Protocol - Lymphangioleiomyomatosis (HCC 02242023)
Protocol - Lymphangioleiomyomatosis (HCC 02242023)
Protocol - Lymphangioleiomyomatosis (HCC 02242023)
“Harder to Breath”
A case of an 84-year-old, female with Lymphangioleiomyomatosis
Objectives
Perhaps we can phrase it this way:
1. To present and discuss the clinical features and for a patient with Lymphangioleiomyomatosis
2. To evaluate and appraise the latest evidenced-based recommendations on the diagnosis and
management of Lymphangioleiomyomatosis
3. To appreciate the interaction of comorbidities, short- and long-term complications of patients
diagnosed with Lymphangioleiomyomatosis
Identifying Data
This is a case of CF, an 84-year-old, female from Mandaluyong City, widow, Catholic who was
admitted last January 18, 2023 due to a chief complaint of dyspnea. I think we can go straight to
admitted for dyspnea
Patient was previously admitted in other institution last December 21, 2022 due to difficulty of
breathing and desaturations as low as 85%. She was managed as a case of Secondary Spontaneous
Pneumothorax sec to Cystic Lung Disease (Lymphangioleiomyomatosis), s/p CTT Insertion last Dec. 23,
2022. Patient was discharged improved and relatively well; and was mostly dependent on her caregiver,
needing assistance to stand up and transfer to a chair.
5 days PTA, patient had productive cough. No other symptoms noted like fever, dyspnea, chest
pain. No consult was done.
1 day PTA, still with productive cough. Denies other symptoms like dyspnea, chest pain, fever.
Patient went to our institution for change of NGT and for CXR in preparation for follow-up with APs the
following day. CXR revealed right sided pleural effusion.
Night PTA, patient was complaining of minimal dyspnea on exertion but the condition was
disregarded since it spontaneously resolved. No medications were taken. No consult was done.
1 hour PTA, noted increased severity of dyspnea and productive cough. This was associated with
desaturations as low as 91-93% at room air. Denied chest pain, fever, and diaphoresis. She was then
brought to our institution; hence, admitted.
Thoughts:
Make a separate entry on the patient’s baseline functional capacity
Based on the HPI she had NGT at home? When was this? What is her feeding regimen?
Nutritional status?
When was the patient diagnosed? How was it diagnosed? What were her presenting features?
Review of Systems (Please review this include only the pertinent negative/positive during
presentation)
General: (-) weight loss, (-) chills, (-) night sweats, (-) anorexia, (-) changes in sensorium or behavior
Skin: (-) cyanosis, (-) jaundice, (-) rash, (-) pruritus
HEENT: (-) headache, (-) dizziness, (-) blurring of vision, (-) ear pain, (-) tinnitus, (-) epistaxis, (-) sore
throat
Cardiovascular: (-) chest pain, (-) orthopnea, (-) PND
Pulmonary: (-) hemoptysis
Gastrointestinal: (-) bowel movement changes, (-) melena or hematochezia (-) hematemesis, (-) vomiting
Genitourinary: (-) dysuria, (-) hematuria, (-) nocturia, (-) urinary incontinence, (-) vaginal bleeding
Extremities: (-) joint pains, (-) weakness, (-) numbness, (-) tremors, (-) muscle cramping
Endocrine: (-) heat or cold intolerance, (-) excessive thirst or hunger, (-) polyuria
Maintenance medications:
1. Telmisartan + Amlodipine 80 mg/5 mg 1 tab OD
2. Nebivolol 5 mg tab OD
3. Digoxin 0.25 mg tab ½ tab OD
4. Rosuvastatin 20 mg tab ODHS
5. Pizotifen + Vitamin B complex (Mosegor Vita) 1 cap BID
6. Symbicort Rapihaler 160 mcg/4.5 mcg 2 puffs BID
7. Apixaban 2.5 mg tab BID
8. Levetiracetam 500 mg/tab 1 tab BID what is the indication for this?
Family History
Patient has a family history of hypertension on the maternal side; otherwise, there is no known family
history of hypertension, bronchial asthma, thyroid disease, malignancy, cerebrovascular disease, or
myocardial infarction.
Physical Examination: I have not seen the patient so this PE is based on what was obtained during
admission
General: Awake, conscious, conversant, not in cardiorespiratory distress; speaks in full sentences
Vital Signs: 160/60 mmHg, 64 bpm, 21 cpm, 36.4oC, 93% at Room Air
HEENT: Anicteric sclerae, (+) pale palpebral conjunctiva, no cervical lymphadenopathies, moist oral
mucosa
no gross deformities
Chest and Lungs: Symmetrical chest expansion, (+) bilateral crackles, mid to base, (+) decreased breath
sounds more on the right, (-) wheezing
Heart: Adynamic precordium, normal rate, (+) irregular rhythm, (-) heaves (-) thrills
Abdomen: Flat abdomen with no deformities, normoactive bowel sounds; soft and non-tender to
palpation
Extremities: Full and equal peripheral pulses; (+) bipedal edema, pitting, grade 1; (-) cyanosis; (-)
generalized or palmar pallor.
Neurologic:
General
GCS 15; Oriented to time, place, and person
Cooperative and responds appropriately during history and examination
Cranial Nerves
CN 1: Not assessed but claims that her sense of smell is intact when she had her morning coffee
CN 2, 3: Pupils 2mm EBRTL; Decreased peripheral vision on the left eye
CN 3, 4 and 6: Full and equal EOMs
CN 5: Intact V1-V3 sensory function; Good TMJ tone
CN 7: No facial asymmetry at rest; able to smile and elevate eyebrows without difficulty
CN 8: Intact Gross hearing on both ears
CN 9, 10: No hoarseness of voice, intact gag
CN 11: Good SCM and trapezius tone
CN 12: Tongue midline
Meningeals
No Brudzinski
No Kernig
Pathologic Reflexes
No Babinski
No Grasp Reflex
Before proceeding to the work-up, you should come up with you primary working impression
followed by a list/table of differentials (provide the rationale for each)
IMPRESSION:
12/29/22 Plain CT Scan of Lower neck: The thyroid gland is normal in size. No enlarged
the Chest lower neck lymph nodes identified
IMPRESSION:
2. Status post right closed tube thoracostomy, with the chest tube
tip abutting the right lateral chest wall.
-Atherosclerotic aorta
1/18/23 Chest UTZ -Real time scanning of both hemithoraces while the patient is in
semi-recumbent position show free intrathoracic fluid collection
on the right hemithorax, with an estimated volume of at least 380
ml in the right. Floating echogenic debris is noted. No loculations
nor septations noted. Associated atelectasis and consolidation of
the adjacent lower lobe is observed.
IMPRESSION:
1/29/23 CXR -Follow-up examination since January 16, 2023 shows interval
increase in the ground glass opacities and veil of haziness now
seen in both lungs, obscuring the cardiac borders,
hemidiaphragms and costophrenic sulci.
IMPRESSION:
-ATHEROSCLEROTIC AORTA
1/30/23 Chest UTZ -Reference was made to a previous chest UTZ dated Jan. 18, 2023.
-There is interval increase in the free pleural fluid seen in the right
hemithorax now with an estimated volume of 819 ml (previously
380 ml). Likewise, there is interval development of free pleural
fluid in the left hemithorax with an estimated volume of 286 ml.
Floating echogenic foci are seen in both hemithoraces.
IMPRESSION:
-Trachea is midline.
Degenerative osseous changes remains seen.
II: Hematology:
III. Chemistry:
Make sure to compute for PF to serum ratios and interpret based on Light’s criteria
IV: Microbiology:
Sputum Gram Stain for Bacteria (1/19/23) More than 25 pus cells/LPF.
Less than 25 epithelial cells/LPF.
Few gram positive cocci singly, in pairs and in
chains seen.
Occasional gram negative diplococci seen.
Many gram positive bacilli seen.
Occasional gram negative bacilli seen.
Fungal elements seen.
Sputum CS (1/19/2023)
Microbial Growth: Predominant growth (moderate growth) of *Alpha Hemolytic Streptococcus ,
**Diphtheroids, Extended-Spectrum-Beta-Lactamase producing Klebsiella oxytoca and
Stenotrophomonas malthophilia after 24 hours of incubation
(*Normal respiratory flora)
(**Possible contaminants, please correlate clinically)
Resistance marker: ESBL (+)
Microoganism/s Isolated: Klebsiella oxytoca
Resistant Amoxicillin/Clavulanic, Ampicillin, Aztreonam, Cefepime, Cefoxitin, Ceftazidime/
Ceftriaxone, Cefuroxime, Chloramphenicol, Ciprofloxacin, Cotrimoxazole,
Ertapenem, Imipenem, Piperacillin-Tazobactam, Tobramycin, Ampicillin/Sulbactam,
Cefazolin, Cefotaxime, Levofloxacin, Ofloxacin, Tetracyclin, Ticarcillin/Clavulanic,
Sensitive Gentamicin, Amikacin,
Intermediate Netilmicin
Pleural Fluid Stain for Acid Fast Bacilli (2/1/23) No Acid Fast Bacilli Seen
Pleural Fluid Gram Stain for Bacteria (2/1/23) Occasional pus cells seen
No definite microorganism seen
Pleural Fluid TB GeneXpert (2/1/23) Mycobacterium tuberculosis NOT DETECTED
Pleural Fluid Culture and Sensitivity (2/5/2023) Microbial Growth:
Growth of *Bacillus spp. from thioglycolate broth
subculture after 24 hours of incubation
(*Possible contaminants, please correlate
clinically)
Current Working Impression: (this should be revised working impression since we already
incorporated the labs)
-Pleural Effusion, Right prob sec to Parapneumonic Process vs Cystic Lung Disease (t/c
Lymphangioleiomyomatosis (di na siya to consider kung previously diagnosed na?)
-Permanent AF
-HASCVD
-Bronchial Asthma Not in Acute Exacerabation – I’m not sure about this since no PFTs can substantiate
this claim
-S/P CVD Infarct with no Residuals (2016, 2022)
-Anemia prob sec to Infection
-S/P CTT Insertion (Dec 2022)
Patient was noted to have shortness of breath with RR of 23 cpm and O2 sat of 99% at 3 LPM. On PE,
noted to have bibasal crackles and decreased breath sounds more on the right. Repeat CBC was done
which revealed increased of WBC from 6.53 to 8.93 with neutrophilic predominance of 74.05 from 70.2;
CRP was elevated; serum electrolytes and creatinine were within normal values. Amikacin 750 mg IV
every 24 hours was then started by IDS service due to increasing infiltrates in CXR. Due to persistence of
dyspnea, patient was referred to Surgery service for pleural catheter insertion. Pleural fluid studies
were requested for analysis. Patient was then scheduled for CTT insertion the following day but since
the patient was on Apixaban, it was put on hold first for 24 hrs prior to CTT insertion.
Furosemide dose was decreased to 40 mg ½ tab every MWF. Patient underwent CTT Insertion in the
right hemithorax. Initial drain was 400 ml of serosanguineous fluid. Patient tolerated the procedure and
noted decreased in dyspneic episodes. Repeat ABG was done which showed respiratory alkalosis with
more than adequate oxygenation; so O2 support was decreased to 2 LPM. Pleural fluid which includes
LDH and total protein, cytology and cell block, diff count and cell count, AFB, GS/CS and TB Gene Xpert
was sent to laboratory for analysis.
Patient tolerated O2 support at 2 LPM with no desaturations noted. Pulmonology ordered no objection
for discharge after completion of antibiotics. Repeat CBC showed normal WBC with slightly increased
neutrophils. Ceftazidime + Avibactam was then discontinued by IDS service. Noted with no active
bleeding on pleural catheter insertion site so Apixaban 2.5 mg /tab ½ tab OD was resumed.
Noted with decreasing pleural catheter output for 24 hours. Patient was discharged improved with no
dyspnea, chest pain, no cough, afebrile and no desaturations at 2 LPM. Advised to have O2 support at
home for back-up if with recurrence of desaturations. Home medications given. Follow-up advised after
2 weeks with repeat CBC and creatinine.
Final Impression:
Final Impression:
Thoughts:
Overall: