Table 1:
Patient Study Set (year of publication) Ref | No. Operatively Managed Patients | Case Profiles | |||
---|---|---|---|---|---|
| |||||
Clinical Manifestations | Lymphatic Imaging/Diagnostics | Surgical/Interventional Management | Documented Outcomes | ||
Study 1 (1978)13 | 1 | Chylothorax, failure to thrive | CXR, Autopsy | Ligation of thoracic lymph vessels | Death |
Study 2 (1980)14 | 1 | Perineal/genital lymphedema | C-LAG | Inguinal lymph node biopsy | New onset lymphatic symptoms |
Study 3 (1980)15 | 2 | Cardiac defects (n=2) | CXR | Cardiac surgery | New onset lymphatic symptoms (n=2) |
*Study 4 (1998) 7 | 1* | Chylous ascites; lymphedema; genital lymphorrhea | MRL, LAS, C-LAG | Abdominal LVA | Marked improvement |
Study 5 (2016)4 | 2 | Lymphedema (n=2); genital lymphorrhea (n=1) | LAS | Thoracic duct ligation (n=1), Scrotoplasty (n=1) | Death (n=1); Improvement with some persistent symptoms (n=1) |
Study 6 (2019)16 | 1 | Lymphedema, lymphocutaneous fistulae, chylocolporrhea | C-LAG, CT, LAS | Lymphatic lipidiol embolization | Resolution without recurrence at 12 months (n=1) |
Study 7 (2019)8 | 7 | Chylothorax (n=6); ascites (n=3) | C-LAG (n=7), MRL (n=6), dc-MRL (n=5) | Lymphatic embolization (n=6), LVA (n=1) | Death (n=2), Stable or Improved (n=4), Resolution (LVA, n=1) |
Study 8 (2020)17 | 1 | Cardiac defects; chylothorax | ICG, dc-MRL | Thoracic duct ligation, two cardiac surgeries | Death |
*Study 9 (2021) 6 | 2* | Lymphedema, ascites, genital lymphorrhea, lymphocutaneous fistula | CT, MRL, LAS, CXR | Inguinal LVA (n=1), lymphatic doxycycline sclerosis (n=1) | Improvement 20-yr post op (LVA, n=1), Stable (n=1) |
Study 10 (2021) 9 | 1 | Acute Chylothorax and Pulmonary Insufficiency | C-LAG, dc-MRL | LVA (thoracic duct) | Fluid overload (immediate), Resolution (1-year) |
CXR= Chest X-Ray; C-LAG = Conventional Oil-Contrast Lymphangiography; CT = Computerized Tomography; MRL = T2-Weighted Magnetic Resonance Lymphography; dc-MRL = Dynamic-Contrast (T1-Weighted) Magnetic Resonance Lymphography; LAS = Lymphoscintigraphy; ICG = Indocyanin Green Lymphography; LVA = Lympho-Venous Anastomosis