Generalized lymphatic anomaly is a rare multisystem congenital disorder in which multiple organs are involved. Imaging features often overlap with other complex lymphatic anomalies and diagnosis is difficult. Treatment options are limited, not remedial and prognosis is poor. We report a 12-year-old male who presented with axillary and chest wall lymphangioma but was subsequently diagnosed as having diffuse lymphangiomatosis affecting lungs, liver, spleen, and bones on computerized tomography scan. We suggest complete radiological evaluation of susceptible adolescent children with lymphangioma to avoid diagnostic delay in this morbid condition. We also discuss radiological features of other similar complex lymphatic anomalies and crucial role of imaging in diagnosis.

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A 63-year-old female presented to pulmonary medicine OPD with complaints of breathlessness and cough for two weeks and intermittent fever for one week. She had pain in the left side of her chest for six months. She was a known case of diabetes mellitus but was not on any treatment. On admission, her blood pressure was 130/90 mmHg, pulse rate of 96/min, respiratory rate of 18/ min and her SpO 2 was 98% at room air. On examination, her heart sounds were normal (no murmurs heard), respiratory system examination revealed bilateral normal vesicular breath sounds with bilateral wheeze, central nervous system and per abdomen examination were unremarkable. A 2D ECHO done revealed a normal study with ejection fraction of 60%. Pulmonary function test showed a restrictive lung disease. Upon chest x-ray she was found to have a homogenous opacity in her left lung lower zone and CT scan revealed a pericardial mass in the posterior aspect with non-homogenous and irregular contrast enhancement in axial and coronal images, along with minimal pleural effusion [Table/ Fig-1]. Decision for surgical removal of the mass was taken due to clinical symptom of breathlessness which could have been produced due to pressure effect on the lung due to the pericardial mass.She underwent anterolateral thoracotomy and excision of the mass was done which was arising from the pericardium, in the posterior aspect. There was no extension of the mass into any cardiac chamber. The mass was sent for histopathological examination and gross examination revealed a soft cystic mass weighing 100 gm and measuring 7 x 6.5 x 5 cm. Outer surface appeared smooth and had a fibrous covering. Cut surface was hemorrhagic with oozing of blood and had spongy, cyanotic appearance. Spongy spaces were filled with blood. Central portion showed grey white areas [Table/ Fig-2].Microscopic examination showed a lesion covered by thick fibrocollagenous covering with focal flattened mesothelial lining on the surface. The lesion was composed of numerous irregular, cavernous spaces set in a loose fibrous stroma. Cavernous spaces had thin walls lined by single layered endothelium, few vessels showed smooth muscles [Table/ Fig-3]. Most of the spaces were filled with blood, while occasional spaces showed fresh thrombi. Stroma showed focal myxoid change. The patient eventually died due to co-morbidities in the postoperational period.

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Introduction The prevalence of accessory fissures in the liver ranges from 6% - 56%, as reported by cadaveric studies, which is much higher than the prevalence of 25% on CT scans. Despite reporting many morphological variations in the liver by various cadaveric studies worldwide, the imaging studies are very few. Radiological imaging of patients undergoing liver surgery is a routine preoperative investigation. Despite there are several occasions where liver variations occur as a surprise during surgery which reflects on the diagnostic capacity of CT. Materials & Methods 60 cadaveric livers removed and stored in 10% Formalin were studied for all morphological abnormalities. The same livers were analysed separately by anatomists using gross examination and radiologists using plain CT imaging. The radiologist reporting the CT findings was blinded from the gross examination findings. The data obtained by both methods were compared by using appropriate statistical methods and the diagnostic accuracy of CT was estimated. Results The common surface morphological variations detected were accessory fissures, accessory lobes, pons hepatis and multilobed caudate and quadrate lobes. Out of the total 89 accessory fissures identified by gross examination, only 73 could be detected by CT scan, thus the sensitivity of CT scan in detecting accessory fissures is 82%. Similarly, sensitivity of CT scan in detecting accessory lobes, pons hepatis, and multilobed caudate and quadrate lobe was calculated as 64% (low), 81% and 19% (very low) respectively. Thus, plain CT has variable sensitivity depending on the morphological variation. Conclusions This study has highlighted some of the diagnostic inaccuracies that may arise during plain CT examination of liver in a person posted for liver surgery or a person with abdominal trauma. Contrast-enhanced CT scans can circumvent many of these problems. Lack of awareness of these issues may affect the normal course of treatment and prognosis in such patients.