Abdominal Lymphatic Malformations Presenting as Protein-Losing Enteropathy

Lymphatic malformations (LMs) - are congenital vascular malformations caused by abnormal embryologic development of the lymphatic system with variable clinical manifestation. Abdominal LMs account approximately 5% of all LMs. The main treatment option for abdominal LMs is surgical. Purpose - to analyze our experience in intestinal and omental LMs diagnosis and treatment, to reveal factors that could influence the complications occurrence risks, that could improve abdominal LMs treatment results in children. Materials and methods. Among 240 pediatric patients, who underwent cystic LMs treatment in a period from December 2012 to July 2022, 43 (19.1%) were diagnosed abdominal and retroperitoneal LMs. 18 (41.8%) patients with intestinal and mesentery affection were included into the study group. Median follow up was 20.7 (95% CI: 12-27) months. Results. Male patients predominated in the study group. Median age at admission was 2.5 (95% CI: 0.75-5) years. 12 (66.6%) had an emergency admission. In 7 (38.8%) children LMs were complicated by intestinal obstruction (n=5; 71.4%), intracystic bleeding and LM cyst infection were observed in n=1, 14.3%) cases both. Surgical treatment was applied in 16 (88.8%) patients, among which 7 (43.7%) underwent laparoscopy. In 4 (25%) cases conversion to laparotomy took place, 5 (31.3%) underwent laparotomic surgeries. No recurrences after surgical treatment were observed. Systemic therapy with mTOR-inhibitors was prescribed in a child with symptomatic total mesentery affection, observational tactics was chosen for asymptomatic child. Conclusions. Intestinal and mesenterial LMs can be asymptomatic or manifest complications, which were observed in 7 (38.8%) children of our group. Macrocystic intestinal LMs are of higher risks of complications development (р=0.0236). Surgical treatment is effective for local intestinal and mesentery affection, excellent treatment result was achieved in 94.1% of patients. In cases with total mesentery affection mTOR-inhibitors systemic therapy is the treatment of choice. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

BackgroundMediastinal and abdominal lymphatic malformations may not be diagnosed until adulthood. Radiologic and pathologic diagnosis is often challenging due to the rarity of the lesion. Surgical excision of these lesions may be curative but lymphatic leak is a known complication. Lymphatic duct embolization may then be required to treat the leak.Case presentationWe describe a patient with post-surgical chylothorax where thoracic duct lymphangiography and embolization was performed by catheterizing the thoracic duct at the venous angle where it drains into the subclavian vein.ConclusionLymphatic duct embolization can be challenging in patients with lymphatic malformations. In these patients, if there is adequate visualization on ultrasound or fluoroscopy, terminal aspect of the thoracic duct can be accessed through the subclavian vein to perform the procedure.

Laparoscopic-assisted resection with adjunct sclerotherapy for abdominal lymphatic malformations: Long-term outcomes from a large cohort study.

BackgroundChronic diarrhea in adults is a common symptom with a wide range of underlying etiologies. Although various strategies have been proposed for evaluation, there are still cases with undetermined origins even after extensive workup. Amyloidosis with gastrointestinal (GI) involvement is one of the causes that should be considered in adult patients with chronic diarrhea. We report a case of primary systemic amyloid light-chain (AL) amyloidosis, presenting initially as chronic diarrhea and weight loss.Case presentationA 43-year-old man with chronic diarrhea and weight loss was referred to our hospital. Prior to his presentation, extensive evaluation including an exploratory laparotomy was carried out and did not yield any valuable findings. An echocardiography performed after repeated episodes of orthostatic hypotension revealed infiltrative cardiomyopathy. Moreover, biopsies of the terminal ileum revealed amyloid deposition confirmed by Congo Red staining. Finally, a diagnosis of systemic AL amyloidosis was made after hematological workup. Anti-plasma cell therapy did ameliorate his GI symptoms.ConclusionAlthough amyloidosis with GI involvement is a rare cause of chronic diarrhea, it should be considered especially in patients with intestinal malabsorption and extra-GI manifestations, such as orthostatic hypotension. The delayed diagnosis in the present case highlights the importance of recognizing clinical “red flags” not seemingly related to one another, and underscores the need to get intestinal biopsies even with normal endoscopic appearance of the mucosa.

Antecedentes: Las malformaciones linfáticas peritoneales son un hallazgo excepcional y benigno. Generalmente se presentan en niños como dilataciones linfáticas en cavidad abdominal. Existen muy pocos casos reportados en los que esta malformación aparece en espacios peritoneales y retroperitoneales, siendo el mesenterio del intestino delgado el más común seguido por el peritoneo. Presentación del caso: Somos los primeros en reportar un caso ecuatoriano sobre una malformación linfática peritoneal en un adolescente de 12.5 años con clínica de abdomen agudo. Un año después el paciente no ha reportado recidivas. Conclusiones: Ante la presencia de un cuadro de abdomen agudo no podemos dejar de lado esta patología, destacamos la ecografía como método diagnóstico y de seguimiento. La resección quirúrgica completa es curativa.

An 8-year-old female spayed Russian Blue cat presented for acute progressive dyspnoea. The patient had pleural effusion and was diagnosed with chylothorax. A multiphase contrast-enhanced CT scan of thoracic and abdominal cavities was performed to facilitate diagnosis and surgical planning. Incidentally, a large lobulated, elongated, cystic and encapsulated peritoneal mass was found. Contrast pooled within the peritoneal mass during lymphography, suggesting lymphatic origin. The patient was eventually euthanased as a result of recurrence of chylothorax. Necropsy revealed multiple irregular, thin-walled pockets of chylous fluid pooling within the mesentery. Histopathology of the affected mesentery was compatible with a diagnosis of lymphatic malformation. To the authors' knowledge, this is the first case describing a feline presentation of mesenteric lymphatic malformation. This is also the first case reporting the presentation of chylothorax for a veterinary patient with mesenteric lymphatic malformation.

Collagenous colitis and lymphocytic colitis, together constituting microscopic colitis, are common causes of chronic diarrhea. They are characterized clinically by chronic nonbloody diarrhea and a macroscopically normal colonic mucosa where characteristic histopathological findings are seen. Previously considered rare, they now have emerged as common disorders that need to be considered in the investigation of the patient with chronic diarrhea. The annual incidence of each disorder is five to ten per 100,000 inhabitants, with a peak incidence in 60- to 70-year-old individuals and a predominance of female patients in collagenous colitis. The etiology and pathophysiology are not well understood, and the current view suggests an uncontrolled mucosal immune reaction to various luminal agents in predisposed individuals. Clinical symptoms comprise chronic diarrhea, abdominal pain, fatigue, weight loss, and fecal incontinence that may impair the patient’s health-related quality of life. An association is reported with other autoimmune disorders, such as celiac disease, thyroid disorders, diabetes mellitus, and arthritis. The best-documented treatment, both short-term and long-term, is budesonide, which induces clinical remission in up to 80% of patients after 8 weeks’ treatment. However, after successful budesonide therapy is ended, recurrence of clinical symptoms is common, and the best possible long-term management deserves further study. The long-term prognosis is good, and the risk of complications, including colonic cancer, is low. We present an update of the epidemiology, pathogenesis, diagnosis, and management of microscopic colitis.

The association between Sjogren’s syndrome (SS) and protein-losing enteropathy (PLE) was scarcly reported. To analyze the clinical, therapeutic, and outcome characteristics of patients with SS and PLE and also to delineate the potential mechanisms and pathways connecting the gut to SS targeted organ’s pathology. Systematic screening was conducted using PubMed/MEDLINE, LILACS, SciELO, Web of Science, and Cochrane, dating 1980 to 2020. SS and PLE were the key words. Eighteen patients with SS and PLE were summarized. The patient’s ages ranged between 20 and 88 years, and only 4 were males. Primary SS was observed in most cases. Anti-Ro was detected in 100% of the cases while anti-La was reported in 64% of them. The clinical manifestations were protein loss, edema of the lower limbs, pleural effusion, ascites, facial edema, anasarca, diarrhea, and weight loss. Among these clinical manifestations, edema of the lower limbs was the most severe. Albumin concentration was 0.9–3.4 g/dL which increased to 2.8–4.3 g/dL after treatment. Small bowel biopsy was performed in all of the cases. Concerning the therapy, all the patients received systemic glucocorticoids. All of them improved. The period of onset of improvement ranged from 3 weeks to 36 months (an average of 3 months). The early diagnosis and appropriate therapy of PLE in patients with anti-Ro positive SS and who present edema, anasarca, or hypoalbuminemia is vital for a beneficial outcome. An excellent clinical improvement in all the cases was observed when treated early enough by cortico-therapy, thus preventing patient’s deterioration, complications, and reducing morbidity and potential mortality.

<h3></h3> Coeliac crisis is a medical emergency, rarely encountered in clinical practice. It may be a consequence of a delayed diagnosis of coeliac disease (CD). Diagnostic criteria for coeliac crisis include severe dehydration including orthostatic changes, renal dysfunction, neurological dysfunction, metabolic acidosis, abnormal electrolyte levels, hypoproteinaemia with Albumin below 3 g/dl and weight loss more than 4.5 kg. A diagnosis is made if more than 2 criteria are present.¹ We describe the case of a 4-year-old girl who presented with coeliac crisis as the first manifestation of undiagnosed coeliac disease, to raise awareness of the diagnostic criteria for coeliac crisis and consideration of this in unwell patients. Our patient presented with chronic diarrhoea, fatigue, and exhaustion. On admission she was not clinically dehydrated, had peripheral oedema, mildly distended abdomen with no signs of ascites. She had 20% weight loss, and her BMI was 13.23 (on 2d centile). A no-biopsy diagnosis of coeliac disease was made as she had IgA anti-tissue transglutaminase antibodies more than ten times the upper limit of normal and positive endomysium antibodies.⁵ Bloods showed compensated metabolic acidosis, with bicarbonate of 13, normal glucose at 4.1, low magnesium at 0.39 mmol/L, low albumin at 13 g/L. She required magnesium and albumin infusions for deficit correction. In addition, she had deranged PT/INR which normalised after two doses of intravenous Vitamin K. She also had transaminitis, and an extensive hepatitis screen. Autoimmune hepatitis was ruled out. An abdominal ultrasound showed fatty liver and normal elastography. Metabolic screen was normal. The levels of Vitamin D, folic acid, copper and zinc were low, and she had supplementation. Our patient met 4 out of the 7 criteria for coeliac crisis. She was commenced on gluten free diet, initially via nasogastric tube, then orally from day 4. She was monitored for refeeding syndrome and discharged following nutritional rehabilitation after 11 days. At her 2 months clinic follow up her bloods were normal; she has gained weight. Four months after her presentation her BMI improved to 17.7 (on 91st centile). Coeliac crisis was first described by Andersen and Di Sant’ Agnese in 1953 when they reported 35 patients with coeliac crisis, among which 3 were complicated by fatality.^{3 4} It is rarely encountered, probably due to better screening and early diagnosis; however, this can occasionally be the initial presentation of CD. The only effective treatment is a life-long gluten free diet. In coeliac crisis, the emergency treatment of electrolyte abnormalities is followed by gluten free diet and monitoring for refeeding syndrome. The role of corticosteroids in coeliac crisis is controversial but there are case reports of coeliac crisis with a good response to steroids, as they can reduce intestinal inflammation and restore the brush border enzymes.² Our patient did not require steroids and responded well to gluten free diet. This case highlights the importance of considering coeliac crisis in an acutely unwell patient with chronic diarrhoea, weight loss and metabolic derangements. <h3>References</h3> Mauro A, Casini F. Celiac crisis as the life-threatening onset of celiac disease in children: a case report. <i>Frontiers in Pediatrcs</i>. DOI 10.3389/fped.2023.1163765. Gupta S, Kapoor K. Steroids in celiac crisis: doubtful role. <i>Indian Pediatrics</i> 2014;<b>51</b>(9):756–757. Di Sant’ Agnese PA. Idiopathic coeliac disease. II. Course and prognosis. <i>Pediatrics</i> 1953 Mar;<b>11</b>(3):224–37. Andersen DH, Di Sant’ Agnese PA. Idiopathic coeliac disease. I. Mode of onset and diagnosis. <i>Pediatrics</i> 1953 Mar;<b>11</b>(3):207–23. Husby S, Koletzko S, Wessels M, <i>et al</i>. European society paediatric gastroenterology, hepatology and nutrition guidelines for diagnosing coeliac disease 2020. <i>JPGN</i> 2020;<b>70</b>:141–157.

Lymphatic anomalies comprise a spectrum of disorders ranging from common localized microcystic and macrocystic lymphatic malformations (LMs) to rare complex lymphatic anomalies, including generalized lymphatic anomaly, Kaposiform lymph-angiomatosis, central conducting lymphatic anomaly, and Gorham-Stout disease. Imaging diagnosis of cystic LMs is generally straightforward, but complex lymphatic anomalies, particularly those with multiorgan involvement or diffuse disease, may be more challenging to diagnose. Complex lymphatic anomalies are rare but associated with high morbidity. Imaging plays an important role in their diagnosis, and radiologists may be the first clinicians to suggest the diagnosis. Furthermore, radiologists are regularly involved in management given the frequent need for image-guided interventions. For these reasons, it is crucial for radiologists to be familiar with the spectrum of entities comprising complex lymphatic anomalies and their typical imaging findings. In this article, we review the imaging findings of lymphatic anomalies, including LMs and complex lymphatic anomalies. We discuss characteristic imaging findings, multimodality imaging techniques used for evaluation, pearls and pitfalls in diagnosis, and potential complications. We also review recently discovered genetic changes underlying lymphatic anomaly development and the advent of new molecularly targeted therapies.

Nutrition plays a very important role in the healthy and in the ill horse. Although research in this field clearly shows that incorrect nutritional practices may lead to severe pathologies, inappropriate feeding plans often continue to be used. A clinical nutrition counselling (CNC) service could thus be of great use to both horse owners and veterinarians. The aim of this study was to provide information on equine patients referred to the CNC service of the University of Turin and to provide standard dietary protocols as used in our Veterinary Teaching Hospital for the most common nutrition-related pathologies. The data were obtained by retrospective analysis of the nutritional records of referred equine patients. The data collected included information about anamnesis, nutritional assessment, current diet, referring person and follow-up of each patient. Sixty-one horses were included in the study. The majority were adult males. The most common breeds were the Italian Saddle Horse and the Friesian Horse. Old horses (>19years) had a statistically lower BCS than brood mares or other adult horses (p<0.01). The most common nutritional pathologies were chronic weight loss (CWL), chronic diarrhoea (CD) and equine gastric ulcer syndrome. All horses received first-cut meadow hay; 85% also ate concentrates. Young horses (<2years) received more hay as a percentage of body weight (BW) than old horses or adults. The hay percentage of BW per day given to animals with CWL was statistically higher than those with CD (p<0.01). The concentrate percentage of BW given to old horses was statistically lower compared to that given to young horses (p<0.05). The concentrate percentage of BW per day given to horses with colic was statistically higher than that given to horses with CD (p<0.05). 28% of cases were referred by the owner and 72% by a veterinarian. Follow-up evaluation was deemed to be 'good' in 92% cases and 'poor' in 8%. In summary, the CNC service could provide an epidemiological observatory to study the prevalence of nutritional issues in the equine population.

Small bowel bacterial overgrowth (SBBO) syndrome is associated with excessive numbers of bacteria in the proximal small intestine. The pathology of this condition involves competition between the bacteria and the human host for ingested nutrients. This competition leads to intraluminal bacterial catabolism of nutrients, often with production of toxic metabolites and injury to the enterocyte. A complex array of clinical symptoms ensues, resulting in chronic diarrhea, steatorrhea, macrocytic anemia, weight loss, and less commonly, protein-losing enteropathy. Therapy is targeted at correction of underlying small bowel abnormalities that predispose to SBBO and appropriate antibiotic therapy. The symptoms and signs of SBBO can be reversed with this approach.

Chronic diarrhea

BackgroundChronic recurrent diarrhoea and weight loss is a common problem in captive callitrichids. These symptoms are common clinical features of marmoset wasting syndrome (MWS), a chronic enteric inflammation of unknown aetiology associated with mortality in captive marmosets. The unknown aetiology of the condition presents problems for conservation projects where affected colonies present higher mortality and lower birth rates. Since a role for the microbiome has been established in chronic enteric inflammation of other species it is possible that the intestinal microbiome undergoes similar changes during MWS.ResultsThe faecal microbiome of pied tamarins (Saguinus bicolor) at Jersey Zoo was determined using 16S rRNA gene amplicon sequencing to compare the composition of the faecal microbiome of tamarins affected by chronic recurrent diarrhoea and weight loss with unaffected individuals. Affected individuals had a higher relative abundance of amplicon sequence variants assigned to Lactobacillus and Helicobacter jaachi while unaffected individuals had a higher relative abundance of some Lachnospiraceae and Ruminococcaceae.ConclusionsAlthough Helicobacter has been shown to reside in healthy wild and captive marmosets and tamarins and appears to form part of the normal microbiota, the results of this study raise the prospect that certain species of Helicobacter may be associated with chronic, recurrent diarrhoea in captive callitrichids. The presence of Lactobacillus may also play a role in the development of MWS. Since depletion of Lachnospiraceae and Ruminococcaceae have been linked to chronic gastrointestinal inflammation in humans, this feature of the microbiome of affected tamarins provides another avenue of further research in the pathogenesis of MWS.

Neurofibromatosis type-1 (NF1), also known as Von Recklinghausen disease, is an autosomal dominant disorder with incidence of one per 4000. Neurofibromas are benign, heterogeneous, peripheral nerve sheath tumors coming up from the connective tissue of peripheral nerve sheaths, particularly the endoneurium. Visceral involvement in disseminated neurofibromatosis is considered rare. Neurofibroma occurs most frequently in the stomach and jejunum, but colon and anorectal canal may also be involved. Gastrointestinal neurofibromas may lead to bleeding, obstruction, intussusception, protein-losing enteropathy and bowel perforation. We encountered a case of diffusely involving the anorectal area by huge neurofibroma, which resulted in pelvic pain with watery diarrhea and urgency.