Toneal Shunt Malfunction in Infant Hydrocephalus

Home , Hydrocephalus, Shunt (medical), Ventriculitis

Kosin Medical Journal 2021;36:44-50. https://doi.org/10.7180/kmj.2021.36.1.44 &D VH 5HSRUWV Idiopathic Intracranial Hypertension following Ventriculoperi - toneal Shunt Malfunction in Infant Hydrocephalus

Sangjun Ahn 1, Jae Meen Lee 1, Young Mi Kim 2, Hyeshin Jeon 3, Chang Hwa Choi 1

1Department of Neurosurgery, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea 2Department of Pediatrics, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea 3Department of Ophthalmology, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea

Idiopathic intracranial hypertension (IIH) is a syndrome defined by elevated intracranial pressure without any abnormal findings. In the present study, we report a rare case of IIH in a patient after ventriculoperitoneal shunt (VPS) due to infant hydrocephalus. A 13-year-old girl with a history of VPS due to infant hydrocephalus was admitted to emergency room with the complaint of severe headache and visual disturbance. Brain computed tomography showed normal findings. However, based on the measurement by lumbar puncture, her cerebrospinal fluid (CSF) pressure was observed to be very high. The shunt function test revealed a VPS malfunction. Thus, we conducted VPS revision in this patient. All symptoms improved immediately after the revision. Thus, it is proposed that IIH should be considered for patients with visual dis - turbance and severe headache after VPS due to infant hydrocephalus without ventriculomegaly.

Key Words : Idiopathic intracranial hypertension, Infant hydrocephalus, Ventriculoperitoneal shunt

Idiopathic intracranial hypertension (IIH) is a (approximately three times the normal) 3 that syndrome defined by elevated intracranial pres - can mainly occur due to hemorrhage and infec - sure without any clinical, laboratory, or radi - tion. VPS can be performed as a treatment. 4,5 ographic evidence of responsible infection, However, cases of IIH without progression to vascular abnormality, space-occupying lesion, hydrocephalus are limited even if VPS is per - or hydrocephalus. 1 Treatment options for IIH formed due to infant hydrocephalus. Herein, include physical modalities (weight loss), ac - we report a rare case of IIH in a patient after etazolamide, furosemide, and lumbar puncture. VPS malfunction due to infant hydrocephalus. Ventriculoperitoneal shunt (VPS) is considered as safe and very effective in patients with un - controlled IIH. 2 Infant hydrocephalus shows CASE ventricle size enlargement due to elevated re - sistance to cerebrospinal fluid (CSF) resorption A 13-year-old girl was admitted to our emer -

Corresponding Author : Jae Meen Lee, Department of Neurosurgery, Pusan National University Hospital, 179, Receive d: Se p. 25, 2020 Gudeok-ro, Seo-gu, Busan 49241, Korea Revise d: Ma y. 12, 2021 Tel: +82-51-240-7257 Fax: +82-51-244-0282 E-mail: [email protected] Accepted : Jun. 01, 2021

44 IIH after VPS malfunction gency department with the complaint of severe aging showed flattening of the posterior sclera headache, seizure, and visual disturbance for the and intraocular protrusion of both the optic nerve past month. There were no other neurological ab - heads, depicting papilledema (Fig. 1B). MR normalities, such as facial palsy. She had a his - venography showed multifocal severe stenosis of tory of VPS due to infant hydrocephalus at 6 bilateral transverse and sigmoid sinuses (Fig. 1C). months after birth. One year before the emer - Her visual acuity was 20/125 in the right eye gency department visit, the patient underwent and 20/200 in the left eye. Her light reflex was VPS (Strata) revision due to distal catheter short - normal. Her bilateral optic discs were elevated ening in another hospital. bilaterally, with retinal veins dilated and tortu - Brain-computer tomography (CT) imaging ous. Multiple retinal hemorrhages were observed finding was not consistent with hydrocephalus (Fig. 1D). Her body mass index was normal (Fig. 1A). Brain magnetic resonance (MR) im - (20.9 kg/m 2).

Fig. 1. (A) Preoperative brain computer tomography (B) Brain magnetic resonance T2 image showing intraocular protrusion of both the optic nerve heads (yellow arrows) (C) Brain magnetic resonance venography showing multifocal stenosis of bilateral transverse and sigmoid sinuses (yellow arrows) (D) Papilledema with marked circumferential disc elevation, and obstruction of vessels on the disc with multiple retinal hemor - rhages in both the eyes were observed in fundus photograph.

45 Kosin Medical Journal 2021;36:44-50.

We taped a ventricular catheter reservoir to rule the liver. Abdomen CT imaging surprisingly re - out shunt infection. Her CSF study was normal vealed that the tip of the distal catheter had found (protein concentration: 51.3 g/l; glucose: 105 g/l; its way through the perihepatic space to the and 3 white cells/mm 3). The shunt pressure was retroperitoneum (Fig. 2A). VPS function test changed from 1.0 to 0.5 (3 cm H 2O). However, (Tc-99m DTPA) suspected malfunction of both her symptoms showed no improvement. distal and proximal VPS catheters (Fig. 2B). We Lumbar drainage was performed to decrease in - suspected that these IIH symptoms were caused tracranial pressure (ICP). The opening pressure by the malfunction of VPS. Thus, we decided to was 39.5 cm H 2O. perform a VPS revision. After CSF drainage, improvement in headache During surgery, the proximal catheter was ob - and visual disturbance was observed. An ab - served to function well. Thus, it was maintained domen X-ray was performed to check the loca - and we changed the distal catheter since valve tion of the distal catheter. It was observed to be (ProGAV). After reaffirming its function, the dis - shifted to a little upper side and located around tal catheter was inserted into the peritoneal cav -

Fig. 2. (A) Abdomen computer tomography axial and coronal images showing catheter tip (yellow circles) stuck in perihepatic space (B) Tc-99m diethylene-triamine-pentaacetate (DTPA) image showing the malfunction of both proximal and distal catheters.

46 IIH after VPS malfunction ity. Her headache improved immediately on a IIH is a syndrome related to elevated intracra - postoperative day one. The overall ventricular nial pressure of unknown cause. It is sometimes size was unchanged (Fig. 3A). Her visual acuity a cerebral emergency. It occurs in all age groups, was also improved to 20/32 in the right eye and especially in children and young obese women. 20/80 in the left eye. She was discharged on post - Patients with IIH may show several symptoms in - operative day 10. Her papilledema and retinal cluding visual disturbance without underlying ex - vascular abnormalities also subsided. Her optic pansive intracranial lesion. 2 A definite diagnosis disc was pale on postoperative one month (Fig. of IIH requires these symptoms and signs listed 3B). She is currently being followed up in the in the modified Dandy criteria (Table 1). 1 The outpatient department without any symptoms. present case met the above criteria. The patient underwent VPS. However, she showed severe headache and visual disturbance without any size DISCUSSION change in the ventricle.

Fig. 3. (A) There is no signiﬁcant change in postoperative brain computer tomography (B) Optic disc swelling sub - sided and retinal hemorrhages were disappeared postoperatively.

Table 1. Modiﬁed Dandy criteria of idiopathic intracranial hypertension

Variable

- Symptoms of increased intracranial pressure (ICP) Headache, nausea, vomiting, transient visual obscuration, or papilledema - No localizing ﬁndings in neurological examination Except for false localizing signs such as abducens or facial palsies - Normal CT/ MRI ﬁnding without evidence of dural sinus thrombosis

- ICP of > 250mm H 2O with normal cerebrospinal ﬂuid cytological and chemical ﬁndings - No other cause of increased ICP

47 Kosin Medical Journal 2021;36:44-50.

There are various causes of IIH, including e n- tages for treating IIH, including its safety and ac - docrine diseases, infections, anemia, head trauma, curacy. 7 In addition, patients without ventricu - and drugs. The choice of treatment must be pre - lomegaly may develop a slit-ventricle syndrome ceded by the correction of these underlying after VPS due to over drainage of CSF. 8 To pre - causes. Treatment options of IIH include physical vent this, we changed the valve to proGAV, a modalities (weight loss), medical treatment for gravity-assisted differential pressure valve, during lowering intracranial pressure, and surgical treat - the revision. 9 ment such as CSF shunting. Acetazolamide and It is well established that shunt obstructio ns furosemide (a carbonic anhydrase inhibitor drug) constitute the majority of shunt system failures, are mainly used as the first-line medical treatment especially for those with distal catheter obstruc - to reduce the rate of CSF production. 2 Topiramate tion. 10,11 In the present case, we found that the tip (an antiepileptic drug), zonisamide (another drug of the distal catheter was trapped in the perihep - with secondary carbonic anhydrase activity), and atic space and blocked at CT. Even if the location methylprednisolone (an anti-inflammatory drug) of the distal catheter on the abdomen X-ray is as - can also be used. Surgical treatment is considered sumed to be appropriate, abdomen CT examina - when medical management fails. Surgical meth - tion is necessary in such a case. ods include optic nerve sheath fenestration, ve - We evaluated the function of the shunt with Tc- nous stenting, and CSF shunting. The purpose of 99m DTPA shuntography that is known to be very all these procedures is to prevent progression of useful in patients with suspected catheter mal - vision loss by reducing ICP. CSF shunting is con - function. Tc-99m DTPA shuntography could guide sidered safe and very effective in patients with surgical revision with specificity and a positive uncontrolled IIH. 2 Because of the small size of predictive value of 100% in surgical findings. 12 the ventricle, numerous difficulties arise in tar - In the present case, 99m DTPA shuntography geting it for the placement of the proximal showed obstruction of both the catheters. How - catheter. Lumbo-peritoneal shunt (LPS) is pre - ever, only the distal catheter was blocked during ferred over VPS. Although LPS is effective in im - VPS, which could probably be due to a high CSF proving the symptoms, it tends to have a high risk pressure of 35 cm H 2O or more, which prevented of re-operation due to shunt malfunction. Chumas the radionuclide from entering the ventricle. et al. 6 reported that 9 out of 10 patients with LPS Thus, it is necessary to check the function of both showed improvement of symptoms. However, 7 the catheters without changing the catheters dur - patients needed revision due to shunt migration, ing the revision. construction, and catheter fracture. Currently, due Infant hydrocephalus can be caused by multiple to the development of cranial electromagnetic etiologies such as congenital anomalies and ge - navigation system, the number of cases undergo - netic disorders. The most common cause is intra - ing VPS is increasing as VPS has many advan - ventricular hemorrhage. 4 In 20% of pediatric

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