Superior mesenteric artery syndrome: An often overlooked cause of abdominal pain!

OBJECTIVES: To determine the prevalence of superior mesenteric artery (SMA) syndrome in patients presenting with abdominal pains, and to evaluate computed tomographic (CT) findings needed for its diagnosis. METHODS: This retrospective record-based study was carried out at the radiology department, from January 2016 to January 2021. All young patients (aged under 25) who underwent CT scans for abdominal pains were reviewed. Post-surgery, tumor, and trauma cases were excluded. Imaging findings for SMA syndrome were recorded as 'suggestive' (reduced aortomesenteric angle and distance with proximal duodenal dilatation), 'possible' (reduced angle and distance without proximal duodenal dilatation) and 'probable' (reduction of either angle or distance). Two radiologists interpreted the findings and consensus reporting was made. Diagnoses were confirmed on clinical grounds (symptomatic improvement by specific treatment and exclusion of other diagnoses), or barium studies. Imaging findings were compared to final diagnoses. McNemar's Chi-square test was used to determine association. RESULTS: Out of 141 patients (mean age=10.8, standard deviation=4), 7 (4.9%) patients mostly females were having SMA syndromes based on 'suggestive' imaging criteria (p=0.0005), and one patient underwent surgery. CONCLUSION: Superior mesenteric artery syndrome is not an uncommon condition and should be considered in differential diagnosis of acute abdomen in young patients after excluding other diagnoses.

Chronic Neutrophilic Leukemia with Monocytosis.

Chronic neutrophilic leukaemia is a very rare disease with diagnosis based on persistent leucocytosis >25×103/µl and monocytes <1×103/µl. The revised WHO criteria 2016 included CSF3R gene mutations as a diagnostic finding. We report the case of a 77-year-old man who was found to have asymptomatic persistent mature neutrophilic leucocytosis with monocytosis. Molecular study confirmed the presence of a CSF3R gene mutation in the absence of morphological or genetic features of myelodysplasia or other forms of myelodysplastic syndrome. The patient's medical history was significant for coronary artery disease, hypertension, chronic obstructive pulmonary disease, bilateral cystic bronchiectasis, moderate pulmonary hypertension, tuberculosis treated 27 years previously, hypothyroidism, and a thyroid nodule. He had hepatosplenomegaly but no lymphadenopathy, and no other malignancy was seen on computed tomography (CT) scanning. At the time of evaluation, he was free of symptoms and had no evidence of infection or drug-induced leucocytosis. The patient was referred to an oncology centre and treated with hydroxyurea and subsequently azacitidine. However, he developed pancytopenia with bone marrow aplasia. He died with neutropenia sepsis. The presence of persistent monocytosis in this case created a diagnostic dilemma as to whether the disease was a variant of chronic neutrophilic leukaemia or was reactive monocytosis. LEARNING POINTS: The presence of a CSF3R gene mutation is diagnostic for chronic neutrophilic leukaemia (CNL).The monocytosis in this patient might have been a new variant of CNL.