Arachnoid Cyst Spontaneous Rupture, Rupture, Cyst Spontaneous Arachnoid IB, Et Al

Recebido: 14deFevereiro de2013- Aceite: 15deJunho2013| 1. ServiçodeNeurologia.CentroHospitalar e UniversitáriodeCoimbra.Portugal. Acta MedPort2014Jan-Feb;27(1):137-141 Inês BrásMARQUES 5. 4. 3. 2. 1. REFERÊNCIAS prosse por optar casal o Se graves. maternas plicações com de e fetal morte de riscos os enfrentando gestação, a prosseguir deixar ou patologia, sem vivo, feto um de dez gravi a interromper entre optar de tem casal o viável, feto um e completa hidatiforme mola uma há que em gemelar (fotografia cortesiade ArturCostaeSilva) Figura 4 Rotura EspontâneadeQuisto Aracnóide Arachnoid CystSpontaneousRupture ABSTRACT Keywords: a sudden and severe headache, especially if starting after minor trauma or a Valsalva manoeuvre, always requires medical evaluation. requires surgical treatment. Patients with arachnoid cysts must be informed on their small vulnerability to cyst rupture and be aware that frequently and life-threatening be may that hypertension intracranial sustained causes space subdural the in cerebro accumulation fluid spinal of Accumulation cysts. arachnoid of complication rare very a is rupture Spontaneous needed. ultimately was treatment cal surgi and enlarged progressively hygroma subdural the treatment, conservative with rupture. occurred improvement clinical Although hypertension. We report an unusual case of progressive refractory headache in an adult patient due to an arachnoid cyst spontaneous intracranial of symptoms with presenting frequently complications, or enlargement after symptomatic become may They adolescents. or children in incidentally diagnosed and asymptomatic usually collections, fluid cerebrospinal congenital benign are cysts Arachnoid Arch GynecolObstet.2008;278:377-82. fetus. co-existent surviving a and mole hydatiform complete a with cy B, Rabinovich Piura R, MaorE,MazorM. A, Hershkovitz Twinpregnan with normalviablechild.BMJ.1957;1:1103. associated mole hydatiform Well-formed pregnancy: Twin JH. Taylor Hum Reprod.2000;15:608-11. Japan. in study collaborative national a fetus: live twin a with existent T,Hando S, TomodaSekiya WakeN, H, Matsui Y. co mole Hydatiform healthy co-twin.Lancet.2002;359:2165-6. and mole hydatiform complete with pregnancies twin of Outcome al. et D, Short RJ, Francis RA, Fisher FJ, Paradinas M, Foskett NJ, Sebire Oncol. Lancet diseases. 2003;4:670-8. throphoblastic gestational of aetiology and Epidemiology C. Vecchia La J, Smith J, Ferlay S, Franceschi A, Altieri

- Produto de concepção: feto, placenta e mola hidatiforme Arachnoid Cysts/complications;Rupture,Spontaneous. 1 Marques IB,etal. Arachnoid cystspontaneousrupture, , J.VIEIRA BARBOSA ica da Ordem dos Médicos www m o c . a s e u g u t r o p a c i d e m a t c a w. w w s o c i d é M s o d m e d r O a d a c fi í t n e i C a t s i v e R 1 Copyright ©Ordemdos Médicos2014 - - - - - 137 6. realização desteartigo. FONTES DEFINANCIAMENTO resse narealizaçãodopresentetrabalho. CONFLITOS DEINTERESSE sistente. per gestacional trofoblástica doença desenvolvem casos dos 50% a 16 entre que e 50%, a inferiores são saudável e vivo recém-nascido um de parto um ter de hipóteses as que informado ser deve casal O pulmonar. metastização pesquisar para trimestral torácica radiografia de realização autores Alguns pré-termo. parto e -eclâmpsia pré de sugestivos sintomas e sinais vaginal, hemorragia tiroideia, função da vigilância a com rastreadas, ser devem maternas complicações as e diferenciado, perinatal centro um para referenciada ser deve grávida a gravidez, a guir 9. 8. 7. Acta MedPort2014Jan-Feb;27(1):137-141 C, et al. P,Marcorelles Y,Laurent Ferec MP, MJ, JJ, Audrezet Bris Chabaud Le 2005;25:772-6. pregnancy. molar co-existing a by complicated nancies preg twin of management and diagnosis Prenatal E. Jauniaux L, Wee X- and Y- cromossomalcontent.HumPathol.1995;26:1175-80. placental evaluate to hybridization situ in fluorescent of Use fetus: ing coexist and mole hydatiform complete with DH. pregnancies field Twin Choi-Hong SR, Genest DR, Crum CP, Berkowitz E, Goldstein DP, Scho Obstet GynecolReprodBiol.2001;94:301-3. form mole with a coexisting live fetus in dichorionic twin gestation. Eur J MC. Sharma S, Goel TakkarS, D, Kochar Deka D, N, Malhotra with alivetwinfetus.EurJObstetGynecolReprodBiol2005;118:21-7. Não existiram fontes externas de financiamento para a para financiamento de externas fontes existiram Não Os autores declaram a inexistência de conflitos de inte 2,3 Diagnosis and outcome of complete hydatiform mole coexisting 9 sugerem a sugerem Prenat Diagn. Prenat Hydati

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------Brain CT after symptoms resolution revealing slight 0). CSF cytochemical examination was normal. 0). CSF cytochemical examination was - 2 In this article we report an unusual case of ara-

1-11 As the headache continued worsening despite sympto Treatment with oral acetazolamide, 250 mg three A A 29-year-old caucasian male patient, with unremar- Acta Med Port 2014 Jan-Feb;27(1):137-141 Port 2014 Acta Med Figure 2 hygroma enlargement Neurosurgery team found no indication for surgical treat ment and symptomatic treatment with tramadol, metamizol and metoclopramide was prescribed. matic treatment, the patient returned four days later. The se with constant, pressure-like, holocranial, was headache by exacerbated intensity, increasing progressively and vere photopho progressive by accompanied and position supine bia and visual blurring. Neurological examination revealed bilateral papilledema with normal visual visual fields acuity, There were no other cranial nerve and pupillary responses. functions abnormalities, focal neurological findings or me puncture Lumbar apiretic. was patient the and signs ningeal with manometry revealed an increased opening pressure (45 cmH times a day, induced with progressive total symptom clinical resolution after improvement eight days. brain However, CT scan revealed slight right enlargement of revealed the image subdural brain later, weeks Two 2). (Fig. hygroma subdural collection disappearance, but simultaneous left ous cyst rupture has been reported rarely, mainly in chil dren. rupture in an adult patient. chnoid cyst spontaneous CASE REPORT kable past medical history, presented to room with severe headache and the vomiting with sudden start emergency three days before after lifting heavy objects. Brain CT scan cyst arachnoid fossa cranial middle left a revealed MRI and and bilateral frontotemporal subdural hygroma (Fig. 1).

138 ------2,9 In collec ACs not so potentially life- Signs and symp congenital 6 1,2,5-8 Traumatic or spontane Head trauma can origin benign 1 2,6,7 1-3,5,7 R e v i s ta C i e n tífi c a d a Or d e m d o s M é d i c o s w w w.a c ta m e d i c a p o r tu g u e s a .c o m 1,7,9 Marques IB, et al. Arachnoid cyst spontaneous rupture, rupture, cyst spontaneous Arachnoid IB, et al. Marques They are usually diagnosed during Quisto Aracnóide/complicações; Ruptura Espontânea. Quisto 1,4,5 with anomalous splitting of the normally fused finding. - Initial brain CT revealing left middle cranial fossa

1-3 Although infrequent, more serious and Clinical manifestations depend on various factors, in In most cases, ACs remain small and asymptomatic. ACs represent about 1% of all intracranial space- Arachnoid cysts (ACs) are formados da sua pequena vulnerabilidade para rotura do quisto e ter conhecimento que uma cefaleia súbita e violenta, especialmente violenta, e súbita cefaleia uma que conhecimento ter e quisto do rotura para vulnerabilidade pequena sua da formados sempre avaliação médica. necessita trauma ou manobra de Valsalva, se surgir após pequeno Palavras-chave: Os quistos aracnóides são colecções de líquido céfalo-raquidiano congénitas e benignas, geralmente assintomáticas e diagnosticadas diagnosticadas e assintomáticas geralmente benignas, e congénitas céfalo-raquidiano líquido de colecções são aracnóides quistos Os incidentalmente em crianças ou adolescentes. Podem tornar-se sintomáticos após crescimento ou complicações, apresentando-se frequentemente com sintomas de hipertensão intracraniana. Reportamos um caso invulgar de cefaleia progressiva e refratária num doente adulto devido a rotura espontânea de quisto Apesar aracnóide. de melhoria clinica com tratamento verificou-se conservador, aumento progressivo de higroma subdural com necessidade de tratamento rotura A cirúrgico. espontânea é uma complicação muito colecção de líquidocéfalo-raquidiano no espaço subdural causa hipertensão intracraniana mantida po A rara dos quistos aracnóides. in ser devem aracnóides quistos com Doentes cirúrgico. tratamento frequentemente necessitando vida, da ameaçadora tencialmente arachnoid cyst and bilateral frontotemporal subdural hygroma Figure 1 rupture of blood vessels around the cyst wall or of the un intracystic producing cavity, cyst in veins bridging supported and/or subdural hemorrhage. threatening complications may occur, making benign as frequently believed. most common presentation, but they may also present with present also may they presentation, but common most deficits. motor focal or hypertension intracranial seizures, children they may cause macrocephaly and delayed psy chomotor development. or CSF circulation disturbance. cluding age and cyst location and size. Headache is the and located in the middle cranial fossa. toms usually follow cyst enlargement with compression or and/ effect mass intracranial structures, adjacent of irritation fossa, with predilection for the left side. they become manifest, especially when large Occasionally, imaging childhood and adolescence, with male nance. gender Their predomi most common location is the middle cranial by secretion of fluid by the arachnoid cells into the resulting the into cells arachnoid the by fluid of secretion by cleft. -occupying lesions, being a common incidental neuro- believed to result from a dysgenesis in embryological de velopment followed membrane arachnoid the of layers outer and inner tions of cerebrospinal fluid (CSF) surrounded by histologi cally normal layers of the arachnoid membrane. They are INTRODUCTION RESUMO

CASO CLÍNICO revealing enlargementoftheleftsubduralhygroma 3 Figure the subdural space. In the second mechanism, spontane mechanism, second the In space. subdural the into disruption later progressively with pressure cyst, internal the cyst increases into entering CSF allowing only space. subarachnoid with nication A mechanism, flap-valve commu a and wall cyst subarachnoid the in tear a create rupture. outcome wasreportedinthetotalityofcases. radiological and clinical Good treatment. conservative to performed in all cases, except in one patient who responded collections in similarity to our patient. Surgical treatment was subdural bilateral with patient a cases, the of one but all in cyst the to ipsilateral was hygroma Subdural patient. one in was present hemiparesis) (right deficit neurological cal fo a and papilledema bilateral was examination in finding common most The symptom. constant a being headache with present were manifestations hypertension intracranial and fossa cranial middle the in located were cysts rupture, ous AC rupture. In the totality of the cases with spontaneous with our patient being the first adult reported with spontane occurred in children or adolescents (age range: 5-16 years), Tablein literature 1. in reported rupture ACs refractory headache. progressive with adults in suspicion clinical of especially in adulthood, probably explains the frequent lack adolescents. and children in majority vast the literature, the in reported rupture taneous spon or traumatic either of cases fifty than less with cysts, DISCUSSION outcome (Fig.5)andwithoutclinicalcomplications. imagiologic satisfactory with performed was fenestration cyst and hygroma subdural of evacuation with craniotomy tures effect withmass and larger contralateral displacement of midline hygroma struc left subdural the of enlargement fied tomatic andclinicallystable. asymp was patient the as waiting, watchful with approach conservative a maintain to decided team Neurosurgery 3). subdural hygroma enlargement with minor midline shift (Fig. Two mechanisms have been proposed to explain ACs explain to proposed been have mechanisms Two We present a summary of the patients with spontaneous arachnoid of complication uncommon very a is Rupture A control CT scan scheduled for two months later Fg 4. ept te bec o smtm, urgent symptoms, of absence the Despite 4). (Fig.

10 - Brain CT after three weeks of acetazolamide treatment acetazolamide of weeks three after CT Brain - The first one would be a minor head injury able to Marques IB,etal. Arachnoid cystspontaneousrupture, ica da Ordem dos Médicos www m o c . a s e u g u t r o p a c i d e m a t c a w. w w s o c i d é M s o d m e d r O a d a c fi í t n e i C a t s i v e R 2-17 h rrt o ti entity, this of rarity The 6,7,9-16 All cases All identi ------139

resolution andreduction ofarachnoidcystvolume 5 Figure ma enlargementwithmasseffect andmidlineshift Figure 4 ettos abi nt fetn te yt dimension. cyst the affecting not albeit festations, mani hypertension intracranial and hygroma of resolution vative treatment with acetazolamide has been reported with subsequent and hygroma subdural intracranial hypertension. in results subdural space the in accumulation CSF manoeuvre. a Valsalva during increase pressure intracranial transient a sudden follows space subdural the into cyst the of rupture ous on a valvular system and risk of obstruction or infection. or obstruction of risk and system valvular a on dependence as inconveniences has but brain, surrounding the of decompression sudden from complications potential used. tially related to cyst location and size rather than the method essen are complications surgical as preference, and ence experi surgeon on mainly depends choice procedure gical effective. and safe being endoscopically,both way. path flow CSF normal the and cavity cyst the between tion adeq an establishes which fenestration cyst is approach surgical preferred currently The pression. mass with effect andaftercystcomplications. cysts cysts, enlarging cysts, symptomatic for indicated is treatment surgical while cysts, asymptomatic versial. was ultimatelynecessary. treatment surgical and enlarged progressively hygroma the acetazolamide, with resolved symptoms patient our though Acta MedPort2014Jan-Feb;27(1):137-141 In clinically stable patients, a good response to conser- to response good a patients, stable clinically In The main objective of surgical treatment is cyst decom cyst is treatment surgical of objective main The contro remains ACs for approach treatment best The 1,2,4 1 Cystoperitoneal shunt is an alternative that reduces that alternative an is shunt Cystoperitoneal 4,5,8

This procedure can be performed by craniotomy or craniotomy by performed be can procedure This

- Brain CT after two months, revealing left subdural hygro Ps-prtv ban T cn ih udrl hygroma subdural with scan CT brain Post-operative - n xetn attd i avsd o sal and small for advised is attitude expectant An 1-17

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CASO CLÍNICO Outcome hygroma resolution; Cyst reduction Symptoms and hygroma resolution NR Symptoms and hygroma resolution Symptoms resolution; Hygroma and cyst reduction Symptoms resolution; Hygroma and cyst reduction Symptoms and hygroma resolution Symptoms and NR Symptoms and hygroma resolution; Cyst reduction Symptoms resolution; Hygroma and cyst reduction NR Treatment evacuation and endoscopic cyst fenestration Subdural- peritoneal shunt NR Conservative (Acetazolamide) Craniotomy and cyst fenestration Subdural- peritoneal shunt Subdural- peritoneal shunt Hygroma Craniotomy and cyst fenestration Subdural- peritoneal shunt Craniotomy and cyst fenestration NR Subdural Collections Ipsilateral SD hygroma Ipsilateral SD hygroma Ipsilateral SD haematoma Bilateral SD hygroma Ipsilateral SD hygroma Ipsilateral SD hygroma Ipsilateral SD hygroma Ipsilateral SD hygroma Ipsilateral SD hygroma Ipsilateral SD hygroma Ipsilateral SD hygroma Acta Med Port 2014 Jan-Feb;27(1):137-141 Port 2014 Acta Med Examination Neurological Neurological Normal Normal “signs and symptoms of increased IP” Bilateral papilledema Bilateral papilledema Normal Normal Fundoscopy with “capillary blush and blurred disk margins” NR Bilateral papilledema, mild right hemiparesis “signs and symptoms of increased IP” 140 – intracranial pressure nausea nausea, nausea, vomiting vomiting vomiting vomiting vomiting vomiting and right vomiting, Clinical anorexia, “signs and “signs and Headache, Headache, Headache, Headache, Headache, Headache, drowsiness nausea and nausea and nausea and hemiparesis and diplopia photophobia symptoms of symptoms of increased IP” increased IP” blurred vision, epigastric pain Headache and Headache and Headache and Presentation and diaphoresis Cyst Location Right MCF Left MCF Left MCF Left MCF Right MCF Left MCF Left MCF Left MCF Left MCF Left MCF Left MCF 5 9 9 11 15 16 12 14 12 16 10 Age (years) R e v i s ta C i e n tífi c a d a Or d e m d o s M é d i c o s w w w.a c ta m e d i c a p o r tu g u e s a .c o m Marques IB, et al. Arachnoid cyst spontaneous rupture, rupture, cyst spontaneous Arachnoid IB, et al. Marques F F F M M M M M M M NR Gender 12 12 7 16 10 – Summary of patients with spontaneous arachnoid cyst rupture arachnoid spontaneous with of patients – Summary 6 15 13 14 9 11 Reference Gil-Gouveia et al 2010 Slaviero et al 2008 2004 Poirrier et al Cakir et al 2003 Cayli et al 2000 Choong et al 1998 Sener 1997 Sener 1997 Ergun et al 1997 FC, Giannotta 1997 SL Albuquerque Cullis P, Gilroy Cullis P, J, 1983 M – male; F – female; MCF –Middle cranial fossa; NR – not reported; SD – subdural; IP Table 1 Table

CASO CLÍNICO Acta MedPort2014Jan-Feb;27(1):141-145 SÁ Recebido: 18deFevereiro de2013- Aceite: 08deSetembro2013 | 2. FaculdadedeMedicina.Universidade Lisboa. Portugal. 1. DepartamentodePediatria.HospitalSanta Maria.CentroHospitalarLisboaNorte.Lisboa.Portugal. RESUMO Pedro MARQUESDA COSTA 8. 7. 6. 5. 4. 3. 2. 1. REFERENCES manoeuvres or trauma after especially headache, violent is also important to explain these patients that a sudden and cranial It pressure. intracranial for in increase extreme with or trauma risk increased with activities with events rare those of relationship the and hemorrhage or to rupture vulnerability cyst small their on headache. informed of be must cause Patients uncommon extremely an and ACs tive measureoffurthercystcomplications. ation, surgical cyst evacuation, withoutcystintervention tions described good clinical results after subdural effusions A recent study about ACs complicated with subdural collec Hospital ProtocolforSnakebiteVictims Management Abordagem deMordeduraOfídio Protocolo de Actuação Hospitalarna Palavras-chave: actualizada dasequipasmédicas envolvidas. e multidisciplinar intervenção uma visa que hospitalar actuação de protocolo de proposta uma expomos e tomada clínica actuação a discutimos actual, literatura na Baseados pediátrica. idade em venenoso ofídio de mordedura de clínicos casos dois apresentamos o constitui protocolada actuação A fatais. complicações de risco gravidade e maior apresenta pediátrica idade em ocorre Quando Portugal. em rara situação uma é venenoso ofídio de mordedura A 1,2 hygroma. JClinNeurosci.2004;11:317-8. subdural as presenting cyst VS. Arachnoid Mehta VaishyaS, R, Gupta gery. 1997;41:951-6. Neurosur reports. case hypertension: intracranial and hygroma dural sub producing rupture cyst Arachnoid SL. Giannotta FC, Albuquerque dural collections. Arq Neuropsiquiatr. sub with complicated cysts arachnoid fossa cranial Middle VanzinJR. RD, Annes LS, Martins ND, Júnior Azambuja L, Frighetto F, Slaviero with 60consecutivecases. effusions—experience subdural by accompanied fossa cranial middle Sprung C, Armbruster B, Koeppen D, Cabraja M. 13. port of three cases and literature review. re hygroma: subdural with rupture cyst Arachnoid R. Martínez-Rumbo Gelabert-González M, Fernández-Villa J, Cutrín-Prieto J, Garcìa J Radiol.2006;79:79-82. of rupture complicating lection Forbes Clair J. St W, Thorne C, Offiah of tearthecyst–casereport.NeurolMedChir. 2010;50:512-4. endoscopic and effusion subdural with presenting cyst fossa Hamada H, Hayashi N, Umemura K, Kurosaki K, Endo S. Middle cranial chnoid cyst.CirCir. 2010;78:551-6. Vega-Sosa A, de Obieta-Cruz E, Hernández-Rojas MA. Intracranial ara rcni cs rpue s vr rr cmlcto of complication rare very a is rupture cyst Arachnoid to additionally patient, our In , MariadoCéuMACHADO Marques daCostaP, etal. Protocoloactuaçãohospitalarnaabordagemdemordeduraofídio, Antivenenos; MordedurasdeSerpentes;Portugal. fenestration was performed as a preven

Acta Neurochir. 2011;153:75-84. ica da Ordem dos Médicos www m o c . a s e u g u t r o p a c i d e m a t c a w. w w s o c i d é M s o d m e d r O a d a c fi í t n e i C a t s i v e R a middle cranial fossa arachnoid cyst. arachnoid fossa cranial middle a 1,2 , RodrigoSOUSA 1,2 2008;66:913-5. subdural collection evacu collection subdural Non-haemorrhagic subdural col subdural Non-haemorrhagic Childs Nerv Syst. 2002;18:609- .

12. 11. 10. 9. FUNDING SOURCES (Poster). 11,to 8 September (EFNS), ties Sweden Stockholm, 2012; Socie Neurological Federation European the of Congress 16th Communication); (Oral Portugal Coimbra, 2011, 19, the Sociedade Portuguesa de Cefaleias, November 18 and of Meeting meetings: Autumn scientific following the at red CONFLICTS OFINTEREST evaluation. medical requires always pressure, intracranial increasing 17. 16. 15. 14. 13. gold standard of care gold standard taneous rupture into the subdural space. Comput Med Imaging Graph. Imaging Med Comput space. subdural the into rupture taneous spon and post-traumatic with associated cysts Arachnoid RN. Sener dural space. Acta Neurochir. 1997;139:692-4. sub the into rupture Spontaneous cyst: arachnoid of complication sual F,Ergüngör E, H, Ökten Beșkonakli Anasiz R, AI, Tașkin Ergun Y. Unu Neurol NeurosurgPsychiatry. P,Cullis G arachnoid cyst:acetazolamidetherapy. BrainDev. 1998;20:319-22. Choong CT, Lee SH. Subdural hygroma in association with middle fossa aphy incomplicatedarachnoidcyst.ChildsNervSyst.2005;21:1061-4. P,Longatti 9. 2010;50:314- Headache. aura. with migraine new-onset simulating cyst Gil-Gouveia R, Miguens J, Coiteiro D. Rupture of middle fossa arachnoid Eur JPaediatrNeurol.2004;8:247-51 arachnoid cyst rupture in a previously asymptomatic child: a case report. Poirrier AL, Ngosso-Tetanye I, Mouchamps M, Misson JP. Spontaneous view. Neurocirurgía.2003;14:72-5. re literature and report case hygroma: subdural with rupture cyst noid Arach G. Karaarslan B, Peksoylu OC, Sayin Kayhankuzeyli; E, Cakir subdural space. BrJNeurosurg2000;14:568-70. the into rupture spontaneous with cyst Arachnoid SR. Cayli 1997;21:341-4. None stated. delive previously was case clinical This stated. None ilroy J. ilroy Marton E, Billeci D. Acetazolamide and corticosteroid ther corticosteroid and Acetazolamide D. Billeci E, Marton 1 , Sara AZEVEDO Arachnoid cyst with rupture into the subdural space. subdural the into rupture with cyst Arachnoid Acta MedPort2014Jan-Feb;27(1):141-145 o cnrs nencoas Nse artigo Neste internacionais. centros nos 1983;46:454-6. 1,2 , Gabriela ARAÚJO E

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CASO CLÍNICO Inês Brás MARQUES, J. VIEIRA BARBOSA Arachnoid Cyst Spontaneous Rupture Acta Med Port 2014:27:137-141

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