MUKOPOLİSAKKARİDOZ TİP 3 TANISI OLAN HASTADA ANESTEZİ DENEYİMİMİZ: OLGU SUNUMU

Abstract

MUKOPOLİSAKKARİDOZ TİP 3 TANISI OLAN HASTADA ANESTEZİ DENEYİMİMİZ: OLGU SUNUMUDr. Öğr. Üyesi. İsmail SÜMERBezmialem Vakıf Üniversitesi, Tıp FakültesiORCID ID: https://orcid.org/0000-0002-0133-0218drismailsumer@gmail.com, 0533 2201576Dr. Öğr. Üyesi. Asım ESENBezmialem Vakıf Üniversitesi, Tıp FakültesiORCID ID: https://orcid.org/0000-0001-7222-7499esen1122@hotmail.com, 0533 5219455Dr. Öğr. Üyesi. Harun UYSALBezmialem Vakıf Üniversitesi, Tıp FakültesiORCID ID: https://orcid.org/0000-0003-0426-8525drharunuysal@hotmail.com, 0506 3433494ÖzetMukopolisakkaridozlar (MPS), lizozomal enzim eksikliğine bağlı olarak glikozaminoglikan vucutta birikimi ile giden, multisistem tutulum gösteren, kalıtsal, kronik ve ilerleyici bir metabolik depo hastalığıdır. Enzim eksikliğine bağlı olarak biriken maddeler hücrelerin normal işlevlerini bozarlar ADDIN EN.CITE <EndNote><Cite><Author>Danos</Author><Year>1995</Year><RecNum>7</RecNum><DisplayText>(1)</DisplayText><record><rec-number>7</rec-number><foreign-keys><key app="EN" db-id="aawxpsrpzwsf9aewp9gv0xw2frwwvr9afwrx" timestamp="1643717026">7</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Danos, O.</author><author>Heard, J. M.</author></authors></contributors><auth-address>Laboratoire Rétrovirus et Transfert Génétique, Institut Pasteur, Paris, France.</auth-address><titles><title>Mucopolysaccharidosis</title><secondary-title>Mol Cell Biol Hum Dis Ser</secondary-title></titles><periodical><full-title>Mol Cell Biol Hum Dis Ser</full-title></periodical><pages>350-67</pages><volume>5</volume><edition>1995/01/01</edition><keywords><keyword>Animals</keyword><keyword>Bone Marrow Transplantation</keyword><keyword>Central Nervous System/metabolism</keyword><keyword>Clinical Trials as Topic</keyword><keyword>Disease Models, Animal</keyword><keyword>Genetic Therapy</keyword><keyword>Humans</keyword><keyword>Mucopolysaccharidoses/genetics/physiopathology/*therapy</keyword></keywords><dates><year>1995</year></dates><isbn>1470-0573 (Print)
1470-0573</isbn><accession-num>9532574</accession-num><urls></urls><electronic-resource-num>10.1007/978-94-011-0547-7_17</electronic-resource-num><remote-database-provider>NLM</remote-database-provider><language>eng</language></record></Cite></EndNote>(1). Mukopolisakkaritler bağ doku komponenti oldukları için biriktikleri dokularda farklı etkilere sebep olabilirler. Deri, solunum yolları, santral sinir sistemi kalp, karaciğer, dalak, kemik ve ligamanlarda depolanır ve bu dokular etkilenebilir. Tipik klinik belirtiler; kaba yüz görünümü, kulak burun boğaz problemleri, iskelet displazileri, büyüme gelişme geriliği, servikal instabilite, servikal kord kompresyonu, organomegali, görme ve işitme bozuklukları, eklem kontraktürleri, herniler ve kardiyopulmoner problemler olarak sıralanabilir ADDIN EN.CITE <EndNote><Cite><Author>Kliegman</Author><Year>2011</Year><RecNum>8</RecNum><DisplayText>(2)</DisplayText><record><rec-number>8</rec-number><foreign-keys><key app="EN" db-id="aawxpsrpzwsf9aewp9gv0xw2frwwvr9afwrx" timestamp="1643717817">8</key></foreign-keys><ref-type name="Book">6</ref-type><contributors><authors><author>Kliegman, R., </author><author>Stanton, B., </author><author>St. Geme, J. W.,</author><author>Schor, N. F.,</author><author>Behrman, R. E. </author><author> </author></authors></contributors><titles><title><style face="normal" font="default" charset="238" size="100%">Nelson Textbook of Pediatrics</style></title></titles><pages><style face="normal" font="default" charset="238" size="100%">480-510</style></pages><volume><style face="normal" font="default" charset="238" size="100%">19th edition</style></volume><dates><year><style face="normal" font="default" charset="238" size="100%">2011</style></year></dates><pub-location>Phialdelphia</pub-location><publisher>Elsevier</publisher><urls></urls></record></Cite></EndNote>(2).MPS tip III olan hastalarda, heparan sülfat yıkımında yetersizlik mevcuttur. Ortak özellikleri hafif somatik bulgularla birlikte yavaş ilerleyen ağır SSS tutulumudur. Bu hastalar genelde MSS tutulumuna bağlı ilerleyici psikomotor gerilik gösterir. Erken dönemde yüzde kabalaşma görülür, nörolojik belirtiler genellikle 2-4 yaş arasında belirlenir ADDIN EN.CITE <EndNote><Cite><Author>Kliegman</Author><Year>2011</Year><RecNum>8</RecNum><DisplayText>(2)</DisplayText><record><rec-number>8</rec-number><foreign-keys><key app="EN" db-id="aawxpsrpzwsf9aewp9gv0xw2frwwvr9afwrx" timestamp="1643717817">8</key></foreign-keys><ref-type name="Book">6</ref-type><contributors><authors><author>Kliegman, R., </author><author>Stanton, B., </author><author>St. Geme, J. W.,</author><author>Schor, N. F.,</author><author>Behrman, R. E. </author><author> </author></authors></contributors><titles><title><style face="normal" font="default" charset="238" size="100%">Nelson Textbook of Pediatrics</style></title></titles><pages><style face="normal" font="default" charset="238" size="100%">480-510</style></pages><volume><style face="normal" font="default" charset="238" size="100%">19th edition</style></volume><dates><year><style face="normal" font="default" charset="238" size="100%">2011</style></year></dates><pub-location>Phialdelphia</pub-location><publisher>Elsevier</publisher><urls></urls></record></Cite></EndNote>(2).Organ patolojileri nedeniyle sık operasyon gerektiren bu hastaların anestezisi sistemik sorunlar nedeniyle özellik gösterir. Özellikle yüz deformitesi, nazofaringeal mukopolisakkarid birikimi ve eklem-kemik deformitelerine bağlı hava yolu sağlanmasındaki güçlük bu hastalarda sık karşılaşılan bir sorundur ADDIN EN.CITE <EndNote><Cite><Author>Kamin</Author><Year>2008</Year><RecNum>9</RecNum><DisplayText>(3)</DisplayText><record><rec-number>9</rec-number><foreign-keys><key app="EN" db-id="aawxpsrpzwsf9aewp9gv0xw2frwwvr9afwrx" timestamp="1643718047">9</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Kamin, W.</author></authors></contributors><auth-address>Pediatric Pneumology, Allergy, Endoscopy and Cystic Fibrosis Center, Children's Hospital, University of Mainz, Mainz, Germany. kamin@kinder.klinik.uni-mainz.de</auth-address><titles><title>Diagnosis and management of respiratory involvement in Hunter syndrome</title><secondary-title>Acta Paediatr</secondary-title></titles><periodical><full-title>Acta Paediatr</full-title></periodical><pages>57-60</pages><volume>97</volume><number>457</number><edition>2008/05/28</edition><keywords><keyword>Airway Obstruction/etiology</keyword><keyword>Anesthesia, General</keyword><keyword>Continuous Positive Airway Pressure</keyword><keyword>Forced Expiratory Volume</keyword><keyword>Humans</keyword><keyword>Magnetic Resonance Imaging</keyword><keyword>Mucopolysaccharidosis II/*complications/diagnosis/physiopathology/therapy</keyword><keyword>Respiration Disorders/diagnosis/etiology/*therapy</keyword><keyword>Sleep Apnea, Obstructive/etiology/therapy</keyword><keyword>Spirometry</keyword><keyword>Vital Capacity</keyword></keywords><dates><year>2008</year><pub-dates><date>Apr</date></pub-dates></dates><isbn>0803-5253</isbn><accession-num>18339190</accession-num><urls></urls><electronic-resource-num>10.1111/j.1651-2227.2008.00650.x</electronic-resource-num><remote-database-provider>NLM</remote-database-provider><language>eng</language></record></Cite></EndNote>(3). Bu hastaların tipik anestezik problemleri; indüksiyon veya ekstübasyon sonrası görülen havayolu obstrüksiyonları, entübasyon zorlukları ve/ veya başarısızlıkları, acil trakeostomi ihtiyaçları, kardiyovasküler ve servikal vertebra sorunlarını içerir ADDIN EN.CITE ADDIN EN.CITE.DATA 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(4) 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Bu olgu sunumunda, MPS tip 3 tanılı diş cerrahisi geçirecek olan hastanın anestezi yönetiminden bahsedilmiştir.Olgu:Olgumuzda diş tedavisi amacıyla anestezi uygulanan MPS Tip 3 hastası 10 yaşında, 35 kg ağırlığında erkek çocuğu sunulmaktadır. Hastanın fizik muayenesinde büyük kafa ve dil, boyunda hareket kısıtlılığı ve mental retardasyon mevcuttu.(Resim 1) Labaratuvar tetkiklerinde bir sorun yoktu. Anestezi öncesinde çocuk nöroloji, çocuk metabolizma hastalıkları ve çocuk kardiyoloji konsültasyonları istendi. Konvulziyon riski nedeniyle antiepileptiklere devam etmesi, anlantoaksiyal subluksasyon riski nedeniyle entübasyonda dikkatli olunması gerektiği belirtildi. Ayrıca çocuk kardiyoloji tarafından yapılan ekokardiyografide MPS’ye bağlı hafif-orta mitral yetmezlik ve hafif aort yetmezliği tespit edildi. İşlem öncesinde endokardit proflaksisi önerildi.Hastaya işlemden 30 dk önce ampisilin 50 mg/kg uygulandı, başka herhangi bir premedikasyon uygulanmadı, operasyona alınan hasta için zor entübasyon şartları hazırlandı. Rutin monitorizasyonu takiben sol el dorsalinden 24G intraket ile damar yolu açıldı. İndüksiyon 1 mg midozolam, 3 mg/kg propofol ve 1mcg/kg fentanil ile yapıldı. Preoperatif ventilasyon sırasında airway yerleştirildi ve ventilasyonda zorluk yaşanmadı, rocuronyum 0,6 mg/kg uygulandı. Sol burun deliğinden yerleştirilen 5 numara spiralli tüp, video laringoskop ve Magill pensi yardımıyla ilerletilerek hasta nazal olarak entübe edildi.(Resim 2) Genel anestezi idamesi %50-%50 oksijen-medikal hava ve %2 sevofluran ve 0.15-0.3 mg/kg/dk remifentanil infuzyonu ile sağlandı. Postoperatif analjezi için 500 mg IV parasetamol uygulandı. Operasyon sonunda 100 mg sugammadex uygulandı ve ventilasyon problemi olmayan hasta ekstübe edildi. Gözlem amaçlı postoperatif bakım ünitesine alındı. Operasyondan sonra 1 gün yoğun bakımda takip edilen hasta problemsiz bir şekilde taburcu edildi. Tartışma:MPS’li hastaların anestezi açısından preoperatif, perioperatif ve postoperatif dönemde zorlukları ve riskleri vardır. MPS hastalarında preoperatif dönemde iyi bir değerlendirme yapılması gereklidir. Anestezi öncesi değerlendirmede zor entübasyon, zor ventilasyon, kardiyak ve servikal spinal gibi sorunlar değerlendirilir ve gerekli hazırlıkları yapılırsa anestezik riskler oldukça düşer. Hastanın servikal ve trakeolaringeal anatomisi ile altta yatan respiratuar ve kardiyak patofizyolojisi hakkında dikkatli olunmalıdır. Preoperatif olarak başta çocuk nörolojisi ve çocuk kardiyolojisi olmak üzere gerekli bölümlerce konsülte edilmelidir ADDIN EN.CITE ADDIN EN.CITE.DATA 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(4) 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Biz hastamızı preoperatif gerekli bölülerle konsulte ederek gerekli hazırlıkları yaptık ve konsültasyon sonucunda verilen tavsiyelere uyduk. Ayrıca göğüs hastalıkları konsültasyonu istenebilir. MPS hastalarında sıklıkla OSAS görülmektedir ve şiddetli OSAS hikayesi olan hastaların anestezi sırasında havayolu acili yaşama ihtimali yüksektir ADDIN EN.CITE <EndNote><Cite><Author>John</Author><Year>2011</Year><RecNum>11</RecNum><DisplayText>(5)</DisplayText><record><rec-number>11</rec-number><foreign-keys><key app="EN" db-id="aawxpsrpzwsf9aewp9gv0xw2frwwvr9afwrx" timestamp="1643718173">11</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>John, A.</author><author>Fagondes, S.</author><author>Schwartz, I.</author><author>Azevedo, A. C.</author><author>Barrios, P.</author><author>Dalcin, P.</author><author>Menna-Barreto, S.</author><author>Giugliani, R.</author></authors></contributors><auth-address>Sleep Disorders Center, Pulmonary Service, Hospital de Clínicas de Porto Alegre, Brazil. angelajohn@terra.com.br</auth-address><titles><title>Sleep abnormalities in untreated patients with mucopolysaccharidosis type VI</title><secondary-title>Am J Med Genet A</secondary-title></titles><periodical><full-title>Am J Med Genet A</full-title></periodical><pages>1546-51</pages><volume>155a</volume><number>7</number><edition>2011/06/04</edition><keywords><keyword>Adolescent</keyword><keyword>Child</keyword><keyword>Child, Preschool</keyword><keyword>Cross-Sectional Studies</keyword><keyword>Echocardiography, Doppler</keyword><keyword>Female</keyword><keyword>Humans</keyword><keyword>Hypertension, Pulmonary/etiology</keyword><keyword>Male</keyword><keyword>Mucopolysaccharidosis VI/*complications</keyword><keyword>Polysomnography</keyword><keyword>Prevalence</keyword><keyword>Respiratory Tract Diseases/pathology</keyword><keyword>Sleep Apnea, Obstructive/diagnosis/epidemiology/*etiology</keyword></keywords><dates><year>2011</year><pub-dates><date>Jul</date></pub-dates></dates><isbn>1552-4825</isbn><accession-num>21638759</accession-num><urls></urls><electronic-resource-num>10.1002/ajmg.a.33902</electronic-resource-num><remote-database-provider>NLM</remote-database-provider><language>eng</language></record></Cite></EndNote>(5).MPS’de görülen spinal ve kraniofasiyal anomaliler nedeniyle zor maske ventilasyonu ve entübasyon beklenebilir. Bizim hastamızda bulunan büyük kafa ve dil, boyunda hareket kısıtlılığı nedeniyle işlem öncesi zor entübasyon şartları hazırlandı. Entübasyon sırasında video laringoskop kullanıldı. Ayrıca her türlü ihtimale karşılık laringeal maske airway ve fleksible laringoskop kullanılmaya hazır bir şekilde bekletildi.Hastalar cerrahi bitiminden sonraki en erken dönemde ekstübe olmalıdır. Bu durum entübasyondan kaynaklanan havayolunda oluşabilecek ödemi azaltmaya ve erken nörolojik değerlendirmeye izin verir ADDIN EN.CITE ADDIN EN.CITE.DATA 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.Ekstübasyon tam olarak uyanmadan, yeterli solumadan, güçlü bir şekilde öksürmeden ve bilinçli hareket etmeden gerçekleştirilmemelidir ADDIN EN.CITE <EndNote><Cite><Author>Walker</Author><Year>2003</Year><RecNum>12</RecNum><DisplayText>(6)</DisplayText><record><rec-number>12</rec-number><foreign-keys><key app="EN" db-id="aawxpsrpzwsf9aewp9gv0xw2frwwvr9afwrx" timestamp="1643718223">12</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Walker, R. W.</author><author>Colovic, V.</author><author>Robinson, D. N.</author><author>Dearlove, O. R.</author></authors></contributors><auth-address>Department of Anaesthetisia, Royal Manchester Children's Hospital, Pendlebury, Manchester, UK.</auth-address><titles><title>Postobstructive pulmonary oedema during anaesthesia in children with mucopolysaccharidoses</title><secondary-title>Paediatr Anaesth</secondary-title></titles><periodical><full-title>Paediatr Anaesth</full-title></periodical><pages>441-7</pages><volume>13</volume><number>5</number><edition>2003/06/07</edition><keywords><keyword>Adolescent</keyword><keyword>Airway Obstruction/*complications/diagnostic imaging</keyword><keyword>Anesthesia/*adverse effects</keyword><keyword>Bronchoscopy</keyword><keyword>Child</keyword><keyword>Decompression, Surgical</keyword><keyword>Humans</keyword><keyword>Male</keyword><keyword>Mucopolysaccharidoses/*complications/diagnostic imaging</keyword><keyword>Mucopolysaccharidosis I/complications</keyword><keyword>Mucopolysaccharidosis II/complications</keyword><keyword>Mucopolysaccharidosis VI/complications</keyword><keyword>Pulmonary Edema/diagnostic imaging/*etiology</keyword><keyword>Radiography</keyword><keyword>Spinal Fusion</keyword></keywords><dates><year>2003</year><pub-dates><date>Jun</date></pub-dates></dates><isbn>1155-5645 (Print)
1155-5645</isbn><accession-num>12791120</accession-num><urls></urls><electronic-resource-num>10.1046/j.1460-9592.2003.00969.x</electronic-resource-num><remote-database-provider>NLM</remote-database-provider><language>eng</language></record></Cite></EndNote>(6) Hastamızı mümkün olduğu kadar erken ekstübe etmek amacıyla kas gevşeticinin geri çevirilmesi için sugammadex kullanıldı. Hastamız sorunsuz şekilde ekstübe edildi.Postoperatif apne riski nedeniyle postoperatif bakım ünitesinde dikkatli takip gerekmektedir. Postoperatif dönemde, hastada oluşabilecek komplikasyonlar ve solunum problemleri olabileceği için hastanın yoğun bakıma transferi düşünülmelidir. MPSli hastalarda multisistem tutulumu olması nedeniyle farklı sistemler etkilenebilir. Hem havayolu problemleri hem de sistemik diğer problemler nedeniyle tüm riskler göz önünde bulundurulmalı ve yaşanabilecek tüm bu zorluklar için dikkatli hazırlık yapılmalıdır.Anahtar Kelimeler: Mukopolisakkaridoz, MPS, MPS tip 3 anestezi yönetimi, pediatrik anestezi ADDIN EN.REFLIST 1. Danos O, Heard JM. Mucopolysaccharidosis. Mol Cell Biol Hum Dis Ser. 1995;5:350-67.2. Kliegman R, Stanton B, St. Geme JW, Schor NF, Behrman RE, . Nelson Textbook of Pediatrics. Phialdelphia: Elsevier; 2011. 480-510 p.3. Kamin W. Diagnosis and management of respiratory involvement in Hunter syndrome. Acta Paediatr. 2008;97(457):57-60.4. Walker R, Belani KG, Braunlin EA, Bruce IA, Hack H, Harmatz PR, et al. Anaesthesia and airway management in mucopolysaccharidosis. J Inherit Metab Dis. 2013;36(2):211-9.5. John A, Fagondes S, Schwartz I, Azevedo AC, Barrios P, Dalcin P, et al. Sleep abnormalities in untreated patients with mucopolysaccharidosis type VI. Am J Med Genet A. 2011;155a(7):1546-51.6. Walker RW, Colovic V, Robinson DN, Dearlove OR. Postobstructive pulmonary oedema during anaesthesia in children with mucopolysaccharidoses. Paediatr Anaesth. 2003;13(5):441-7.