OBJECTIVE: A female Rio Cauca caecilian Typhlonectes natans (estimated as between 10 and 18 years of age) housed at the Smithsonian National Zoological Park in Washington, D.C., developed progressive severe coelomic effusion over a 4-week period. The coelomic effusion was diagnosed via radiographs and ultrasound, and a sample of the fluid was obtained for analysis, which revealed a low-protein transudate suggestive of inflammation. As the coelomic effusion progressed, the caecilian became tachypneic, hyporexic, and lethargic. The caecilian was started on antibiotics and a diet trial, but signs continued despite therapy.
METHODS: An exploratory celiotomy was performed, which revealed adipose tissue torsion with local lymphangiectasia and a presumptive biliary cyst. Surgical correction was unable to be achieved due to concern for fatal hemorrhage, as the vasculature associated with the torsion was severely distended. Due to the severity of the torsion and associated risks, the caecilian was euthanized intraoperatively and subsequently necropsied for histologic evaluation.
RESULTS: After reviewing the caecilian\'s presentation and the progression of disease, it is suspected that the severe coelomic effusion was secondary to lymphangiectasia, which occurred subsequent to the adipose tissue torsion.
CONCLUSIONS: This is the first reported case of adipose tissue torsion and associated clinical disease in an aquatic caecilian and should be a differential for progressive coelomic effusion in this species.

Intestinal lymphangiectasia (IL) is characterized by the dilation of intestinal lymphatic vessels, which can rupture and cause loss of lymph into the intestine. Due to the high content of proteins, lipoproteins, and lymphocytes in the intestinal lymph, loss of lymph might result in hypoproteinemia, hypoalbuminemia, hypogammaglobulinemia, and lymphocytopenia. In addition, there may be a depletion of minerals, lipids, and fat-soluble vitamins. IL can be primary due to inherent malfunctioning of the lymphatic system, or secondly, a result of various factors that may hinder lymphatic drainage either directly or indirectly. This condition has emerged as a subject of significant clinical interest. Given that the intestinal lymphatic system plays an important role in the body\'s fluid homeostasis, adaptive immunity, nutrient and drug absorption, intestinal transport, and systemic metabolism, its dysfunction may have wider implications. Although primary IL is rare, with varied clinical features, complications, treatment response, and outcomes, secondary IL is more common than previously believed. The definitive diagnosis of IL requires endoscopic demonstration of whitish villi (which frequently resemble snowflakes) and histological confirmation of dilated lacteals in the small intestinal mucosa. Treatment of IL is challenging and involves dietary modifications, managing underlying medical conditions, and using medications such as sirolimus and octreotide. Recognizing its prevalence and diverse etiology is crucial for targeted management of this challenging medical condition. This article provides a comprehensive exploration of the clinical implications associated with IL. In addition, it offers valuable insights into critical knowledge gaps in the existing diagnostic and management landscape.

Renal lymphangiectasia (RL) is a rare condition in which lymphatic vessels are dilated giving rise to cyst formation in peripelvic, perirenal and intrarenal locations. Knowledge about RL is limited and based upon individual case reports. This can be genetic or acquired. There is no significant association with any gender or age. It can be manifested as focal or diffuse forms and can be unilateral or bilateral. Most of the cases present with abdominal or flank pain. The diagnosis is based on radiological imaging. Due to rarity of diseases, it has potential to be misdiagnosed as other cystic disease of kidneys. The treatment is mainly conservative but prolonged follow up for associated complications like hypertension and renal vein thrombosis is required. We have presented a case of bilateral renal lymphangiectasia with the review of available literature.

OBJECTIVE: Dermatochalasis is a common disorder of the elderly, often requiring upper blepharoplasty. Although it is mainly accepted as a process of aging, its clinical and histological findings vary among patients. The aim of this study was to classify types of dermatochalasis based on their clinical and histological findings.
METHODS: This retrospective study included patients with dermatochalasis who had undergone senile blepharoplasty at a single center. Clinical parameters such as margin-to-reflex distance 1 (MRD1), eyelid contour, visual field, and pre-existing medical conditions were assessed. Histological analysis was conducted of eyelid tissues stained with hematoxylin and eosin (H&E) and D2-40 to evaluate dermal edema, inflammation, lymphatic changes, and stromal depth.
RESULTS: This study included 67 eyes of 35 patients. The mean age of the patients was 69.0 ± 8.3 years, and the average MRD1 was 1.8 ± 1.3 mm. In correlation analysis, two distinct types of dermatochalasis based on the histological findings were identified: lymphangiectasia-dominant and stromal edema-dominant types. The difference between nasal and temporal side MRD1(NT-MRD1) showed the area under the ROC curve of 0.718 of for distinguishing the two histological types of dermatochalasis was 0.718.
CONCLUSIONS: Our novel classification of senile dermatochalasis based on morphological and histological analysis provides insights into the underlying pathology and may help to predict surgical outcomes and complications.

UNASSIGNED: Adequate evaluation of patients with Hennekam Syndrome (HS) is challenging for physicians, because of multi-organ involvement and complex pathophysiology. We report the first case in an African American with lymphedema, who developed protein-losing enteropathy (PLE) and was successfully diagnosed with HS from cause-and-effect complications by Waldmann\'s Disease (WD) and comorbid Celiac Disease (CD).
UNASSIGNED: As far as we know, this is the 51st case of HS worldwide and the first one in an African American. The examined patient met all diagnostic criteria for HS, suggesting a dysfunction in the development of the lymphatic system, with associated comorbidities including developmental delay, gastrointestinal pathologies, facial and hearing abnormalities, and cardiac defects. Primary intestinal lymphangiectasia (WD) is a consequence of HS, which ultimately results in PLE and worsening interstitial lymph buildup. Based on our findings, CD, a complication not yet reported in HS, may arise from WD. Other autoimmune diseases may be seen in HS: a previous report demonstrated positive anti-thyroid stimulating hormone antibodies in HS patients. We propose that in HS, increased interstitial lymph (WD, if intestinal) with protein loss induces TNF-α- and IL-6-mediated immune reactions in the affected visceral organs, causing autoimmune pathologies. The interstitial lymph fluid-induced TNF-α and IL-6-mediated immunopathogenic reactions lead to inflammation and subsequent destruction of the intestinal mucosa. The chronic inflammatory increase in TGF-β causes gastric mucosa hypertrophy, which results in gastric fold thickening. Eventually, wider tight junctions develop, increasing gastric mucosa permeability, and leading to gastropathy. Considering the examined patient\'s history of gastroenteritis and the literature stating that CD is a non-mucosal cause of gastropathy and PLE, it is suggested that sequelae of GI complications occur in a cause-and-effect chain in HS. HS results in WD, which causes CD, resulting in hypertrophic gastropathy and loss of parietal and chief cells, eventually leading to malabsorption and PLE (Figure 1). HS primarily affects various organs due to inflammatory-mediated damage and accumulation of lymph fluid. Other findings for HS include keratoconjunctivitis sicca (dry eye disease), fibrous lymphedema exhibiting lymphorrhea, chylous ascites, anemia, and parathyroid abnormalities. Immune impairment in HS predisposes patients to autoimmune disorders, therefore autoimmunity (CD) and WD are concomitant comorbidities of HS. HS-associated comorbidities are primarily due to inflammation and damage to immune cell transport or underlying health conditions affecting proper lymphatic function. However, it is suggested that HS mutations may disrupt the development of the lymphatic system leading to further complication. complications can be compound heterozygous, and there is a need for further research to identify nearby genes that can cause concomitant co-morbidity.

The lymphatic system has a pivotal role in immune homeostasis. To better understand this, we investigated the impact of Primary Lymphatic Anomalies (PLA) on lymphocyte numbers and phenotype.
The study comprised (i) a retrospective cohort: 177 PLA subjects from the National Primary Lymphatic Anomaly Register with clinical and laboratory data, and (ii) a prospective cohort: 28 patients with PLA and 20 healthy controls. Patients were subdivided using established phenotypic diagnostic categories and grouped into simplex (localised tissue involvement only) and systemic (involvement of central lymphatics). Further grouping variables included genital involvement and the likelihood of co-existent intestinal lymphangiectasia. Haematology laboratory parameters were analysed in both cohorts. In the prospective cohort, prospective blood samples were analysed by flow cytometry for markers of proliferation, differentiation, activation, skin-homing, and for regulatory (CD4+Foxp3+) T cells (Treg).
In patients with PLA, lymphopaenia was frequent (22% of subjects), affected primarily the CD4+ T cell subset, and was more severe in subjects with systemic versus simplex patterns of disease (36% vs 9% for lymphopaenia; 70% vs 33% for CD4+ cells). B cells, NK cells and monocytes were better conserved (except in GATA2 deficiency characterised by monocytopaenia). Genital oedema and likelihood of concomitant intestinal lymphangiectasia independently predicted CD4+ T cell depletion. Analysing CD4+ and CD8+ T cells by differentiation markers revealed disproportionate depletion of naïve cells, with a skewing towards a more differentiated effector profile. Systemic PLA conditions were associated with: increased expression of Ki67, indicative of recent cell division, in naïve CD4+, but not CD8+ T cells; increased levels of activation in CD4+, but not CD8+ T cells; and an increased proportion of Treg. Skin-homing marker (CCR10, CLA and CCR4) expression was reduced in some patients with simplex phenotypes.
Patients with PLA who have dysfunctional lymphatics have a selective reduction in circulating lymphocytes which preferentially depletes naïve CD4+ T cells. The presence of systemic disease, genital oedema, and intestinal lymphangiectasia independently predict CD4 lymphopaenia. The association of this depletion with immune activation and increased circulating Tregs suggests lymphatic-lymphocyte interactions and local inflammatory changes are pivotal in driving immunopathology.

Chylopericardium can be due to a variety of secondary causes like trauma, radiation, tumors, following cardiac surgery, etc., or may be idiopathic due to abnormal lymphatic system and mediastinal lymphangiectasia, which is a rare entity. Here, we present a case of a 34-year-old previously healthy male presenting with idiopathic chylopericardium. 2D echocardiography revealed massive pericardial effusion without features of cardiac tamponade. Following pericardiocentesis, a CT scan of the thorax and MR lymphangiogram were done to arrive at a diagnosis of idiopathic chylopericardium. In addition to medical management, surgical treatment included partial pericardiectomy and sclerotherapy of the mediastinal lymphatic sac. The patient had an uneventful post-operative period.

Lymphangiectasia is the benign malformation of lymphatic channels associated with either focal or diffuse dilation of vessels and impaired lymph drainage. This malformation has the potential to create a cystic mass due to the accumulation of lymphatic fluid. While rare in adults, intussusception, the telescoping of the proximal bowel into the distal bowel, can be caused by a mass within the bowel. In this case, a near-obstructing cystic colon mass developed in a 74-year-old man; this was later found to be a large lymphangiectasia. In addition, this near-obstructing colonic lymphangiectasia served as the lead point in a colo-colonic intussusception. Due to this complication, the mass was immediately removed by a laparoscopic oncologic right-extended hemicolectomy which proved to be both diagnostic and therapeutic.

Primary intestinal lymphangiectasia (PIL) is a rare congenital disorder characterized by lymphatic system obstruction, resulting in the leakage of lymph into the bowel lumen. We present the case of a 6-year-old boy with recurrent diarrhea and weight loss. On examination, bilateral pitting edema in the lower limbs was observed. Laboratory investigations revealed hypoalbuminemia and lymphopenia. Contrast-enhanced CT of the abdomen showed thickening of the jejunum, echogenic fat islands, and enlarged lymph nodes in the mesentery. The diagnosis was confirmed by endoscopic biopsy. The patient was managed with a high-protein diet and replacement of the long-chain triglycerides with medium-chain triglycerides. Gradual improvement in symptoms was observed with regular follow-up. PIL is a protein-losing enteropathy that causes hypoproteinemia, hypolymphopenia, and hypoglobulinemia. PIL usually presents with peripheral edema, weight loss, abdominal pain, and chronic diarrhea. Diagnosis is based on characteristic endoscopic and histopathologic findings. Management involves a multidisciplinary approach, including nutritional modifications, medical therapy, and, in rare cases, surgical resection. PIL remains a challenging diagnosis due to its nonspecific clinical presentation. Clinicians should maintain awareness of this disorder for prompt identification and management.

BACKGROUND: The role of diet in the pathogenesis and treatment of chronic enteropathies (CE) in dogs is unresolved.
OBJECTIVE: To compare the ability of diets composed of hydrolyzed fish, rice starch, and fish oil without (HF) or with prebiotics, turmeric, and high cobalamin (HF+) against a limited ingredient diet containing mixed nonhydrolyzed antigens and oils (control) to resolve clinical signs and maintain serum cobalamin and folate concentrations in dogs with nonprotein losing CE (non-PLE). To determine the ability of hydrolyzed fish diets to support recovery and remission in dogs with PLE.
METHODS: Thirty-one client-owned dogs with CE: 23 non-PLE, 8 PLE.
METHODS: Randomized, blinded, controlled trial. Diets were fed for 2 weeks; responders continued for 12 weeks. Nonresponders were crossed over to another diet for 12 weeks. Response was determined by standardized clinical evaluation with long-term follow-up at 26 weeks. Concurrent medications were allowed in PLE.
RESULTS: Nineteen of 23 (83%; 95% confidence interval [CI], 60%-94%) non-PLE CE responded clinically to their initial diet, with no difference between diets (P > .05). Four nonresponders responded to another diet, with sustained remission of 18/18 (100%; 95%CI, 78%-100%) at 26 weeks. Serum cobalamin concentration was increased (P < .05) and maintained by diet. Serum folate concentration decreased posttreatment (P < .05) but was restored by dietary supplementation. Hydrolyzed fish diets supported weight gain, serum albumin concentration, and recovery (P < .05) in dogs with PLE.
CONCLUSIONS: Changing diet, independent of antigen restriction or supplemental ingredients, induced long-term remission in dogs with non-PLE CE. Serum cobalamin and folate concentrations were maintained by diet. Hydrolyzed fish diets supported clinical recovery and remission in PLE.