Scuglia Marianna, Conforti Andrea, Valfrè Laura, Totonelli Giorgia, Iacusso Chiara, Iacobelli Barbara D, Meucci Duino, Viggiano Milena, Fusaro Fabio, Diociaiuti Andrea, Morini Francesco, El Hachem May, Bagolan Pietro
Neonatal Surgery Unit, Medical and Surgical Department of the Fetus, Newborn, and Infant, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Congenital Esophageal Disorders Unit, Medical and Surgical Department of the Fetus, Newborn, and Infant, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Front Pediatr. 2021 Aug 18;9:709223. doi: 10.3389/fped.2021.709223. eCollection 2021.
Lymphatic malformations (LMs) are rare entities, sometimes difficult to treat, that may be life-threatening when intricately connected to airway structures. Invasive treatments are occasionally required, with sclerotherapy considered the treatment of choice and surgery as a second-line approach. The aim of the present study was to evaluate our multidisciplinary team experience in treating newborns affected by LMs requiring operative management, while defining early outcomes. Retrospective review of all consecutive patients admitted for LMs requiring operative management between January 2000 and January 2019. Patients were mainly characterized based on anatomical district of the LM (and further stratified based on the development of respiratory distress), need for tracheostomy, number of sclerotherapies, indication for surgery, and residual disease beyond the 1st year. Morbidity and mortality were also evaluated. Fisher exact test and Mann-Whitney test were used as appropriate. Statistical significance was set at < 0.05. Fifty-seven patients were included in the study, 36 with cervicofacial and/or mediastinal LMs and 21 with LMs of other anatomical districts. Due to the risk of developing respiratory distress at birth, patients with cervicofacial and/or mediastinal LMs were divided into two groups (8/36 group A vs. 28/36 group B). Group A patients are at higher risk for tracheostomy (7/8 group A vs. 1/28 group B, = 0.0001) and more often require surgical reduction of the residual lymphatic abnormality (5/8 group A vs. 4/28 group B, = 0.013). They also require sclerotherapies more often, but the difference is not statistically significant (8/8 group A vs. 19/28 group B, = 0.15). Patients with cervicofacial/mediastinal LMs frequently suffer from persistent residual disease beyond the 1st year of life, significantly more often in group A (7/8 group A vs. 12/28 group B, = 0.043). LMs are rare conditions with potential life-threatening behavior. Their intrinsic clinical complexity requires a multidisciplinary approach to the affected patients. Planning a long-term follow-up is essential because of the late-term problems those patients may experience.
淋巴管畸形（LMs）是一种罕见的疾病，有时难以治疗，当与气道结构复杂相连时可能危及生命。偶尔需要进行侵入性治疗，硬化疗法被认为是首选治疗方法，手术作为二线治疗方法。本研究的目的是评估我们多学科团队治疗需要手术治疗的新生儿淋巴管畸形的经验，同时确定早期治疗效果。回顾性分析2000年1月至2019年1月期间所有因淋巴管畸形需要手术治疗而连续入院的患者。主要根据淋巴管畸形的解剖部位（并根据呼吸窘迫的发生情况进一步分层）、气管切开术的需求、硬化治疗的次数、手术指征以及1年后的残留疾病情况对患者进行特征描述。还评估了发病率和死亡率。适当使用Fisher精确检验和Mann-Whitney检验。设定统计学显著性为<0.05。本研究纳入了57例患者，其中36例患有头颈部和/或纵隔淋巴管畸形，21例患有其他解剖部位的淋巴管畸形。由于出生时发生呼吸窘迫的风险，患有头颈部和/或纵隔淋巴管畸形的患者被分为两组（A组8/36例 vs. B组28/36例）。A组患者气管切开术的风险更高（A组7/8例 vs. B组1/28例，P = 0.0001），并且更常需要手术减少残留的淋巴异常（A组5/8例 vs. B组4/28例，P = 0.013）。他们也更常需要硬化治疗，但差异无统计学意义（A组8/8例 vs. B组19/28例，P = 0.15）。患有头颈部/纵隔淋巴管畸形的患者在1岁以后经常患有持续性残留疾病，A组更为显著（A组7/8例 vs. B组12/28例，P = 0.043）。淋巴管畸形是一种罕见疾病，具有潜在的危及生命的行为。其内在的临床复杂性需要对受影响的患者采取多学科方法。由于这些患者可能会出现晚期问题，因此规划长期随访至关重要。
