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Table 1 Baseline characteristics of surgical patients with lymphangioleiomyomatosis

From: Intraoperative mechanical ventilation and incidence of pneumothorax in lymphangioleiomyomatosis

Variables

All patients

(n = 12)

PP group

(n = 4)

nPP group

(n = 8)

Age (y)

41 ± 10

37 ± 9

43 ± 11

Diagnosis

   

 TSC-LAM

1 (8.3)

1 (25.0)

0 (0.0)

 Sporadic-LAM

11 (91.7)

3 (75.0)

8 (100.0)

Preoperative variables

   

CT gradea

   

 I

5 (45.5)

0 (0.0)

5 (62.5)

 II

1 (9.1)

1 (33.3)

0 (0.0)

 III

5 (45.5)

2 (66.7)

3 (37.5)

VEGF-D (pg/mL)b

1145 ± 832

1208 ± 528

1119 ± 971

FEV1%pred (%)c

77.2 ± 25.8

69.5 ± 7.8

79.1 ± 28.7

DLco%pred (%)d

60.2 ± 29.7

36.0 ± 15.6

66.3 ± 29.9

6MWD (m)e

501 ± 69

491 ± 91

505 ± 66

PaO2 (mmHg)

80.1 ± 13.5

71.4 ± 10.2

84.5 ± 13.3

SpO2 (%)

96 ± 4

93 ± 6

98 ± 2

Preoperative PTX

6 (50.0)

3 (75.0)

3 (37.5)

History of pleurodesis

2 (16.7)

0 (0.0)

2 (25.0)

Menstruation status

   

 Pre-menopausal

9 (75.0)

3 (75.0)

6 (75.0)

 Peri-menopausal

1(8.3)

1 (25.0)

0 (0.0)

 Post-menopausal

2 (16.7)

0 (0.0)

2 (25.0)

Current pregnancy

4 (33.3)

2 (50.0)

2 (25.0)

Preoperative mTOR inhibitors therapy

3 (25.0)

0 (0.0)

3 (37.5)

Intraoperative variables

   

Surgical type

   

 VATS lobectomy or pleurodesis

6 (50.0)

2 (50.0)

4 (50.0)

 Partial nephrectomy

1 (8.3)

0 (0.0)

1 (12.5)

 Hysterectomy and bilateral salpingoophorectomy

1 (8.3)

0 (0.0)

1 (12.5)

 Cesarean section

4 (33.3)

2 (50.0)

2 (25.0)

Surgery duration (min)

79 ± 40

79 ± 28

79 ± 47

Timing of surgery

   

 Emergent

1 (8.3)

1 (25.0)

0 (0.0)

 Elective

11 (91.7)

3 (75.0)

8 (100.0)

ASA physical status

   

 1

2 (16.7)

0 (0.0)

2 (25.0)

 2

5 (41.7)

1 (25.0)

4 (50.0)

 3

4 (33.3)

2 (50.0)

2 (25.0)

 4

1 (8.3)

1 (25.0)

0 (0.0)

Anesthetic technology

   

 General anesthesia

8 (66.7)

3 (75.0)

5 (62.5)

 Regional anesthesia

4 (33.3)

1 (25.0)

3 (37.5)

Anesthesia duration (min)

119 ± 44

122 ± 24

117 ± 53

Mechanical ventilation

8 (66.7)

3 (75.0)

5 (62.5)

 Ventilation duration (min)

1324 ± 3250

3208 ± 5328

194 ± 173

 Mean peak airway pressure (mmHg)

23 ± 4

22 ± 6

23 ± 3

 Respiratory rate

14 ± 4

17 ± 6

13 ± 2

 Back to ICU without extubation

2 (16.7)

1 (25.0)

1 (12.5)

  1. The results are presented as the means ± standard deviations (SD) or n (%)
  2. PP group, patients developed postoperative pneumothorax; nPP group, patients did not develop postoperative pneumothorax; TSC, tuberous sclerosis complex; LAM, lymphangioleiomyomatosis; CT, computerized tomography; VEGF-D, vascular endothelial growth factor-D; FEV1, forced expiratory volume in 1 s; DLco, diffusing capacity for carbon monoxide; 6MWD, 6-min walking distance; PaO2, partial pressure of oxygen in arterial blood; SpO2, room air pulse oxygen saturation; PTX, pneumothorax; mTOR, mammalian target of rapamycin; VATS, video-assisted thoracoscopic; ASA, American Society of Anesthesiologists; ICU, intensive care unit
  3. a Sample size for CT grade was 11, with 3 in PP group and 8 in nPP group. CT grade is classified based on the proportion of cystic lesions in total lung. I: < 1/3, II: 1/3 – 2/3, III: > 2/3
  4. b Sample size for VEGF-D was 10, with 3 in PP group and 7 in nPP group
  5. c Sample size for FEV1%Pred was 9, with 2 in PP group and 7 in nPP group
  6. d Sample size for DLco%Pred was 9, with 2 in PP group and 7 in nPP group
  7. e Sample size for 6MWD was 11, with 3 in PP group and 8 in nPP group
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172