학술논문
Surgical creation of lymphocutaneous fistulas for the management of infants with central lymphatic obstruction.
Document Type
Academic Journal
Author
Laje P; Division of General, Thoracic and Fetal Surgery, The Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, HUB Building-Suite 2527, Philadelphia, PA, 19104, USA. laje@chop.edu.; Jill and Mark Fishman Center for Lymphatic Disorders, The Children's Hospital of Philadelphia, Philadelphia, PA, USA. laje@chop.edu.; Smood B; Division of Cardiovascular Surgery, Department of Surgery, The Hospital of the University of Pennsylvania, Philadelphia, PA, USA.; Division of Cardiothoracic Surgery, Department of Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.; Smith C; Jill and Mark Fishman Center for Lymphatic Disorders, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.; Department of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.; Pinto E; Division of General, Thoracic and Fetal Surgery, The Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, HUB Building-Suite 2527, Philadelphia, PA, 19104, USA.; Jill and Mark Fishman Center for Lymphatic Disorders, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.; Krishnamurthy G; Jill and Mark Fishman Center for Lymphatic Disorders, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.; Department of Radiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.; Taha D; Division of Neonatology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.; Dori Y; Jill and Mark Fishman Center for Lymphatic Disorders, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.; Department of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.; Maeda K; Jill and Mark Fishman Center for Lymphatic Disorders, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.; Division of Cardiovascular Surgery, Department of Surgery, The Hospital of the University of Pennsylvania, Philadelphia, PA, USA.; Division of Cardiothoracic Surgery, Department of Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Source
Publisher: Springer International Country of Publication: Germany NLM ID: 8609169 Publication Model: Electronic Cited Medium: Internet ISSN: 1437-9813 (Electronic) Linking ISSN: 01790358 NLM ISO Abbreviation: Pediatr Surg Int Subsets: MEDLINE
Subject
Language
English
Abstract
Purpose: Central lymphatic obstructions are associated with anasarca and high mortality. We hypothesized that opening dilated cutaneous lymphatic channels by creating a lymphocutaneous fistula (LCF) would decompress the lymphatic circulation and improve anasarca.
Methods: We reviewed all patients that had at least one LCF created between 9/2019 and 12/2022. LCF efficacy was determined by changes in weight, urine/diuresis, ventilation, and clinical status.
Results: We created eleven LCFs in four infants. LCFs initially drained 108 cc/kg/d (IQR68-265 cc/kg/d). Weights significantly decreased after LCF creation (6.9 [IQR6.1-8.1] kg vs. 6.1 [IQR 4.9-7.6] kg, P = 0.042). Ventilatory support decreased significantly in all patients after at least one LCF was created, and 3/4 patients (75%) had significantly lower peak inspiratory pressures (28 [IQR 25-31] cmH2 O vs. 22 [IQR 22-24] cmH 2 O, P = 0.005; 36 [IQR36-38] cmH 2 O vs. 33 [IQR 33-35] cmH 2 O, P = 0.002; 36 [IQR 34-47] cmH 2 O vs. 28 [28-31] cmH 2 O, P = 0.002). LCFs remained patent for 29d (IQR 16-49d). LCFs contracted over time, and 6/11 (54.5%) were eventually revised. There were no complications. Two patients died from overwhelming disease, one died from unrelated causes, and one remains alive 29 months after their initial LCF.
Conclusion: LCFs provide safe and effective temporary lymphatic decompression in patients with central lymphatic obstruction. While LCFs are not a cure, they can serve as a bridge to more definitive therapies or spontaneous lymphatic remodeling.
Level of Evidence: IV.
(© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Methods: We reviewed all patients that had at least one LCF created between 9/2019 and 12/2022. LCF efficacy was determined by changes in weight, urine/diuresis, ventilation, and clinical status.
Results: We created eleven LCFs in four infants. LCFs initially drained 108 cc/kg/d (IQR68-265 cc/kg/d). Weights significantly decreased after LCF creation (6.9 [IQR6.1-8.1] kg vs. 6.1 [IQR 4.9-7.6] kg, P = 0.042). Ventilatory support decreased significantly in all patients after at least one LCF was created, and 3/4 patients (75%) had significantly lower peak inspiratory pressures (28 [IQR 25-31] cmH
Conclusion: LCFs provide safe and effective temporary lymphatic decompression in patients with central lymphatic obstruction. While LCFs are not a cure, they can serve as a bridge to more definitive therapies or spontaneous lymphatic remodeling.
Level of Evidence: IV.
(© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)