Case Report Author's Personal Copy Chiari Malformation and Hydrocephalus Masking Neurocysticercosis Sharad Rajpal1, Colson Tomberlin2, Andrew Bauer1, Robert C. Forsythe3, Sigita Burneikiene1,4 Key words - BACKGROUND: Various diagnostic characteristics associated with neuro- - Chiari malformation cysticercosis have been well studied; however, their potential to be implicated - Hydrocephalus - Neurocysticercosis in other differential diagnoses has not been well demonstrated. - Subarachnoid cysts - CASE DESCRIPTION: We report the case of a 55-year-old Hispanic man who Abbreviations and Acronyms underwent a Chiari decompression surgery, which was complicated with hy- CP: Cerebellopontine drocephalus. Despite a ventriculoperitoneal shunt placement, he continued to MRI: Magnetic resonance imaging have headaches and was soon found to have several skull base subarachnoid VP: Ventriculoperitoneal lesions, which were later diagnosed as the sequelae of an active neuro- From the 1Boulder Neurosurgical Associates, 2University of cysticercosis infection. Colorado Boulder, 3Bouder Valley Pathology, and 4Justin Parker Neurological Institute, Boulder, Colorado, USA - CONCLUSION: This case report highlights the importance of overlapping To whom correspondence should be addressed: symptoms between diseases in a short temporal context. Sharad Rajpal, M.D. [E-mail: [email protected]] Citation: World Neurosurg. (2018) 114:68-71. https://doi.org/10.1016/j.wneu.2018.03.010 duraplasty. He had an uneventful hospital After approximately 8 months, the pa- Journal homepage: www.WORLDNEUROSURGERY.org course and was discharged home after 3 tient was seen in the emergency depart- days. The patient did well for several ment again for a fever, headache, balance Available online: www.sciencedirect.com months but then presented with recurrent problems, and myalgias. MRI of the brain 1878-8750/$ - see front matter ª 2018 Elsevier Inc. All rights reserved. headaches about 5 months after his showed a large arachnoid cyst in the left surgery. Brain imaging demonstrated a cerebellopontine (CP) angle measuring   BACKGROUND large 7.4 9.6 cm pseudomeningocele 3.1 1.7 cm and another arachnoid cyst in within the surgical site and extending into the right ambient cistern measuring 2.8  Neurocysticercosis is the leading neuro- the posterior tissues of the neck (Figure 2A 1.7 cm (Figure 5A and B). The larger cyst parasitic infection in the United States, and B). in the CP angle was causing compression affecting approximately 2000 individuals 1 The patient was taken to the operating on the brainstem. The VP shunt valve every year. The disease is caused by the was readjusted in the clinic over 2 ingestion of eggs from the tapeworm Taenia room for exploration and revision of the suboccipital pseudomeningocele and place- separate visits in an attempt to increase solium that are excreted in the stool of fl ment of a lumbar drain. At the time of sur- the ow rate with close monitoring of people with taeniasis. Neurocysticercosis the skull-based cysts for any size changes. symptoms range from headaches to gery, a small pinhole was visualized at the edge of the duraplasty graft and revised with a Five months later, the patient continued seizures but can also present with to complain of headaches, dizziness, loss symptoms of hydrocephalus, which can 6-0 nonabsorbable polypropylene suture, fat be caused by chronic arachnoiditis, graft, and dural sealant. He was hospitalized meningeal fibrosis, or intraventricular with continuous lumbar drainage and dis- cysts.2 Here we report a unique presentation charged home on the eighth day reporting an of neurocysticercosis following surgical improvement in his headaches. Approxi- intervention for a Chiari I malformation. mately 4 weeks later, the patient returned to the emergency department complaining of worsening bitemporal headaches, nausea, CASE DESCRIPTION and vomiting. A magnetic resonance imaging A 55-year-old Hispanic male presented to the (MRI) scan indicated that he had developed clinic complaining of balance issues, hydrocephalus with reaccumulation of fluid diplopia, visual field deficits, and ongoing in the subarachnoid space consistent with a headaches that had persisted for about a pseudomeningocele measuring 5.6  4.8 cm year. Radiographic imaging demonstrated (Figure 3A and B). Due to the recurring nature thepresenceofanArnoldChiariImalfor- of hydrocephalus, the patient was taken to mation (Figure 1). He was taken to the the operating room and underwent a right operating room and underwent posterior frontal ventriculoperitoneal (VP) shunt Figure 1. Radiographic imaging fossa decompression via a suboccipital placement (Figure 4). He did well and was demonstrated the presence of an Arnold Chiari I malformation. craniectomy with C1 laminectomy and discharged home the next day. 68 www.SCIENCEDIRECT.com WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2018.03.010 Author's Personal Copy CASE REPORT SHARAD RAJPAL ET AL. CHIARI MALFORMATION AND NEUROCYSTICERCOSIS Figure 4. Due to the recurring nature of Figure 2. (A and B) Brain imaging demonstrated a large 7.4  9.6 cm pseudomeningocele within the hydrocephalus, the patient was taken to the surgical site and extending into the posterior tissues of the neck. operating room and underwent a right frontal ventriculoperitoneal shunt placement. of lateral vision, and left sided face and the cyst fluid and sent to Pathology. tongue numbness. The cyst in the left CP Figure 5C His postoperative MRI, angle had increased to 3.8 x 2.6 cm demonstrating an improvement in his but no scolex were observed. Pathologic fi although the cyst in the right ambient compressive cyst. and histologic evidence was suf cient to fi cistern decreased in size to 1.8 x 1.4 cm. Histologic analysis (Figure 6AeC)ofthe con rm the diagnosis of neurocysticercosis. The decision was made to proceed with firm lesion, measuring 1.4  1.1 cm, The patient was seen by an infectious surgical exploration and cyst fenestration showed the classic 3-layer distinctive disease expert and placed on close obser- because they were becoming more symp- wall of pleomorphic neurocysticercosis vation for further possible infection and tomatic and not responding to his VP with microtriches/microvilli, an outer symptoms related to neurocysticercosis. In shunt valve adjustments. The patient eosinophilic cuticle layer, a cellular layer this case, anthelminthic drugs were not subsequently underwent a retrosigmoid with uniform cellular dark nuclei, and an prescribed because it was felt the patient fi craniotomy for fenestration of the left CP inner reticular layer containing loose fibrils. had undergone de nitive surgical treat- angle cyst. During his surgery, a small In addition, intense lymphoplasmacytic ment of his neurocysticercosis. The patient firm lesion was unexpectedly noted within inflammation and dystrophic calcifications recovered without any postoperative com- plications or remaining symptoms other than slight balance impairment. His follow-up imaging at 4 months (Figure 7A and B) has demonstrated complete resolution of the arachnoidal cyst in the left CP angle and near-complete resolu- tion of the right ambient cistern cyst measuring 1.2  0.4 cm. DISCUSSION A host of symptomatic or asymptomatic signs can occur depending on the size, location (parenchymal or extrapar- enchymal), and number of cysts in patients infected with neurocysticercosis. An extraparenchymal racemose form of neu- rocysticercosis is observed to compose up to 54% of cases with cysts located in the cisternal/subarachnoid spaces, spinal cord, Figure 3. (A and B) Magnetic resonance imaging scan indicated that he had developed hydrocephalus or ocular bulb.3 Seizures are the most with reaccumulation of fluid in the subarachnoid space consistent with a pseudomeningocele common clinical presentation of this measuring 5.6  4.8 cm. form, but hydrocephalus may occur due to WORLD NEUROSURGERY 114: 68-71, JUNE 2018 www.WORLDNEUROSURGERY.org 69 Author's Personal Copy CASE REPORT SHARAD RAJPAL ET AL. CHIARI MALFORMATION AND NEUROCYSTICERCOSIS Figure 5. (AeC) Magnetic resonance imaging of the brain measuring 3.1  1.7 cm and another arachnoid cyst in the right showed a large arachnoid cyst in the left cerebellopontine angle ambient cistern measuring 2.8  1.7 cm. mechanical obstruction or inflammatory hydrocephalus, which is also a common to elevated intracranial pressure from an reaction and has been associated with comorbidity of a Chiari malformation and obstructive process secondary to a higher mortality.4 later develop arachnoid cysts. neurocysticercosis infection. However, given Although signs of hydrocephalus can be Our case is unique in that the patient the close temporal proximity of the easily recognized on imaging studies, initially presented with a Chiari malformation Chiari symptoms and the observed cysts, uninflamed extraparenchymal cysticerci without any clinical evidence of a neuro- it is also possible that intracranial may be difficult to identify on computed cysticercosis infection. It was only after hy- hypertension from the cysts caused tomography or MRI scans due to the thin drocephalus was treated with a VP shunt that an acquired Chiari malformation or membrane and the cyst being isodense to he developed enlarging subarachnoidal cysts, worsened the congenital form and it cerebrospinal fluid.5 This diagnostic aspect which were later diagnosed to be infectious in became symptomatic. Future considerations can often be overlooked when diagnosing etiology; this was also