Primary Pulmonary Lymphatic Flow Disorder Associated Plastic Bronchitis

Lymphatic plastic bronchitis (PB) most commonly occurs in children with congenital heart disease as a result of secondary pulmonary lymphatic flow disorder (PLFD). However, PB caused by primary PLFD is rare. The clinical symptoms of lymphocytic PB are nonspecific; thus, the diagnosis may be long delayed, especially in the absence of chylothorax. We made a retrospective analysis of two children diagnosed with PB due to primary PLFD, in order to contribute to further understanding of these disorders. Patient 1, an eight-year-old boy, presented with chronic productive cough and expectorated milky-white mucous plugs accompanied by intermitted wheezing for one year. Patient 2, a nine-month-old girl, presented with episodes of acute respiratory distress with expectoration of milky-white bronchial casts for four months. There was no obvious evidence of infection in either child. Bilateral thickening of bronchovascular bundles and interlobular septal, as well as multiple patchy ground-glass opacities were seen on chest computed tomography (CT) in both patients. Lymphangioscintigraphy demonstrated pulmonary lymph reflux in both patients and slowed lymphatic drainage of the lower limbs in patient 1. Primary PLFD was considered for both patients, and a diagnosis of yellow nail syndrome was made in patient 1. Both patients received lymphatic interventional treatment, but all experienced recurrence following the procedure.

Lymphatic Complications Prevention and Soft Tissue Reconstruction after Soft Tissue Sarcoma Resection in the Limbs

Background and Objectives: The definitive treatment of soft tissue sarcomas (STS) requires a radical surgical removal of the tumor, which often leads to large soft tissue defects. When they are located in the limbs, significant damage to the lymphatic pathways is not uncommon. In the present article, we present different techniques aimed at both reconstructing the defect and restoring sufficient lymph drainage, thus preventing short- and long-term lymphatic complications. Materials and Methods: Between 2018 and 2020, 10 patients presenting a soft tissue defect with lymphatic impairment received a locoregional reconstruction by means of either pedicled or free SCIP flap. Seven patients required a second flap to reach a good dead space obliteration. In six cases, we performed an interpositional flap, namely a soft tissue transfer with lymphatic tissue preservation, and in four cases a lymphatic flow-through flap. In all cases, the cause of the defect was STS surgical excision. The average age was 60.5 years old (ranging 39–84), seven patients were females and six were males. Results: All the patients were successfully treated. In two cases, minor post-operative complications were encountered (infected seroma), which were conservatively managed. No secondary procedures were required. The average follow-up was 8.9 months (ranging 7–12 months). No signs of lymphedema were reported during this time. In all cases, complete range of motion (ROM) and a good cosmetic result were achieved. Conclusions: A reconstructive procedure that aims not only to restore the missing volume, but also the lymphatic drainage might successfully reduce the rate of postoperative complications. Both lymphatic interpositional flaps and lymphatic flow-through flaps could be effective, and the right choice must be done according to each patient’s needs.

Novel Use of a Synovis™ Coupler device for a Surgical Lymphovenous Anastomosis for the treatment of refractory Chylothorax in a patient with Central Lymphatic Flow Disorder.

Refractory post-operative chylothorax in congenital heart disease is difficult to treat. We present a case of intractable neonatal chylothorax after cardiac surgery due to central lymphatic flow disorder that was treated by creating a lymphovenous anastomosis of the thoracic duct to the left external jugular vein for definitive treatment

Comparison of normal hindlimb lymphatic systems in rats with detours present after lymphatic flow blockage

In the present study, we aimed to identify the normal hindlimb lymphatic systems in rats and compare them with the detours after lymphatic flow blockage. The lymphatic systems of the hindlimbs of normal rats were investigated via lymphography using a near-infrared fluorescence imaging system. The lymphatic vessels were stained using Evans Blue. The lymphatic flow was blocked through lymphatic vessel ligation combined with inguinal and popliteal lymph node dissection. Detours that appeared after 30 days were visualized using lymphography and immunostaining with anti-podoplanin antibodies. Three main results were obtained in the present study. First, the deep medial system, the superficial medial system, a connection between the superficial and deep medial lymphatic systems, and the superficial lateral system, were elucidated. Second, three types of detours, namely the detour of the lateral abdomen, the detour to the lymphatic vessel near the midline of the abdomen, and the detour to the contralateral inguinal lymph node, were identified after lymphatic flow blockage. Lastly, detours were located in the fatty layer above the panniculus carnosus muscle and their lumina were wide. The histology suggested that the detour was a pre-collecting lymphatic vessel. Lymphatic routes in the rat hindlimbs after lymphatic flow blockage were different from those of the normal rat lymphatic system. It was suggested that the detour is a pre-collecting lymphatic vessel and that encouraging its development may be a new method of simple lymphatic drainage.

nPTD classification: an updated classification of gastric cancer location for function preserving gastrectomy based on physiological lymphatic flow

Background The correlation between tumor location and lymphatic flow distribution in gastric cancer has been previously reported, and PTD (Proximal – Transitional – Distal) classification was proposed. Our group updated and developed the nPTD classification. Method We retrospectively studied gastric cancer patients who underwent the dye method sentinel node biopsy from 1993 to 2020. The inclusion criteria were a single lesion type 0 cancer of ≤5 cm in the long axis, clinically node-negative, and invasion within the proper muscle layer pathologically. In this study, the distribution of dyed lymphatic flow was evaluated for each occupied area of the tumor. Results We included 416 patients in this study. The tumors located in the watershed of the right and left gastroepiploic arteries near greater curvature had extensive lymphatic flow; therefore, a newly circular region with a diameter of 5 cm is set on the watershed of the greater curvature between P and T zone as the ‘n’ zone. In addition, for cancers located in the lesser P curvature, lymphatic flow to the greater curvature was not observed. Therefore, the P zone was divided into two: the lesser curvature side (PL) and the greater curvature side (PG). Conclusions The advantage of the nPTD classification is that it provides not only proper nodal dissection but also adequate function-preserving gastrectomy. If the tumor is localized within the PL, the proximal gastrectomy resection area can be further reduced. In contrast, for cancers located in the ‘n’ zone, near-total gastrectomy is required because of the extensive lymphatic flow.

Warty Lesion Over Amputated Stump

Introduction: Amputation can disturb the quality of life, and it can be worsened when the amputated stump suffers from any dermatoses. It is mandatory for an amputee and the doctor to know about the common dermatoses that an amputated limb can suffer from. The prosthesis should be properly fitting, and hygiene has to be maintained. Case Presentation: A 49-year-old male presented with a warty lesion over the amputated limb. Histopathological examination revealed verrucous hyperplasia, and further examination showed improperly fitted prosthesis. A well-fitted prosthesis with proper padding cured his lesion. Conclusions: The case report highlights how a properly fitted prosthesis corrects the lymphatic flow and thereby reduces complications. It is also important to maintain stump hygiene, as the humid, relatively anaerobic environment inside the prosthesis can trigger the growth of various organisms.

Intrahepatic Dynamic Contrast‐Enhanced Magnetic Resonance Lymphangiography: Potential Imaging Signature for Protein‐Losing Enteropathy in Congenital Heart Disease

Background Protein‐losing enteropathy (PLE) is a significant cause of morbidity and mortality in congenital heart disease patients with single ventricle physiology. Intrahepatic dynamic contrast‐enhanced magnetic resonance lymphangiography (IH‐DCMRL) is a novel diagnostic technique that may be useful in characterizing pathologic abdominal lymphatic flow in the congenital heart disease population and in diagnosing PLE. The objective of this study was to characterize differences in IH‐DCMRL findings in patients with single ventricle congenital heart disease with and without PLE. Methods and Results This was a single‐center retrospective study of IH‐DCMRL findings and clinical data in 41 consecutive patients, 20 with PLE and 21 without PLE, with single ventricle physiology referred for lymphatic evaluation. There were 3 distinct duodenal imaging patterns by IH‐DCMRL: (1) enhancement of the duodenal wall with leakage into the lumen, (2) enhancement of the duodenal wall without leakage into the lumen, and (3) no duodenal involvement. Patients with PLE were more likely to have duodenal involvement on IH‐DCMRL than patients without PLE ( P <0.001). Conclusions IH‐DCMRL findings of lymphatic enhancement of the duodenal wall and leakage of lymph into the duodenal lumen are associated with PLE. IH‐DCMRL is a useful new modality for characterizing pathologic abdominal lymphatic flow in PLE and might be useful as a risk‐assessment tool for PLE in at‐risk patients.

IMAGING OF LYMPHATIC DYSPLASIA IN NOONAN SYNDROME: CASE STUDIES AND HISTORICAL ATLAS

To determine the historical use and utility of various lymphatic imaging modalities in Noonan syndrome (NS) patients, we performed a comprehensive literature review by collecting the published medical imaging of NS lymphatic dysplasias. We correlated imaging findings with clinical phenotypes and treatment. Our analysis of lymphatic imaging modalities provides an algorithmic approach to imaging and patient care across the spectrum of NS developmental defects. A total of 54 NS cases have been published since 1975. Using the observations reported in 15 reviewed publications, an association was made between disruptions in central lymphatic flow and poor clinical presentations/outcomes in NS patients.

Laparoscopic low anterior resection for rectal cancer wherein the inferior mesenteric artery arose from the superior mesenteric artery: a case report

Background Few cases have been reported of colorectal cancer with inferior mesenteric artery (IMA) branching abnormalities; therefore, the lymphatic flow in such cases remains unknown. We report the first case of locally advanced rectal cancer in which the IMA arose from the superior mesenteric artery (SMA) in which we achieved to visualize the lymphatic flow. Case presentation A 65-year-old woman complaining of bloody stools was investigated in our hospital and suspected with rectal cancer. Colonoscopy and abdominal enhanced computed tomography (CT) revealed a circumscribed, localized ulcerative tumor in the rectum. 3-Dimensional contrast-enhanced computed tomography (3D-CT) showed that the IMA arose from the SMA. The patient was diagnosed with rectal cancer (cT3N0M0, cStage IIa) and laparoscopic low anterior resection was performed. The sigmoid colon was resected using the medial approach. Only the plexus of the colic branch of the lumbar splanchnic nerve was observed at the site where the root of the IMA usually exists and showed interruption of the indocyanine green (ICG) fluorescence-illuminated lymphatics. The root of the IMA was ligated, and Japanese D3 lymphadenectomy was performed, preserving the accessory middle colic artery. All fluorescent lymph nodes were resected. The pathological diagnosis was pT4aN1aM0 stage IIIb. The patient’s postoperative course was uneventful. Adjuvant chemotherapy was administered, and the patient was recurrence-free at 1.5 years after surgery. Conclusions We were able to perform safe and appropriate surgery oncologically, despite abnormal vascular anatomy, due to preoperative identification using 3D-CT and intraoperative navigation using ICG administration.