Dysphagia 증례 아주대학교의과대학소화기내과학교실 이광재
Definition of dysphagia Dysphagia: Abnormality in the passage of liquids or solids from the oral cavity to the stomach Oropharyngeal dysphagia Oropharyngeal tumors, neurogenic and myogenic disorders that affect the function of the oropharynx, larynx, and upper esophageal sphincter Esophageal dysphagia Mechanical causes or motility disorders affecting the body of the esophagus, the lower esophageal sphincter, or cardia
정상인의연하운동 A. 음식물이혀등에위치 B. 혀가전방, 상방으로이동한후, 이어서뒤로이동하면서음식물이구인두 (oropharynx) 로이동 C. 후두가닫히고, 인두가수축 D. 상부식도괄약근이이완하면서음식물이식도로진입 Robin Smithuis
Coughing, choking, or nasal regurgitation
Laryngeal Cancer Corrosive esophagitis Eso. cancer Web
Nonobstructive causes for dysphagia Oropharyngeal dysphagia Myopathic Dermatomyositis, Polymyositis, Myasthenia gravis, Myotonic dystrophy, Oculopharyngeal dystrophy, Neurological Brainstem tumors, Head trauma, Stroke, Cerebral palsy, Multiple sclerosis, Amyotrophic lateral sclerosis Parkinson disease Dementia Esophageal dysphagia Esophageal motility disorders
45/ 남 주소 : 삼킴곤란 (Dysphagia) 기간 : Remote: 1 년, Recent: 7 일 내원 1 년전부터간헐적으로삼킴곤란과소화되지않은음식물의역류가있어오다가내원 1 주전부터물도삼키기힘들정도의삼킴곤란이발생하여내원함 과거력 : 특이소견없음 가족력 : 특이소견없음 사회력 Alcohol (-), Smoking (+) 30PYS 생체징후및신체검사소견 : 특이소견없음
WBC 9.9 10³/ μl H 3.4 9.3 (neutrophil) (79.2%) Hb 14.1 g/dl 10.7 14.6 Hct 40.6 % 32.4 44.0 PLT 323 X10³/ul 143 376 Glucose 101 mg/dl 70 110 BUN 3.1 mg/dl L 5.0 18.0 Creatinine 0.7 mg/dl 0.3 0.7 T.Protein 7.3 g/dl 6.0 8.0 Albumin 4.5 g/dl 3.5 5.3 ALT(GPT) 33 U/L 8 31 AST(GOT) 16 U/L 5 40
다음검사나처치로가장적합한것은? 1) 비디오투시연하검사 2) 식도내압검사 3) 24시간보행성식도임피던스, 산도검사 4) 식도부지확장술 5) 식도조직검사
Esophageal body LES
가장적합한진단명은? 1) 아칼라시아 2) 호두까기식도 3) 미만성식도경련 4) 비효율적식도운동 5) 피부경화증식도
Esophageal mottility disorders esophageal aperistalsis and incomplete LES relaxation LES dysfunction Body dysfunction LES and body dysfunction Achalasia TLESR Hypotensive LES Incomplete LES relaxation Ineffective esophageal motility Nutcracker esophagus Diffuse esophageal spasm Low amplitude Peristaltic failure High amplitude peristaltic contractions Simultaneous contractions 20% of swallows
The selective loss of inhibitory, nitrergic neurons with preservation of cholinergic neurons Autoimmune, neurodegenerative, and viral etiologies in the pathogenesis of achalasia However, the exact cause has yet to be elucidated. 전형적인아칼라시아는식도체부에 10-40 mmhg 의낮은진폭의동시성수축혹은무연동운동을보이고하부식도괄약근의이완부전을보임.
가장적합한치료는? 1) 자가확장형인공관 ( 스텐트 ) 삽입술 2) 부지확장술 3) 풍선확장술 4) 위장운동촉진제 5) 양성자펌프억제제
Treatments of achalasia 신경세포의손상및소실에대한근본적인치료는없다. 하부식도괄약근의압력을낮춰음식물의통과를수월하게해서증상을완화시키는것이목표 약물요법, 풍선확장술, 보톡스주입, 그리고근절개술
약물치료 ( 평활근이완제 ) Nitrates: 두통등의합병증이많아서칼슘통로억제제를더선호 칼슘통로차단제 (nifedipine): 30% 에서두통, 어지러움, 부종등의부작용, 사용할수록더많은용량이필요, 일시적인효과 고령이나동반된질환으로확장술이나근절개술이어려운환자, 보톡스주입치료에효과가없었던환자 보톡스주입 하부식도괄약근에보톡스를주입하면하부식도괄약근압력이저하 단기간의증상호전에는도움을주지만수개월내에환자의반수이상에서증상이재발 풍선확장술에효과가적은환자, 풍선확장술, 근절개술을시행하기어려운환자, 첫보톡스주입에효과가있었던환자에시도
풍선확장술 초기호전율 85% 로높지만 5년후에 40% 의환자들이호전을유지 시간이가면효과가감소 (74% at 6 months, 68% at 12 months and 58% after 36 or more months) 합병증발생율이 3-9%, 식도천공, 식도파열, 위식도역류, 출혈 확장술후하부식도괄약근압이 50% 이상감소한경우에효과좋다.
외과적근절개술 복강경을이용한 Heller 근절개술을권장 초기증상의호전율이 90%, 5년경과후에도 80% 이상의환자들이호전을유지 위식도역류 (36%), 식도점막손상, 식도천공, 수술후누출의합병증 구강내시경을이용한근절개술 Peroral endoscopic myotomy (POEM) 단기간치료성적은좋으나장기간성적에대한자료가부족 기술적으로어렵다.
30mm balloon 시술후 liquid diet 시도하였으나힘들어함. 35mm balloon으로 2차 balloon dilation 후증상호전되어퇴원함.
38 세 / 남 주소 : 흉통및삼킴곤란 기간 : 4 주 내원 4 주전부터발생한흉통및삼킴곤란으로타병원에입원하여상부위장관내시경검사및보존적치료하였으나증상의호전이없이물도못삼키는삼킴곤란과수시로발생하는심한흉통발작이지속되어본원으로전원됨. 과거력 : 특이사항없음. 가족력 : 특이사항없음. 사회력 : 소주 2 병 / 회 x 2-3 회 / 주 x 15 년 Smoking 1 갑 / 일 x 15 년 생체징후및신체검사소견 : 특이소견없음
WBC 5990/mm3 (Neutro 51%, Lympho 36%, Mono 5%) Hgb 15.7 g/dl, Plt 217,000/mm3, ESR 4 mm/hr Alb 4.5 g/dl, AST/ALT 23/39 IU/L, T. Bil 1.1 mg/dl, BUN/Cr 10.4/0.9 mg/dl, Na + 137 meq/l, K + 4.4 meq/ml 심전도 : 정상 흉부단순 X-ray: 정상
다음검사나처치로가장적합한것은? 1) 비디오투시연하검사 2) 식도내압검사 3) 24시간보행성식도임피던스, 산도검사 4) 식도부지확장술 5) 식도조직검사
Esophageal body LES
가장적합한진단명은? 1) 전형적인아칼라시아 (Type I) 2) 호두까기식도 3) 격렬형아칼라시아 (Type III) 4) 비효율적식도운동 5) 피부경화증식도
Vigorous achalasia" Defined by the presence of normal to high-amplitude simultaneous esophageal body contractions in the presence of a nonrelaxing LES High-amplitude and long-duration esophageal body contractions may overlap with diffuse esophageal spasm. Diffuse esophageal spasm At least 20% of swallows must be simultaneous. If every swallow sequence is simultaneous or if failed LES relaxation is noted, the diagnosis of achalasia needs to be strongly considered.
가장적합한치료는? 1) 풍선확장술 2) 자가확장형인공관삽입술 3) 칼슘통로차단제 4) 위장운동촉진제 5) 식도부지확장술
Pneumatic balloon dilation 30 mm Rigiflex balloon, 15 PSI, for 60 s
Dysphagia: slight improvement, but Chest pain: recurrent, not improved
Pneumatic dilation: poor response factors Related to patient Younger age (< 40 yrs) Male gender Wide esophagus Related to procedure Single dilation Small size balloon (< 30 mm) LES pressure > 10-15 mmhg within 1 yr of procedure Poor esophageal emptying on post-treatment barium swallow Related to manometry Type I & III pattern on high resolution manometry
가장적합한치료는? 1) 풍선확장술 2) 자가확장형인공관삽입술 3) 식도절제술 4) 보톡스주입치료 5) 식도부지확장술
Botulinum Toxin Injection on esophageal body and LES
Dysphagia: much improved, Chest pain: much improved
55 세 / 여 주소 : 삼킴곤란 기간 : 2 주 내원 2 개월전부터안면부종이발생하였고, 1 개월전부터발열및오한없이전신근육통이발생하였으며, 2 주전부터양상지와하지의근위부무력감과삼킴곤란이있고, 내원 1 주전부터는유동식이및고형식이모두삼키기어려워서입안에음식물이고여여러번삼키기를시도하였고, 연하시기침이자주동반되어내원함. 과거력 : 특이사항없음. 가족력 : 특이사항없음.
생체징후및신체검사소견 : 전신부종, 양측상안검및안와주변에피부발진이관찰흉부및복부에서이상소견없음. 상지및하지의근력이근위부에서 MRC grade 3-4 로평가 Shirmer 검사상양안모두 4 mm/5 분으로안구건조증소견
말초혈액검사 : 백혈구 5,700/mm3, 혈색소 10.9 g/dl, 혈소판 109,000/mm3, 전해질검사 : Na 138 meq/l, K 3.8 meq/l, Cl 102 meq/l 생화학검사 : BUN 13.6 mg/dl, Cr 0.56 mg/dl, 혈청총단백 5.2 g/dl, 알부민 3.2 g/dl, AST 131 IU/L, ALT 51 IU/L, 총빌리루빈 0.4 mg/dl, 혈청 LDH 823 IU/L, 혈청 CK 826 IU/L 로상승, ANA 는 1:640 으로양성, 류마티스인자양성, anti-ds-dna antibody 음성, anti-jo-1 antibody 음성, anti-sm antibody 음성, anti-ro antibody 양성, anti-la antibody 음성, anti-rnp antibody 음성, C3, C4 는정상범위. 상부위장관내시경검사, 단순흉부, 복부방사선검사, 복부초음파검사, 뇌 MRI 에서특이소견이관찰되지않았다.
다음검사나처치로가장적합한것은? 1) 비디오투시연하검사 2) 식도내압검사 3) 24시간보행성식도임피던스, 산도검사 4) 식도부지확장술 5) 식도조직검사
비디오투시연하검사 Administration of radiopaque material (usually barium) mixed with liquid and food in smaller to larger amounts and thinner to thicker viscosities as tolerated
1. To identify normal and abnormal anatomy and physiology of the swallow. 2. To evaluate integrity of airway protection before, during, and after swallowing. 3. To provide recommendations regarding the optimum delivery of meal (e.g., oral versus nonoral). 1. Disrupted lingual propulsion (weak, disorganized) 2. Delayed or absent swallow response (aspiration before swallow) 3. Reduced epiglottic inversion 4. Reduced pharyngeal propulsion (aspiration after swallow) 5. Reduced laryngeal closure (aspiration during swallow) 6. Reduced sensation to residue 7. Reduced esophageal sphincter (UES) opening (aspiration after swallow)
비디오투시연하검사 1. Weak lingual propulsion 2. Delayed swallow response (aspiration before swallow) 3. Reduced epiglottic inversion 4. Reduced pharyngeal contraction (aspiration after swallow) 5. Retained residue in valleculae and piriform sinus
식도내압검사
Electromyography from left biceps brachii muscle : distinguishing weakness of myopathic origin from neuropathic disorders Increased membrane irritability : Increased insertional activity and spontaneous fibrillations, Abnormal myopathic low amplitude, short duration polyphasic motor potentials
Muscle biopsy from right biceps brachii muscle: the definitive test for establishing the diagnosis of inflammatory myopathy and excluding other causes of muscle weakness Diffuse extensive myonecrosis and regeneration with perifascicular atropy and perivascular lymphocytic infiltrations
가장적합한진단명은? 1) 전형적아칼라시아 2) 피부근염 3) 피부경화증식도 4) 비효율적식도운동 5) 윤상인두군아칼라시아
Dermatomyositis is an idiopathic disorder that includes an inflammatory myopathy and characteristic skin manifestations. Polymyositis includes the inflammatory myopathy without the cutaneous findings. Symmetric proximal muscle weakness Typical rash of DM Elevated serum muscle enzymes Myopathic changes on electromyography Characteristic muscle biopsy abnormalities The etiology of DM remains unknown. The average age at diagnosis is 40, and almost twice as many women are affected as men.
가장적합한치료는? 1) 자가확장형인공관 ( 스텐트 ) 삽입술 2) 칼슘통로억제제 3) 풍선확장술 4) 스테로이드 5) 보톡스주입치료
Oral prednisone 0.5 to 1.5 mg per kg daily until serum creatine kinase normalizes, then slowly taper over 12 months Consider adjunctive therapy if no improvement in objective muscle strength after three months of therapy (azathioprine or methotrexate) 스테로이드치료후 (prednisolone 40 mg/ 일 ) 삼킴곤란호전되고, 근력약화등의증상이점진적으로호전되었고, 퇴원후서서히감량함.
46 세 / 남 주소 : 삼킴곤란과흉통 기간 : 수개월 내원수년전부터간헐적으로발생하는삼킴곤란과흉통이있었고, 수개월전부터자주반복되어타병원에서 EGD 시행하였고, GERD 의심하에치료하였으나호전없어서내원함. 과거력 : 기관지천식및알레르기비염 가족력 : 특이사항없음. 생체징후및신체검사소견 : 특이사항없음 일반혈액검사 : 백혈구 6,290/mm3 (eosinophil 4.4%), 혈색소 12.9 g/dl, 혈소판 139,000/mm3, 전해질검사및생화학검사 : 특이소견없음
Esophageal body LES 24hr ph-impedance monitoring: no pathologic acid or nonacid reflux, no symptom correlation with symptoms
EGD
가장적합한진단명은? 1) 아칼라시아 2) 위식도역류질환 3) 피부경화증식도 4) 호산구성식도염 5) 바이러스성식도염
Diagnostic criteria of Eosinophilic esophagitis The presence of symptoms : dysphagia, food impaction, heartburn and chest pain 15 eosinophils per hpf in the esophageal biopsy Exclusion of other disorders such as GERD Unknown pathogenesis but associated with allergic disorders (eosinophil infiltration related to an allergic response?) Treatment of EoE Dietary elimination of causative foods Systemic or topical steroids 경구스테로이드 (prednisolone 30 mg, 하루 1 회 ) 치료후증상호전되었고, 이후감량함.