a case of chylothorax
TRANSCRIPT
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Dr.E.ThirulogachandarPROF.S.TITO’S UNIT
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Karthik a 15 yr male admitted withc/o swelling of Rt.chest-1 week breathlessness -1 week dry cough- 1week fever -4 days
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HOPI;H/o Swelling Rt.upper chest -1 week Onset was spontaneous, progressively
increased to face over a period of 3 weeks after admission.
No h/o trauma.H/o breathlessness -1 week grade 3 on onset
progressed to grade 4 after 3 weeks. H/o dry cough -1 week.H/o fever -4 days low grade.Noh/o chestpain, syncope, haemoptysis, hoarseness of voice
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Past history:nil relevant
Personal history :mixed diet
Family history : nil significant illness
General examn: pt conscious, oriented, febrile comfortable, pallor+, not jaundiced, no cyanosis, no clubbing, no lymphadenopathy.Pulse:82/mt,BP 96/72 mmHg,RR-17/mt
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Inspection&palpation: Rt. Upper chest is swollen with fullness
supraclavicular region, Trachea in midline, AI in normal place, movements diminished Rt.side, vocal fremitus increased in Rt.infraclavicular,axillary&suprascapular regions Percussion :impaired resonance present in
Rt.infraclavicular,axillary and suprascapular regions.
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Auscultation :Bilateral air entry prtesent NVBS+bilaterally Br.breath sounds present in
Rt.infraclavicular,axillary suprascapular and areas.
VR increased in same regionsCVS: S1,S2 heard,no murmursAbd:soft,no organomegalyCNS:no FND.
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MASS lesion Rt. Upper lobe-?cause
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CBC-Hb-8.6 BLOOD-SUGAR-96
TC-7400 UREA-18
DC-P72L27E3 CREATININE-0.7
ESR-12/24 Na-134,K-3.9
PCV-24% LFT-WNL
PLT-1.OLAC
MCV-72.5
MCH-27.4
MCHC-30.1
RBC 3.0 MILLIONS
URINE-ALB-nil
sug-nil
dep-1-4pc/hpf
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Pleural fluid Analysis: colour-milky white Gram’s stain&AFB- neg culture-neg Cholesterol-214mg/dl TGL-2800mg/dlUSG Abd;NORMAL WITH Lt.pleural effusion
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CT guided FNAC-Necrotic mass with inflammation
Biopsy-skeletal muscle bundles with blood clot-non diagnostic
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β HCG-neg α-FP-neg LDH-neg Ab to Ach receptor-not done
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CTS OPINION:As he had massive pleural effusion-chylothorax with respiratory distress Lt.ICD was placed chyle was drained.
about 200 ml of chyle was draining daily.
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In view of diagnostic dilemma -5 weeks and GC deterioration of the pt, he was sent to CTS Dept,GGH,Chennai .
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CT-guided biopsy repeated-nondiagnostic Planned open biopsy and deferred. Finally median sternotomy and whole
tumour was excised and sent for HPE.
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Thymoma –stage B 1
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CHYLOTHORAX: 1.Malignancy (50%) lymphoma(3/4),Br.car 2.trauma,surgery-25% 3.Miscellaneous- sarcoidosis,ccf lymphangiomatosis, yellow nail syndrome, kaposi’s sarcoma, post RT, Tb,filariasis,histoplasmosis,cirrhosis 4.Idiopathic-neonatal
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Conservative 50% resolve Octreotide Cessation of oral intake Total parenteral nutritionSURGICAL TREATMENT: Thoracic duct ligation Pleurodesis Pleuro peritoneal shunt pleurectomySURGICAL INDICATION:>1litre/day for 2 days or persistent leak for
2 weeks, nutritional,metabolic complications, post oesophagectomy status
Treatment of chylothoraxMEDICAL
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anterioranterior
Anterior
Posterior-Des.aorta,azygosvein,nodes,ganglia
Middle-Heart,tachea,bronchus,nodes,vessels
THYMUS,FAT,NODES
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the functional unit of the thymus gland is the Lobule◦ composed of a cortex & medulla◦ epithelial cells◦ lymphocytes
thymomas are, by definition, primary tumors of benign-appearing thymic epithelial cells
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Separated into three histologic categories— 1. Thymoma. 2. Thymic carcinoma. 3.Neuroendocrine tumor.
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Given this definition, most thymomas are “benign”◦ the majority behave in an indolent fashion
◦ however, they can invade and metastasize (uncommon)
◦ when a thymoma is contained within the thymic capsule, it is referred to as “benign” or “noninvasive”
◦ when it penetrates through the capsule, it is referred to as “malignant” or “invasive”
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Represent 20% of all mediastinal neoplasms in adults
Most common anterior mediastinal neoplasm in adults (less common in children)
incidence of thymoma is 0.15 cases per 100,000
Age range is generally 40-60 Equal prevalence on gender. No known risk factors (+/- myasthenia gravis) Thymic carcinoma represents less than 1% of
thymic malignancies.
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95% in anterior mediastinum. Neck. Left hilar region. Within lung parenchynma. Anterior cardiophrenic angle.
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Most important gross feature — The presence or absence of encapsulation of tumor and the gross invasion into adjacent structure.
The invasion present — The thymoma must be considered malignant lesion regardless the microscopic appearance.
Extensive spread. Incidence of distal metastasis is 3%.
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Stage I: Intact capsule or growth within the capsule Stage II: Invasion into surrounding fatty tissue or
mediastinal plura◦ Stage IIA: microscopic invasion through the capsule◦ Stage IIB: macroscopic and microscopic invasion through
the capsule Stage III: Macroscopic invasion into neighboring organs
(pericardium, great vessels, lung) Stage IVA: Pleural or pericardial dissemination Stage IVB: Lymphogenous or hematogenous metastases
Masaoka et al. Cancer 1981;48:2484-2492
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40-60 yrs. Sex distribution — Equal. - asymptomatic in 1/3 of pts - chest pain, breathlessness, cough, SVC syndrome, paralysis of hemidiaphragm -hoarseness, weight loss, fatigue, fever,If ass. With MG-Mc in women
Fatiguablity,ptosis,diplopia,dysphagia, weakness
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Standard posteroanterior and lateral chest radiographies.
CXR—Smooth or lobulated mas, right side the silhouette sign present, left side the sign abscent.
Calcification — 10%. CT—Delineate the extent of mass,
cannot not differentiating benign and malignant, assessing intrathoracic spread of an invasive thymoma.
Ach receptor Ab
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Unnecessary for a suspected locally symptomatic thymoma, because the capsule of tumor may be violated by invasive procedure.
Only distinguish the tumor from the other malignant tumour. It is to establish the diagnosis before making decision of therapy.
Fine needle biopsy by CT or sono-guide. Extend substernal mediastinoscopy. VATS.
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Stage-Directed◦ Stage I: Surgical resection only◦ Stage II: Surgical resection +/- adjuvant
radiotherapy◦ Stage III: Surgery with adjuvant radiotherapy or
neoadjuvant chemotherapy, surgery, and adjuvant radiotherapy
◦ Stage IV: Chemoradiotherapy without surgical resection
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Cisplatin remains cornerstone of therapy “CODE” regimen: neoadjuvant cisplatin,
epirubicin/doxorubicin, etoposide. Improved survival in pts with stage III and IV dz when compared with historic controls.
Somatostatin or somatostatin analogs with prednisolone have been reported to induce both PR and CR in pts with refractory disease
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Thymoma is associated with a wide variety of paraneoplastic syndromes
Mechanism is unknown Theory:
◦ medullary/dendritic cell areas are required for induction of tolerance.
◦ If these areas are abnormal, positively selected autoreactive CD4+ T-cells may escape surveillance and generate autoantibody-producing B-cells
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Most commonly associated disease. 30-50%of patient with thymoma
associate with MG. Only 10-15% patient with MG have
thymoma. Little effect on local presention, clinical
behavior, prognosis.
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In the Johns Hopkins series 10%of pts with thymoma had PRCA, 2nd most common paraneoplastic syndrome
Antigen has not been determined Usually responds to rx with corticosteroids
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Def: thymoma, hypogammaglobulinemia, defects in cell-mediated immunity (low or absent B cells, CD4 lymphopenia, and inverted CD4:CD8 ratio
Develops in 3-6% of pts with thymoma Most develop syndrome within six years of
dx of thymoma Immunologic abnormalities do not correct
with corticosteroids or thymectomy
Tarr et al. Medicine. Vol 80 No 2 March 2001
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Recurrent sinopulmonary infections Mucocutaneous candidiasis Encapsulated organisms CMV, HSV, VZV PCP MTB
Tarr et al. Medicine. Vol 80 No 2 March 2001
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THANK YOU
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Mayo Clin Proc. 2005;80(7):867-870
Tumourrs of mediastinum –CHEST 2005;126; 2693-2909
European journal of CTS 2010;37;13-25
European journal of CTS 2002;21;307-313