Physical Medicine and Rehabilitation Role in Pre-Operative

Management of Patient with Giant Bullae of Right Lung

with Scoliosis and a History of Systemic Lupus Erythematosus:

A Case Report

Andreas Ricky

, Tresia Fransiska U Tambunan

Department of Physical Medicine and Rehabilitation, Dr. Cipto Mangunkusumo Hospital,

University of Indonesia, Jakarta, Indonesia

Cardiorespiratory Division, Department of Physical Medicine and Rehabilitation, Dr. Cipto Mangunkusumo Hospital,

University of Indonesia, Jakarta, Indonesia

Keywords: Giant Bullae, Systemic Lupus Erythematosus, Pre-operative Management, Pulmonary Rehabilitation

Program.

Abstract: Giant bullae refers to the enlargement of one or more bullae occupying more than one-third of the

hemithorax. It creates larger and less efficient lung sacs that can’t properly manage the normal gas exchange

during breathing. Systemic Lupus Erythematosus (SLE) is a chronic inflammatory disease with pulmonary

manifestations is giant bullae due to excessive surface tension secondary to surfactant failure. Female

patient with history of SLE presenting with shortness of breath during moderate-intensity activities. CT

Scan showed giant emphysematous bullae in her right hemithorax. Patient had double curve scoliosis which

may affect her chest expansion. Evaluation of respiratory functions showed lung restrictive disease and its

predictive post-operative value of forced expiratory volume in one second (ppoFEV1) below 30%. During 8

weeks pulmonary rehabilitation program patient’s clinical state is improved and associated with increase in

forced expiratory volume in one second (FEV1). Home based aerobic exercise consisted of walk 15

minutes/day, 5 days a week. Due to her limited chest expansion, active breathing exercise and scoliosis

program exercise administered in the rehabilitation program. This case report elaborates on the effect of the

pre-operative pulmonary rehabilitation program in improving respiratory function and its readiness to

undergo surgical treatment.

1 INTRODUCTION

Giant bullae, referred to as vanishing lung syndrome

as a clinical syndrome, characterised by large bullae

that occupying at least one-third of one or both

hemithoraces (Huang et al., 2014; Garg et al., 2016).

Emphysema in giant bullae causes a loss of

elasticity in the walls of the small air sacs in the

lung. Eventually, the walls of the sacs stretch and

break, which creates larger, less efficient sacs that

can’t properly handle the normal exchange of

oxygen and carbon dioxide that occurs during

breathing. Difficulty in fully exhaling usually leads

to the capture of air in the lungs, known as

hyperinflation (Giant Bullae. Health Encyclopedi,

2019).

SLE is a chronic inflammatory disease of

autoimmune origin that can affect virtually every

organsystem of the human body (Lopez Velazquez

and Highland, 2018). Pulmonary manifestations of

SLE include airway disease, pleuritis (with or

without effusion), inflammatory and fibrotic forms

of interstitial lung disease (ILD), alveolar

hemorrhage, acute lupus pneumonitis (ALP),

pulmonary hypertension, giant bullae and

thromboembolic disease.

Pulmonary manifestations in systemic lupus

erythematosus (SLE) are relatively common but

giant bullae is the least common clinical

manifestation. Fewer than 100 cases have been

reported in the medical literature. Although giant

bullae can be asymptomatic, patients are usually

experience dyspnea caused by underlying

172

Ricky, A. and Tambunan, T.

Physical Medicine and Rehabilitation Role in Pre-Operative Management of Patient with Giant Bullae of Right Lung with Scoliosis and a History of Systemic Lupus Erythematosus: A Case

Report.

DOI: 10.5220/0009087501720176

In Proceedings of the 11th National Congress and the 18th Annual Scientiﬁc Meeting of Indonesian Physical Medicine and Rehabilitation Association (KONAS XI and PIT XVIII PERDOSRI

2019), pages 172-176

ISBN: 978-989-758-409-1

emphysema. Surgical resection is treatment of

choice for patients with giant bullae. The goals are

to improve the quality of life for those in whom

medical treatment has failed and to resolve

complications while preserving lung function.

Surgical resection is considered only after an

assessment of exercise capacity, pulmonary-function

testing, and smoking cessation. Determination of the

preoperative bulla volume allows the prediction of

the expected increase of postoperative FEV1 (Giant

Bullae. Health Encyclopedia, 2019).

Based on the recommendations of the Enhanced

Recovery After Surgery (ERAS®) and the European

Society of Thoracic Surgeons (ESTS) pulmonary

rehabilitation and prehabilitation is strongly

recommended and so is perioperative nutrition

screening and oral nutritional supplements

(Batchelor, 2019). We report a case of a patient with

giant bullae, scoliosis, and SLE whose pulmonary

function improved significantly after following

prehabilitation program.

2 CASE PRESENTATION

A 43-year-old woman, a nonsmoker presented in

August 2019 with shortness of breath during

moderate-intensity activities. Patient has a history of

giant bullae of her right lung which was diagnosed

on January 2019. Patient started to experience

shortness of breath while conducting regular

activities which gradually worsened. She would feel

shortness of breath after climbing one flight of stairs

although she could still walk 500 meters without the

symptom. Patient also began experiencing fever.

The fever was accompanied with frequent vomiting

and non-productive cough. A chest radiograph (Fig.

1) revealed increased lucency in the upper half of the

right hemithorax suspected as several loculated

pleural effusions. This was followed by a chest

computed tomography (CT) scan that revealed a

giant bullae occupying her right lung accompanied

by pleural effusion on both bases of her lungs.

(Fig.2).

Figure 1: Chest radiograph suspect loculated pleural

effusion in right upper zone, with increased interstitial

markings in lower zone.

Figure 2: CT chest – Giant bullae in right lung, with

loculated pleural effusion and calcified pleura at basal

right lung.

Physical Medicine and Rehabilitation Role in Pre-Operative Management of Patient with Giant Bullae of Right Lung with Scoliosis and a

History of Systemic Lupus Erythematosus: A Case Report

173

Patient has a history of SLE that was first

diagnosed in the year 2000. Afterwards put her in

routine medication (methylprednisolone, myfortic).

In 2006 she began experiencing back pain that

gradually worsened over the time and diagnosed

with osteoporosis. Patient also took kolkatriol and

CaCO3 once a day. The patient denied any history

of tuberculosis, diabetes mellitus type 2, stroke, and

cardiovascular illnesses.

The patients social history; The patient is a

housewife. She was a never smoker, but had

exposure to environmental tobacco smoke. She did

have any exposure to biological or industrial dusts.

Therewas no family history of respiratory illnesses.

On examination, she had a heart rate of 99 beats

per minute, and a blood pressure of 140/94. The

respiratory rate was 21 breaths per minute, with an

oxygen saturation of 97% on ambient air. She was of

small build with BMI 16,4 (underweight).

Respiratory examination revealed a vesicular breath

sounds, with reduced sound on the right hemithorax,

no rhonchi, no wheezing. Hypersonor was found in

lateral right hemithorax during chest percussion. She

had thoracoabdominal breathing pattern, with

contraction of inspiratory accessory muscle, slightly

delayed chest expansion on right hemithorax, and

inadequate effort to cough. Chest expansions were

decreased, with a difference between inspiration and

expiration of 5.

In postural examination, patient left shoulder is

slightly higher than the right, vertebral alignment S-

shaped with convexities on left mid-thoracic region

and right thoracolumbar region, pelvic obliquity

positive with right side higher than the left, very

slightly observable hump on right mid-thoracic

region of the vertebrae. Spasm paravertebral muscles

was found on the concave side. Schober test is 15 +

4 cm, and with Adams test showed a functional

scoliosis with hump (height 1 cm) on right side of

mid-thoracic region (T6) 6 cm from midline.

Patient was consulted for a rehabilitation

program as the patient is waiting for surgery. Pre-

operative management aimed to increase

cardiorespiratory endurance, strengthening of

diaphragm muscle, increase chest wall mobility,

reduce muscle imbalance due to scoliosis, energy

conservation, and improve nutritional status.

Table 1: Baseline lung function and capacity test.

Chest Expansion 5cm – 5 cm – 6cm

PFR 2.70 L/s

FVC 1.09 L (32% pred)

FEV1 1.00 L (36% pred)

FEV1/FVC 91.74%

6MWT

Mileage: 418 meter

(predicted mileage

514.2 meter) =

81.29%

Vo2Max: 16.8

METS: 4.80

Chest Expansion 5cm – 5 cm – 6cm

Table 2: Lung Function and capacity test after

rehabilitation program.

4 weeks 8 weeks

Chest

Expansion

5cm – 5 cm –

8cm

5cm – 5 cm –

8cm

PFR 3.31 L/s 3.74 L/s

FVC

1.9 L

(66% pred)

2.59 L

(86.9% pred)

FEV1

1.71 L

(69.5% pred)

2.20 L

(87% pred)

FEV1/FVC 90% 84.94%

6MWT

Mileage: 409

meter (predicted

mileage 518,6

meter) =

78.86%

Vo2Max: 15.4

METS: 4.4

Mileage: 465

meter (predicted

mileage 517

meter) = 89.2%

Vo2Max: 18.4

METS: 5.20

3 DISCUSSION

Giant bullae is a disorder characterized by bullae

occupying more than a third of the hemithorax, and

mostly affects men who are smokers (Kiang, Tan

and Li, 2015) In our case, although the she never

smoked a day in her life, the patient was exposed to

cigarette smoke since childhood as her late father

was a heavy smoker. In addition, nowadays she

lives by a garbage disposal site that burns garbage

on a daily basis. Thus, it may not be unreasonable to

assume that different triggers incite the same

cascade of subcellular inflammatory mediators that

cause the destruction of alveolar walls and resulting

in permanent and abnormal enlargement of distal

airspaces. Interestingly, GB in our patient may have

and associations with medical conditions (and

systemic lupus erythematous), (Kiang, Tan and Li,

2015)

Enlarging bullae cause symptoms by interfering

with respiratory mechanics and gas exchange. As

they grow larger, they compress on normal lung

parenchyma, reduce lung compliance, and increase

work of breathing. As dead space fraction increases

with bullae formation, gas exchange is also impaired

This symptom similar in our setting that showed

KONAS XI and PIT XVIII PERDOSRI 2019 - The 11th National Congress and The 18th Annual Scientiﬁc Meeting of Indonesian Physical

Medicine and Rehabilitation Association

174

dyspnea was the main problem, and pulmonary

functional study showed restrictive lung disease.

Patient with single giant bulla with underlying

normal lung is the “ideal candidates” and stand the

best chance of success of surgery. Success is defined

as both a lessening of the pressure and other

symptoms, and the recovery or restoration of lung

function.(Giant Bullae. Health Encyclopedia, 2019)

It is still uncertain whether the patient in this case

falls in this category. Even with a single giant bulla

and remaining lungs mostly in normal condition, the

CT result showed a fibrosis in segment 9 of left lung

accompanied by pleural effusion. In addition the

single giant bulla was formed by combination of

multiple giant bulla existed in the right lung with the

size of approximately 11.4 x 6.7 x 17.6 cm3.

Before planned surgical treatment of lung cancer,

the patient's respiratory system function should be

evaluated. According to the current guidelines (fig

3), the assessment should start with measurements of

FEV1 (forced expiratory volume in 1 second) and

DLco (carbon monoxide lung diffusion capacity).

Pneumonectomy is possible when FEV1 and DLco

are > 80% of the predicted value (p.v.). If either of

these parameters is < 80%, an exercise test with

VO2 max (oxygen consumption during maximal

exercise) measurement should be performed. When

VO2 max is < 35 % p.v. or < 10 ml/kg/min,

resection is associated with high risk. If VO2 max is

in the range of 35-75% p.v. or 10-20 ml/kg/min, the

postoperative values of FEV1 and DLco (ppoFEV1,

ppoDLco) should be determined. The exercise test

with VO2 max measurement may be replaced with

other tests such as the shuttle walk test and the stair

climbing test. The distance covered during the

shuttle walk test should be > 400 m. Patients

considered for lobectomy should be able to climb 3

flights of stairs (12 m) and for pneumonectomy 5

flights of stairs (22 m).(Trzaska-Sobczak, M

Skoczyński and Pierzchała, 2014)

Figure 3: Algorithm for assessing respiratory system

function in lung cancer surgery candidates.

In this patient the ppoFEV1 was 15 pv and

ppoVO2 max was 6.73 ml/kg/min which means

surgical treatment was not advised and other therapy

was recommended. In 21 studies (including 5 RCTs)

focusing on pre- rather than postoperative

rehabilitation, the intervention was delivered mainly

in the outpatient setting or in a training facility.

Prescribed exercises included aerobic training (lower

and/or upper limbs), with the addition of strength

training in some studies. Respiratory exercises were

also included in the majority of studies. The addition

of other elements, such as relaxation techniques and

educational sessions, were inconsistent. The median

duration was 4 weeks (range 1–10 weeks) with a

frequency of 5 sessions per week (range 2–

14 weeks) of moderate to high intensity, generally

tailored to the patient’s tolerance.(Batchelor, 2019)

This patient receives pre-operative pulmonary

rehabilitation program in outpatient setting which

includes: (1) aerobic exercise 5 times a week, (2)

respiratory exercises, and (3) exercise program for

scoliosis. Aerobic exercise prescribed for moderate

intensity although in reality the patient was allowed

to do them as tolerated. Based on her 6-minute-

walking-test result which was 418 meters in 6

minutes (VO2 max 16.8, METs: 4.80), we decided

to prescribe her aerobic exercise starting from 50%

of the maximum walking distance from her 6MWT,

thus ideally prescribing the patient with 30 minutes-

walk (with the same pace as when she walked the

6MWT) for 3 days a week. However, as the patient

has had a history of SLE with multiple

musculoskeletal manifestation in addition to her

sedentary lifestyle, we made the decision to

prescribe her aerobic exercise that started low and

progress slowly starting with walking for 15

minutes, 5 days a week, instead.

As for her respiratory exercise, the patient

receives a set of exercise that consisted of

diaphragmatic breathing, deep breathing, chest

expansion, and pursed lip breathing. Aside from

relaxation effect the exercise may give to the patient

each one was prescribed for different reasons. As

the right hemithorax could not expand

symmetrically with the left one, thoracic expansion

could be assisted by excursion of the diaphragm and

flexing and abducting the upper extremities. If the

patient were to follow through with surgery later on,

the patient would be prepared with diaphragm and

intercostal muscles that has good strength and

flexibility. She would also benefit from the

relaxation breathing when experiencing post-

surgical pain. While pursed lip breathing helps in

airway clearance as the patient has limited

Physical Medicine and Rehabilitation Role in Pre-Operative Management of Patient with Giant Bullae of Right Lung with Scoliosis and a

History of Systemic Lupus Erythematosus: A Case Report

175

inspiratory capacity. In addition, patient was also

prescribed 2 session of chest physical therapy

aiming to educate her chest mobility exercise.

Moreover, a TheraBand strengthening exercise is

also planned to strengthen her pectoralis mayor and

core muscles that may help with overall recovery.

Scoliosis exercise programs were aimed to

correct the muscle imbalance. It consist of (1)

strengthening weakened muscles on the convex side,

(2) stretching of muscle spasm on concave side with

6 seconds hold, 10 repetitions, 3-5 times/day.

ESPEN guidelines recommend delaying surgery to

allow for preoperative enteral nutrition in patients

with at least one of the following criteria: weight

loss >10–15% within 6 months, body mass index

(BMI) <18.5 kg/m2 and serum albumin <30 g/l (with

no evidence of hepatic or renal dysfunction).

Current general recommendations suggest

administration of 5–7 days of oral supplements

before surgery in patients at risk of

malnutrition.(Batchelor, 2019)

In this case the patient’s BMI falls less than 18.5

kg/m2, and there hasn’t been any lab work up to rule

out hepatic nor renal dysfunction considering

patient’s history of SLE. Patient is planned to be

consulted to nutritionist regarding her perioperative

nutrition preparation. 4 weeks after home-based

program the chest expansion is increase in lower

segment. And also increasing in pulmonary function

test, ppoFEV1 29 pv, and ppoV02max 6.16

ml/kg.min. After 8 weeks of program FEV1 is

increased to 2.20 L (87% pred), so does the

ppoFEV1 (36 pv) and ppoVo2max (7.37 ml/kg.min)

(table 2). Although patient still was not advised to

undergo surgical treatment, better pulmonary

function already help patient to do her daily activity.

Patient right now can climb 2 flights of stairs

without feeling any shortness of breath, and can do

housework without any symptoms.

4 CONCLUSIONS

Pulmonary rehabilitation can be given to patient

with giant bullae in pre-operative setting. This will

lead to better cardiorespiratory endurance,

respiratory function, and better endurance to do her

daily activity. Pre-operative pulmonary

rehabilitation program should be given to the giant

bullae patient to increase her readiness to undergo

surgical treatment.

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10.1016/j.chest.2016.08.1029.

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KONAS XI and PIT XVIII PERDOSRI 2019 - The 11th National Congress and The 18th Annual Scientiﬁc Meeting of Indonesian Physical

Medicine and Rehabilitation Association

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