Primary splenic epithelial cyst is an unusual event in everyday surgical practice with about 800 cases reported until date in the English literature. Splenic cysts may be parasitic or non-parasitic in origin. Nonparasitic cysts are either primary or secondary. Primary cysts are also called true, congenital, epidermoid or epithelial cysts. Primary splenic cysts account for 10% of all benign non-parasitic splenic cysts and are the most frequent type of splenic cysts in children. Usually, splenic cysts are asymptomatic and can be found incidentally during imaging techniques or on laparotomy. The symptoms are related to the size of cysts. When they assume large sizes, they may present with fullness in the left abdomen, local or referred pain, symptoms due to compression of adjacent structures (like nausea, vomiting, flatulence, diarrhoea) or rarely thrombocytopenia, and occasionally complications such as infection, rupture and/or haemorrhage. The preoperative diagnosis of primary splenic cysts can be ascertained by ultrasonography (USG), computed tomography or magnetic resonance imaging, although the wide use of USG today has led to an increase in the incidence of splenic cysts by 1%. However, careful histopathological evaluation along with immunostaining for presence of epithelial lining is mandatory to arrive at the diagnosis. The treatment has changed drastically from total splenectomy in the past to splenic preservation methods recently.
Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or subserosa leading to protein loosing enteropathy.Most often affected were children and generally diagnosed before third year of life but may be rarely seen in adults too. Bilateral pitting oedema of lower limb is the main clinical manifestation mimicking the systemic disease and posing a real diagnostic dilemma to the clinicians to differentiate it from other common systemic diseases like Congestive cardiac failure, Nephrotic Syndrome, Protein Energy Malnutrition, etc. Diagnosis can be made on capsule endoscopy which can localise the lesion but unable to take biopsy samples. Thus, recently double-balloon enteroscopy and biopsy in combination can be used as an effective diagnostic tool to hit the correct diagnosis. Patients respond dramatically to diet constituting low long chain triglycerides and high protein content with supplements of medium chain triglyceride. So early diagnosis is important to prevent untoward complications related to disease or treatment for the sake of accurate pathological diagnosis.
The primary splenic lymphoma is extremely uncommon, can present with grave complications like hypersplenism and splenic rupture. In view of vague clinical presentation, it is difficult to arrive at the diagnosis. In such circumstances, histopathological diagnosis is very important. A precise diagnosis can only be made on histopathology and confirmed on immunohistochemistry.Emergency splenectomy is preferred as an effective therapeutic and diagnostic tool in cases with giant splenomegaly. Core biopsy is usually not advised due to a high risk of post-core biopsy complications in view of its high vascularity and fragility. Aim behind highlighting the topic is to specify that core biopsy/ fine needle aspiration cytology can be used as an effective diagnostic tool to arrive at correct diagnosis to prevent untoward complications related to disease and treatment. Anticoagulation therapy is vital after splenectomy to avoid portal splenic vein thrombosis.
Crohn's disease (CD) is a chronic idiopathic inflammatory disease of gastrointestinal tract characterized by segmental and transmural involvement of gastrointestinal tract. Ileocolonic and colonic/anorectal is a most common and account for 40% of cases and involvement of small intestine is about 30%. Isolated involvement of stomach is an extremely unusual presentation of the disease accounting for less than 0.07% of all gastrointestinal CD. To date there are only a few documented case reports of adults with isolated gastric CD and no reports in the pediatric population. The diagnosis is difficult to establish in such cases with atypical presentation. In the absence of any other source of disease and in the presence of nonspecific upper gastrointestinal endoscopy and histological findings, serological testing can play a vital role in the diagnosis of atypical CD. Recent studies have suggested that perinuclear anti-neutrophil cytoplasmic antibody and anti-Saccharomycescervisia antibody may be used as additional diagnostic tools. The effectiveness of infliximab in isolated gastric CD is limited to only a few case reports of adult patients and the long-term outcome is unknown.
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