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CASE OF THE MONTH. DR. SHAILESH MANANDHAR PAEDIATRIC RESIDENT 1 ST YEAR IOM. Patient’s Profile. Name : Kapindra Regmi Age / Sex – 12yrs / M Add : Dolkha I.P No : 12153 DOA : 2062/7/12 at 9:30 pm DOD : 2062/8/8 at 11:00 am
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CASE OF THE MONTH DR. SHAILESH MANANDHAR PAEDIATRIC RESIDENT 1ST YEAR IOM
Patient’s Profile • Name : Kapindra Regmi • Age / Sex – 12yrs / M • Add : Dolkha • I.P No : 12153 • DOA : 2062/7/12 at 9:30 pm • DOD : 2062/8/8 at 11:00 am • Admitting Diagnosis: Lt. Pyopneumothorax with neck mass ? Lymphoma • Final Diagnosis : Malignant thymoma Stage 3 with Lt. chylopneumothorax
Chief complains • Progressive difficulty in breathing for 10 days • Difficulty in swallowing for 10 days • Upper abdominal swelling for 5 days
History of Present Illness • Patient was apparently well 20 days back. Then , he developed mild to moderate grade fever intermittent not a/w chills & rigor but with profuse night sweats lasting for 10 days. • Cough with mucoid expectoration, no hemoptysis, no vomiting, no dysuria, no pain abdomen • Chest pain more on left side and on coughing. • Complained throat pain during swallowing • Taken treatment for local medical shop tab. Ofloxacin 200mg BD for 7 days – fever subsided but cough not improved
HOPI Contd………….. • Subsequently mother noticed swelling in neck ; followed by swelling of upper abdomen couple of days later. • Gradual difficulty in breathing and swallowing • Bowel and bladder habit were normal • No noisy breathing • No H/O joint pain or swelling, rashes, swelling of legs, no hoarseness of voice, no jaundice. • No h/o bony tenderness. • No H/O loss of consciousness or seizure
HISTORY OF PAST ILLNESS Never needed medical care and medication.
FAMILY HISTORY • Only child in family • Mother: 35years/ House-wife • Father : expired 4 years back in RTA • Low socio economic class family • Mother earn living by working in own small field and other people’s houses in village • No H/ O similar illness/ TB in the family or in close contacts. • No H/O consanguinity of marriage. • No H/O pet in the family.
BIRTH HISTORY - FT/S/F/NVD at Home . Ante-natal/intra-partum or postpartum period: Uneventful • IMMUNIZATION HISTORY – not immunized • DEVELOPMEENTAL HISTORY - Normal for his age • NUTRITIONAL HISTORY - Taking less than required calorie.(80 Kcal/kg/day)
TREATMENT HISTORY • Admitted In Kathmandu Model hospital, Dolkha one week back. • Inj. Ceftriazone 50mg/kg/d q 12hr for 5 days • History of aspiration of thick white fluid from left chest twice. • Referred from there with discharging diagnosis of left pyopneumothorax with neck mass ? Lymphoma to cardio thoracic surgery department for evaluation and management of neck mass and chest tube insertion.
ON EXAMINATION • Child was conscious oriented to time, place and person but looked dyspneic and tachypnic. • Vitals: Pulse: 116b/min normo volumic R/R : 48/min regular B.P : 90/40 mm Hg Temp: 1000C • Anthropometry: wt. 25 kg Ht. 136 cm wt. for age : 62.5 % of expected ht. for age : 90 % of expected wt. for ht. : 83.3 % of expected
Puffy face with mild submandibular region swelling. • Distended neck veins. • Visible swelling on anterior lower part of neck. On palpation hard fixed mass approx. 3 cm by breadth ill defined borders arising from retrosternal region. Can’t get lower border of mass. Neck mass –doesn’t move on deglutition/ protrusion of tongue.
Lymphnodes- Single Rt. Axillary 1*1 cm, multiple cervical and submandibular 0.5 to 1cm • Pallor / jaundice / cyanosis – absent • Clubbing- absent • Pedal edema – absent. • Ear/nose and throat examination- normal • Joints : Normal • Bony tenderness: Absent • Non pitting edematous swelling of anterior chest and upper abdomen.
RESPIRATORY EXAMINATION • INSPECTION- Fullness in Lt. anterior chest wall. Reduced movement on same side. • PALPATION- trachea- deviated to left side. Reduced tactile and vocal fremitus in Lt. side on lower half. • PERCUSSION- stony dullness on Lt. lower part of chest. Hyperresonant note on Lt. upper part of chest. • AUSCULTATION- absent BS on Lt. side of chest. Normal vesicular breath sound with no added sound on right side of chest.
PER ABDOMEN :- Inspection: mild distension of upper part of abdomen; visible veins with flow up to downward at supraumbilical region, central umbilicus. No scar marks, No visible peristalsis Palpation: No tenderness on superficial or deep palpation. Liver/Spleen non palpable. No abdominal LN palpable. Hernial orifices: Intact Both testes were palpable at lower end of inguinal canal normal size with intact sensation. Percussion: Tympanic. No fluid thrill or shifting dullness Auscultation: normalbowel sound
CVS : apex beat localized at 4th ICS 3cm medial to left nipple. S1,S2 – N , No murmur • CNS : - conscious, cooperative and oriented to time, place and person. - cranial nerves grossly intact - muscle tone/power/bulk were normal in all limbs - no involuntary movements - superficial and deep tendon reflexes N - Sensory examination with in normal limit - Normal Gait.
PROVISION DX:Lt. Pyopneumothorax with neck mass ? Lymphoma ? Tuberculosis • INVESTIGATIONS CBC: Hb: 13.4 gm% TC: 12,100 (N-75, L- 15, E-8, M- 2) Platelet:160,000/mm ESR : 12 Na: 139 K: 4.5m mol/L Urine R/E: normal
Chest tube was inserted on left side - whitish turbid fluid ~ 2.5 litre drained and admitted in Medical Ward. • Inj. Cloxacillin 100mg/kg/d • Inj. Amikacin 15mg/kg/d • Tab. Paracetamol 6hrly
D3 of admission • Reduced dyspnea, puffiness of face and look comfortable. • Tem. 1000F PR 102/m B.P: 96/60 mm Hg • Chest tube drain ~ 1.5 lit of milky white fluid with little dirty white fibrinous sediments . • Eating well .urine - N INV: Pleural Fluid Analysis- TC: 21,900/cumm (L- 91%, N- 9%) protein – 61 micromol / L
A> Lt. chylopneumothorax ? Lymphoma ? Tuberculosis • Plan- pleural fluid triglyceride level sr.creatinin, urea, LFT Sputum for AFB Mantoux test
D4,D5 of admission • Complains cough and chest pain otherwise comfortable. • Vital stable still have low grade fever. • Non pitting edema of left upper limb noted. • Chest tube draining more milky fluid upto 2.5litres. • No dehydration, passing urine. • Air entry on left side present. INV - pleural fluid triglyceride level – 9.3 m mol/L ( 0.5 – 1.8 m mol/L) Sr.creatinin – 0.4mg/dl Urea – 20 mg/dl. Sr. albumin 2g% Total protein – 3g% SGPT 15IU SGOT 16 IU
Blood C/S – sterile after 96 hrs. Pleural fluid C/S- E.coli isolated sensitive to Imipenam Intermediate sensitive to Amikacin resistant to Ampi, cipro, genta, ceftazedime, tobramycin, cephalexin, cotrim. USG - Multiple enlarged LNs in neck, mediastenum and clavicular region with left pleural effusion. Liver- enlarged in size, no SOL other viscera WNL. • X- ray chest Lateral view • Plan for neck mass / lymph node biopsy.
D6 • Patient’s condition static • Mx – no induration • Sputum for AFB- Negative. • Advice to take high protein diet.
D8 – D10 • Looked better • Fever subsided, still complains cough. • Neck swelling and venous prominence reduced than admission day. • Neck mass felt softening than before. • Chest tube functioning; draining still 1 -2 lit/day
D12 • Case referred to CTVS department, TUTH for expert opinion and further management. • Case seen by Dr. P. Sayami, with impression of mediastinal lymphoma with left chylothorax, USG guided FNAC of mediastinal mass and follow up with report was adviced.
D15 • USG guided FNAC of neck mass done – Mixed population of mature and immature lymphoid cells; with few clusters of atypical epithelial cells – moderately Pleomorphic, oval to spindle nuclei and scanty cytoplasm and few squamous cells in background. Impression: Malignant Thymoma.
Plan for CT scan thorax • Patient party counseled about the disease and possible therapy and poor prognosis.
D17 – D20 • Patient look dysneic with puffy face. • Complaining of dysphagia more for solid food • Temp. 1010F PR 120/m BP- 90/50 mmHg • No Ptosis, no diplopia, no muscle weakness • No rashes, joint pain. • Chest - absent BS on left lower 2/3rd. • Chest tube not draining since yesterday. • Chest tube irrigation done and Chyle started draining.
D21 • CT scan thorax- Large lobulated soft tissue density well defined mass in anterior superior mediastenum that shows heterogenous enhancement with contrast; encasing all the major vessels of the mediastenum including aortic arch and pulmonary arteries. Thick irregular enhancement of pleura with nodular thickening at places. Impression- Malignant thymic mass
D22 • Consultation with CTVS team was done for further management of case with CT report. • With impression of advanced thymic carcinoma – inoperable, case was referred for Radiotherapy from their side.
D23 • Patient general condition- same • Patient party were given option for treatment after explaining prognosis of disease. • Referred to Bhaktapur Cancer Hospital for further treatment.
D25 • Patient came back from Bhaktapur Cancer Hospital with a referral letter to oncology department. Patient was advised palliative chemotherapy of Ipbosphamide and adriamycin and asked to come on follow up for radiotherapy.
D26 • On oncology department patient party again counseled about the poor prognosis of disease and possible expenses. • Then, they refused treatment.
Discharged on request on 2062-08-07 WITH FINAL DIAGNOIS – Thymic carcinoma ( stage 3) with Lt. chylopneumothorax & superior vena cava syndrome without obvious paraneoplastic syndromes.
ANATOMY : • Mediastinum – • pleural cavity laterally • Thoracic inlet superiorly • Diaphragm inferiorly. • Anterior– Thymus, Fat and LN • Middle – Heart, pericardium, ascending and transverse aorta, branchiocephalic veins, trachea, bronchi, LN • Posterior – descending thoracic aorta, esophagus, azygos vein, autonomic ganglia and nerves, thoracic LN
Differential diagnosis of mediastinal mass by anatomic location ANTERIORMIDDLEPOSTERIOR Thymoma Lymphoma Neurogenic tumor Teratoma/seminoma Pericardial cyst Bronchogenic cyst Lymphoma Broncogenic cyst Enteric cyst Parathyroid adenoma Metastatic cyst Xanthogranuloma Intrathoracic goiter Systemic granuloma Diaphragmatic hernia Lipoma Meningocele Lymphagioma Paravertebral abscess Aortic aneurysm