect). After many good clinical years until about 2012, he thereafter during the next years experienced increasing overdrainage-symptoms (headache, dizziness, sometimes also subjective affection of vision and hearing) when rapidly changing to the upright position. The symptoms were reversed effectively in the recumbent position. In 2016 the shunt was removed and ICP was monitored in 4 days along with clinical observation. He clearly improved, and his clinical symptoms related to change in body position disappeared. He now appears to be shunt independent for more than three years.

### **3.3 Case 3**

*The third patient* was a 3 year-old boy admitted with a short history of lost vision for 36 hours, reduced pupil reactivity, ataxia and poor general condition. Fundoscopy demonstrated choked discs and cerebral MRI was normal including unobstructed venous outflow. Lumbar puncture revealed normal CSF composition and increased CSF pressure, but the ICP level was difficult to measure due to lack of cooperation. A lumbar infusion test during general anesthesia demonstrated increased CSF opening pressure as well as slightly increased outflow resistance.

Due to the dramatic clinical symptoms with complete visual loss, corticosteroid treatment was implemented and an acute shunt procedure was performed during the same general anesthesia. A proximal catheter was introduced into cisterna Magna and connected to a low pressure Holter valve with diversion to the peritoneal cavity.

His vision gradually reappeared within days, and after one week there was normal vision and pupillary reactivity to light. Some ataxia and clumsy motor function persisted for weeks, but after 6 months his clinical condition was quite normal. During the first two years of treatment he experienced a few episodes with headache, ataxia and diplopia (VIth nerve paresis) which resolved spontaneously within a couple of days or after pumping on the Holter valve. At the age of 5 years (1991) he demonstrated episodes of overdrainage in the upright position, which subsided after implementation of an ASD (anti-syphon-device) distal to the valve.

Thereafter his clinical condition was uneventful for many years. During his university studies (in 2006), he became acutely ill with signs of increased ICP (headache and vomiting). There was no choked discs, but lumbar puncture revealed markedly increase CSF pressure level (50 cm H2O) and no signs of infection. Once more MRI was normal (**Figure 1**). After a shunt revision (LP-shunt), his clinical condition normalized within a few days. In 2009, he experienced shunt failure once more, again followed by rapidly improvement after shunt revision.

*Will CSF Diversion in Patients with Idiopathic Intracranial Hypertension (IIH) Lead… DOI: http://dx.doi.org/10.5772/intechopen.96291*

#### **Figure 1.**

*(Case 3) demonstrates normal intracranial structures during the episode of shunt failure in 2006 (lower row) including unobstructed venous outflow (upper and middle row).*

Thereafter he has been working full time, and has been clinically quite well for another 11 years.

This case with a very rewarding clinical result indicate that this 35 year-old man in excellent condition most likely is permanently shunt dependent.

#### **3.4 Case 4**

*The fourth patient* was a 4 year-old boy presented with headache, bilateral papilledema, ataxia, and unstable level of consciousness and episodes of blurred vision. The ventricles were small, venous outflow was unaffected and a lumbar infusion test during general anesthesia (normocapnia) revealed ICP of 24 mm Hg and increased resistance to outflow of CSF. A medium Holter shunt was implanted from cisterna Magna to the peritoneal cavity and the symptoms normalized. Three years later he experienced acute shunt failure(disconnection) with headache, papilledema, vomiting and a lumbar pressure of 45 mm Hg. After revision, the situation once more normalized. Ten years later, in 2004, he experienced another episode of shunt failure with headache, vomiting, diplopia and bilateral papilledema. Lumbar pressure was highly increased. A VPshunt (Codman Medos) was now implanted. During the next two years the situation was more unstable, with episodes of clinical symptoms related to over- as well as underdrainage. In 2006, he experienced episodes of visual loss, and ICP monitoring revealed pathological ICP. A lumboperitoneal shunt (OSV II) was therefore implanted and the VPshunt removed. During the subsequent 12 years he has been clinically well and fulfilled a master degree at the university.
