Victor R.

Potenciano Medical Center

Department of Internal Medicine
Grand Rounds Presentation

“Harder to Breath”
A case of an 84-year-old, female with Lymphangioleiomyomatosis

Presented by: Brian S. Olaje, MD

Internal Medicine Resident YII

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

1. To report and present a case of Lymphangioleiomyomatosis

2. To summarize the current guidelines on the approach and management of
Lymphangioleiomyomatosis
3. To understand the possible complications that may arise 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

History of Present Illness

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

Past Medical History

(+) Bronchial Asthma-Late Onset: unrecalled if with Pulmonary Function Test
Last attack: more than 10 years ago, on Salbutamol nebulization PRN during attack.
(+) HASCVD; Usual SBP: 130 mmHg, Highest SBP: 180 mmHg
(+) Valvular Heart Disease, Severe Mitral Stenosis
(+) Permanent AF, NYHA III
(+) Chronic CVD Infarct
-1st episode last 2016 with no residuals; presenting as slurring of speech and right sided body
weakness
2nd episode last October 26, 2022 as right lower extremity weakness assessed as Acute CVD
Infarct, Parasagittal prob Cardioembolic.
(+) History of Gouty Arthritis, currently not on maintenance medications
S/P Appendectomy (year unrecalled)
S/P CTT Insertion (12/23/2022, SLMC)
Denied DM, PTB, COPD, Liver/Thyroid/Kidney Disease
No known allergies to food and drugs
Denied use of supplement or herbal medications

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?

Personal and Social History

Patient has no vices such as cigarette smoking, alcohol consumption nor recreational drug use. Patient is
functionally dependent and is bedridden since October 2022. Patient is fully vaccinated to COVID-19
with Sinovac and Pfizer as her booster.  What was her previous occupation? Did she have any
exposure to second hand smoke and burning of fossil fuels? Any occupational lung disease that may
predispose her to such? Does that patient have children? Who takes care of her finances?

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

MMTs and Sensory: No motor and sensory deficit

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)

Laboratory Work-Up Done

I: Imaging
Date Imaging Result

12/21/22 Non-contrast FINDINGS:

Axial Multislice
-Significant right-sided pneumothorax is appreciated with
CT scan of the
resultant passive atelectasis of the adjacent lung There are
chest
innumerable, vari-sized, thin-walled cysts diffusely scattered in
both lungs. Ground-glass densities with areas of consolidation are
seen in the right upper and middle lobes, predominantly in
Where was this
central distribution. Linear densities are also appreciated in the
done?
middle lobe, lingula, and both lower lobes.
In your PPT
-Bilateral pleural effusion is observed, more in the right.
presentation,
show the most -Heart is enlarged, with multi-chamber involvement and
important slice pronounced left atrial dilatation (6.8 cm). The main (3.6 cm) and
then go straight night (3.2 cm) pulmonary artery are also dilated. The aorta and its
to branches, as well as some of the coronary arteries, are diffusely
interpretation. calcified. Dense calcifications are also seen in the aortic and mitral
Do not put valves including its annulus.
sentences in the
-The trachea and both mainstem bronchi are patent without
slides
endobronchial lesion. Esophagus is grossly intact

-An enlarged subcarinal lymph node is detected, measuring 1.4

cm in short axis AP diameter

-Degenerative changes in the form of spurs and cortical endplate

 irregularities are exhibited along the visualized osseous
structures: Compression deformity of T12 is noted. Narrowing of
the L2-L3 disc space is observed. Mild posterior displacement of
L2 in relation to L3 is appreciated.

- Incidentally, the imaged upper abdomen shows a dilated left

renal pelvocalyceal system A 1.5 cm exophytic cyst is seen in the
right superior renal pole. Few tiny calcific densities are likewise
noted in the superior calyx of the left kidney, relating to
nephrolithiases. A dedicated CT scan of the abdomen is
recommended for further evaluation.

IMPRESSION:

-Findings of innumerable vari-sized cysts diffusely scattered in

both lungs with associated significant right-sided pneumothorax
may relate to lymphangioleiomyomatosis

-Ground glass densities and consolidation in the night upper and

middle lobes may suggest pulmonary edema changes, possibly
from left heart compromise (e.g. severe mitral valve
regurgitation). Other imaging differentials include viral
pneumonia or hemorrhage. Please correlate clinically

-Subsegmental atelectasis and/or fibrosis, both lungs

-Bilateral pleural effusion

-Enlarged subcarinal lymph node

-Cardiomegaly with pulmonary arterial hypertension Mitral and

aortic valve calcifications

-Atherosclerotic vessel disease

-Degenerative osseous and disc changes with T12 compression

deformity

-Mild retrolisthesis, L2 over L3

-Other findings, as described

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

Non-contrast? Lungs and airways: The innumerable var-sized thin-walled cystic

lesions scattered diffusely in both lungs appear unchanged, and
Where was this
still demonstrate some degree of subpleural predominance. There
done?
is marked regression of ground glass opacities and consolidation
in the right upper and middle lobes. Unchanged linear densities
are stal noted in the lingula, night middle lobe and both lower
lobes relating to subsegmental atelectasis and/or fibrosis

Pleura: Resolution of the night pneumothorax with better

aeration of the right lung. A medium caliber tube is seen entering
the 5th intercostal space on the night lateral chest wall looping
posteriorly with its tip apposing the right lateral chest wall.
Progression of the right pleural effusion. Minimal pleural effusion
is still noted on the left.

Heart: Heart is enlarged with the same degree of bilateral atrial

dilatation. The aortic and mitral valves are calcified. Normal origin
and configuration of the great vessels. The main pulmonary artery
remains dilated, still measuring 3.6 cm. The night pulmonary
artery is less prominent in size, measuring 2,8 cm (previously3.2
cm) in diameter. Wall calcifications are again seen along the
thoracic aorta, its branches, and coronary and coronary arteries.
No pericardial effusion

Mediastinum and Hila: Normal intrathoracic esophagus. No

mediastinal mass. There is interval decrease in size of the
subcarinal lymph node, now measuring 0.6 cm (previously 14 cm)
in its short axis diameter. No enlarged mediastinal or har lymph
nodes seen.

Bones: Degenerative osseous changes, multi-level degenerative

disk disease, and vertebral body compression deformity of T12
are again noted.

Chest Wall: Unremarkable. No axillary lymph node.

Upper abdomen: Hydronephrosis is again seen in the imaged left

kidney

Others: Feeding tube is seen with distal segment within the

stomach.

IMPRESSION:

1. Interval resolution of right-sided pneumothorax

2. Status post right closed tube thoracostomy, with the chest tube
tip abutting the right lateral chest wall.

3. Bilateral pleural effusion, with progression on the right, and

minimal and unchanged on the left.

4. Innumerable vari-sized cystic structures in both lungs with

some degree of subpleural predominance, this may still relate to
lymphangioleiomyomatosis. Interstitial lung disease (may be usual
 interstitial pneumonia, UIP) is also considered.

5. Marked interval regression of right upper and middle lobe

ground glass opacities, ascribed to pulmonary edema changes or
viral pneumonia

6. Subsegmental atelectasis and/or fibrosis in both lungs.

7. Cardiomegaly (bilateral atrial enlargement); pulmonary arterial

hypertension

8. Calcified mitral and aortic valves.

9. Atherosclerotic vessel disease.

10. No enlarged mediastinal or hilar lymph nodes:

1/16/23 CXR -Cardiomegaly with early signs of pulmonary congestive changes

-Right-sided pleural effusion, ultrasound correlation is

recommended for further evaluation

-Concomitant pneumonia is also considered

-Atherosclerotic aorta

-Degenerative osseous changes

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.

-There is gross evidence of significant free pleural flid in the left

hemithorax.

IMPRESSION:

RIGHT -SIDED PLEURAL EFFUSION WITH FLOATING DEBRIS AND

ASSOCIATED CONSOLIDATION/ATELECTASIS OF ADJACENT LOWER
LOBE

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.

-Heart size cannot be properly ascertained. Cephalization of the

pulmonary vasculature are noted. Calcific foci line the aorta.
 -Trachea is midline.

-Spurs are seen in the endplates of the thoracic vertebral bodies.

-The previously reported thoracostomy tube is no longer

appreciated in the present study. A feeding tube is also seen in
place with its tip cut off from the field of view.

IMPRESSION:

-PULMONARY CONGESTION AND BILATERAL PLEURAL EFFUSION

WITH SIGNS OF DISEASE PROGRESSION.

-CONCOMITANT PNEUMONIA ALSO CONSIDERED.

-ATHEROSCLEROTIC AORTA

-DEGENERATIVE OSSEOUS CHANGES

1/30/23 Chest UTZ -Reference was made to a previous chest UTZ dated Jan. 18, 2023.

-Scanning of both hemithoraces were done while the patient is in

high-sitting position.

-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.

-No loculations or septations seen in both hemithoraces.

IMPRESSION:

BILATERAL PLEURAL EFFUSION, MORE IN THE RIGHT

2/1/23 CXR Clinical data: status post closed tube thoracostomy

-Follow examination since January 28, 2023, now shows a

thoracostomy tube in place with its tip projected medially at the
level of the right 5th intercostal space. No pneumothorax or
pneumomediastinum noted.

-There is interval decrease in the ground glass and hazy opacities

seen in both lungs; still obscuring the cardiac borders,
hemidiaphragms and costophrenic sulci.

-Heart size cannot be properly ascertained. Pulmonary vascular

markings are not attenuated. Aorta is atherosclerotic.

-Trachea is midline.
 Degenerative osseous changes remains seen.

-No other remarkable interval findings.

II: Hematology:

CBC: Did we investigate the cause of the anemia?

CBC 1/18/2023 1/30/2023 2/2/2023 Reference Range
Hgb 10.6 (L) 9.6 (L) 9.7 (L) 12.00-16.00 g/dL
Hct 33.1 (L) 31.3 (L) 32.1 (L) 38 – 47%
RBC 3.97(L) 3.72(L) 3.78 4 – 6 x 10^6 u/L
MCV 83.4 84.1 84.9 80 – 100 fL
MCH 26.7 25.8 (L) 25.7 (L) 26 – 34 pg
MCHC 32 30.7 (L) 30.2 (L) 31 – 37 g/dL
RDW 15.9 16.1 (H) 15.9 11 – 16%
WBC 6.53 8.93 10.36 4.5 – 11 x 10^3/gL
Neutrophils 70.2(H) 74.5(H) 78.4 (H) 50 – 70%
Lymphocytes 14.5(L) 11.9 (L) 10.1 (L) 20 – 40%
Monocytes 9.3 (H) 9.8 9.8 0 – 7%
Eosinophils 5.7 (H) 1.4 1.2 0 – 5%
Basophils 0.3 0.3 0.5 0 – 1%
Platelet Ct. 379 446(H) 491 (H) 150 – 400 x 10^3/uL

III. Chemistry:

Blood Chemistry 1/18/23 1/30/23 Normal value

Creatinine 68 umol/L 51 umol/L 45.00-84.00 umol/L

eGFR 72 mL/min/1.73m^2 85 mL/min/1.73m^2 60-120
mL/min/1.73m^2
Na 133 mmol/L (L) 134 mmol/L (L) 136-145 mmol/L

K 5.1 mmol/L 4.70 mmol/L 3.50- 5.10 mmol/L

Cl 97.0 mmol/L (L) 87 mmol/L (L) 98-107 mmol/L

Protein 63 g/L 62-81 g/L (above 60 yo)

Albumin 30 g/L (L) 32-46 g/L

Globulin 33.00 g/L

A/G ratio 0.91

RBS 118 mg/dL

 High Sensitive 0.074 ng/ml Female: less than 0.011
Troponin I ng/ml
Procalcitonin <0.05 <0.50 ng/ml

SGPT/ALT 32 U/L 0.00-55.00 U/L

SGOT/AST 31.9 U/L 0.00-33.00 U/L

C-Reactive 12 mg/L NEGATIVE: Less than 6

Protein mg/L

Make sure to compute for PF to serum ratios and interpret based on Light’s criteria

LDH 02/1/2023 2/2/2023 Normal Value

Serum LDH 181.00 120-240 U/L

Pleural LDH 80 U/L

Pleural Protein 02/1/2023

Pleural Protein 28 g/L

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

Microoganism/s Isolated: Stenotrophomonas malthophilia

Sensitive Ceftazidime, Cotrimoxazole, Levofloxacin
Intermediate Chloramphenicol

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)

Pleural Fluid Diff Count and Cell Count (2/1/23)

Appearance before Hazy, yellow fluid Cells (Total) 1,773 cu.mm
centrifugation Red Blood Cells 1,600 /cu.mm
Appearance after Clear, yellow fluid with White Blood Cells 173/cu.mm
centrifugation red cell button Lymphocytes 0.70
Neutrophils 0.30

Pleural Fluid Cytology and Cell Block (2/3/23)

Gross Description:
Specimen received for cytospin and cell block labeled “Pleural fluid”, consists of approximately 80 ml
of amber-colored turbid fluid
Microscopic Description:
Cytospin and cell block show many benign and reactive mesothelial cells, neutrophils, lymphocytes
 and red cells in a fibrinous background. No malignant cells seen.
CYTOLOGIC DIAGNOSIS:
ACUTE ON CHRONIC INFLAMMATORY PATTERN WITH REACTIVE MESOTHELIAL CELLS

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)

I think we can rephrase this to:

Spontaneous pneumothorax, right from cystic lung disease (Lymphangioleiomyomatosis)
Bilateral pleural effusion from 1. Parapneumonic, 2. Diffuse cystic lung disease
s/p CTT insertion (December 2022)
Heart failure, functional class II-III from 1. Severe mitral stenosis, 2. HASCVD in permanent AF
Anemia from 1. Chronic disease, 2. Infection/Inflammation, 3. r/o nutritional (mababa ang MCH
MCHC)
s/p CVD infarct x 2 (2016, 2022), no residuals
Degenerative osteoarthritis, thoracolumbar spine
Renal cyst, right

Course in the Wards

Day of Admission (1/19/23):

Patient seen at the ER not in distress with the following vital signs: BP 160/60 mmHg, HR 64 bpm, RR 21
cpm, T 36.4oC, O2 sat: 97% at room air. On PE, GCS 15, awake, conscious, coherent, not in distress, (+)
pale palpebral conjunctiva, (+) bilateral crackles mid to base, (+) decreased breath sounds more on the
right, with irregular rhythm and (+) bipedal pitting grade 1 edema. Patient was hooked to O2 support at
2 LPM via NC. Laboratories were requested such as CBC with Hgb 10.6, Hct 33.1, WBC 6.53, Neutrophils
70.2, Lymphocytes 14.5, Platelet 379; ECG showed AF in SVR, LVH with strain pattern and inferior wall
ischemia; high sensitive Troponin I of 0.074 (which is 6.7x elevated); creatinine of 68 with eGFR of 72; Na
of 133, K of 5.1 and Cl of 97. Patient was maintained on HL and previous oral feeding with Ensure Gold
with HMB 1200 kcal (1.5: 1dilution) in 6 equal feedings was continued. Patient was then admitted to
Cardiologist-of-choice where the following medications were continued: Telmisartan + Amlodipine 80
mg/5 mg 1 tab OD, Nebivolol 5 mg 1 tab OD, Digoxin (Lanoxin) 0.25 mg ½ tab OD, Rosuvastatin 20 mg 1
tab ODHS, Mosegor Vita 1 cap BID, Symbicort rapihaler 160 mcg/4.5 mcg 2 puffs BID, Apixaban 2.5 mg
tab BID and Levetiracetam 500 mg tab BID. Patient was referred to Pulmonologist-of-choice, where
chest UTZ and serum procalcitonin were requested. Started on NAC 600 mg tab ODHS for productive
cough. Advised to retrieve copy of CT scan and CXR previously done from other institution.

1st Hospital Stay (1/19/23):

Patient was noted to have increased purulent secretions. Sputum GS/CS was requested and NAC was
increased to BID. Started on Piperacillin-Tazobactam 4.5 g IV Q8H (last antibiotics given on previous
admission are Cefuroxime and Levofloxacin) and Duavent nebulization Q12H followed by chest
physiotherapy. Maintained on O2 support at 2 LPM via NC.

2nd to 3rd Hospital Stay (1/20-21/23):

Patient had fever with a Tmax of 38.1 and was given Paracetamol 500 mg tab for fever. For Blood CS x2
sites if with recurrence of fever. O2 support was decreased to 1 LPM via NC which the patient tolerated.
Chest UTZ revealed right-sided pleural effusion with floating debris and associated
consolidation/atelectasis of adjacent lower lobe.

4th Hospital Stay (1/22/2023):

Patient still had cough but decreased in severity, denied dyspnea and chest pain. Azithromycin 500 mg 1
tab OD was started. Continued O2 support at 1 LPM with no desaturations noted.

6th Hospital Stay (1/24/2023):

Sputum CS result was released which revealed growth of multiple drug resistant organism Klebsiella
oxytoca which is resistant to current antibiotic used (Piperacillin-Tazobactam) and Stenotrophomonas
malthophilia. Azithromycin and Piperacillin-Tazobactam was then discontinued. Patient was then
referred to Infectious Disease Specialist.

7th – 8th Hospital Stay (1/25/2023):

Patient was seen by IDS service and was started on Ceftazidime + Avibactam 2.5 g IV Q8H to infuse for 2
hours. Repeat CBC, procalcitonin and CRP was requested in next blood extraction. NGT was accidentally
removed by patient. Pulmonology service suggested referral to GI service for possible PEG insertion
once patient is more stable. Patient was then referred to GI service for evaluation for possible PEG
insertion

9th Hospital Stay (1/27/2023):

Patient cleared for PEG insertion by different services. She was seen by GI service and suggested
evaluation by occupational therapy for dysphagia testing. PEG insertion was temporarily put on hold
because patient claimed that she was able to swallow and family was still undecided with the procedure.

10th -11th Hospital Stay (1/28-29/23):

Relative claimed that JP drain was accidentally removed by caregiver upon bed turning. Patient was
referred due to difficulty of breathing with RR of 24 cpm and O2 sat at 98% at 1 LPM. On PE, noted to
have distended neck vein, bilateral crackles and decreased breath sounds on bilateral lung field.
Furosemide 40 mg IV was then given. STAT repeat CXR was done which showed interval increase in the
ground glass opacities and veil of haziness seen in both lungs, obscuring the cardiac borders,
hemidiaphragms and costophrenic sulci. Repeat Chest UTZ was done which showed interval increase in
the free pleural fluid seen in the right hemithorax with an estimated volume of 819 ml (previously 380
ml) and interval development of free pleural fluid in the left hemithorax with an estimated volume of
286 ml. PEG Insertion was temporarily put on hold since patient was not stable. O2 support was
increased to 3 LPM via NC. Cardio service started Furosemide 40 mg tab OD via NGT.

12th Hospital Stay ( 1/30/2023):

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.

14th Hospital Stay (2/1/2023):

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.

15-16th Hospital Stay (2/2-3/2023)

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.

17th Hospital Stay (2/4/2023)

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:

Hospital-acquired pneumonia (S. maltophilia), resolved

Bilateral pleural effusion, resolving, from 1. Parapneumonic, 2. Diffuse cystic lung disease
(lymphangioleiomyomatosis)
Spontaneous pneumothorax, right, resolved, from cystic lung disease (lymphangioleiomyomatosis)
s/p CTT insertion (December 2022)
s/p CTT insertion (February 1, 2023)
Heart failure, functional class II-III from 1. Severe mitral stenosis, 2. HASCVD in permanent AF
Anemia from 1. Chronic disease, 2. Infection/Inflammation, 3. r/o nutritional (mababa ang MCH
MCHC)
s/p CVD infarct x 2 (2016, 2022), no residuals
Degenerative osteoarthritis, thoracolumbar spine
Renal cyst, right

Final Impression:

-Pleural Effusion, Bilateral sec to Parapneumonic Process and Lymphangioleiomyomatosis, Resolving

-CAP-MR sec to MDRO Infection, Resolved
-Permanent AF
-HASCVD
- Valvular Heart Disease, Severe Mitral Stenosis

Thoughts:

 What was the patient’s home medication regimen?

 Any plans for pulmonary rehab?

Overall:

 Differentials based on the patient’s presentation

 Discuss approach to diagnosis and management
 Was this sporadic versus genetic? Why and why not?
 What are other comorbidities we need to screen and management
 What are the short- and long-term complications? How will we address these?
 Do we have local data in the Philippines?