Lymphangiomatosis is the presence of multiple lymphangiomas, which are rare, congenital and present as multicystic lesions in neck and axilla, and less commonly, in thorax and abdomen. We present a rare case of disseminated lymphangiomatosis, presenting with complaints of postprandial abdominal fullness and early satiety in the late fourth decade of life. Our case highlights that indeed lymphangioma can present at any age and not necessarily in early childhood and should be kept as a differential for slow-growing intraabdominal swellings. 

A 39-year-old male, presented with post-prandial fullness of abdomen for one month with no history of fever, cough, jaundice, hematemesis, malena, bleeding per rectum, or trauma. The patient was operated upon for the same complaints 2 years back, where exploratory laparotomy with splenectomy and mesenteric cyst excision was done. Histopathology showed lymphangiohemangioma of the spleen and cystic lymphangiectasia respectively. On examination, the patient was vitally stable. A small 6 x 5 cm cystic swelling in the left side of the neck, between the two heads of the sternocleidomastoid muscle, not moving on deglutition, non-tender with positive transillumination was present. On abdominal inspection, there was a diffuse swelling over the abdomen with a previous laparotomy scar present. A soft, non-tender, cystic, intra-abdominal swelling was palpable measuring approximately 15 x 25 cm which was not moving with respiration. Percussion showed dull note and normal bowel sounds were heard on auscultation. Ultrasound examination confirmed the cystic hygroma in the neck. Computed tomography (CT) scan of the Neck, Thorax, and Abdomen (Figure 1a) was done which showed a) Multiple well-defined, thin walled, non-enhancing unilocular and multilocular cysts of variable sizes scattered throughout the abdomen and pelvis in the intraperitoneal as well as retroperitoneal locations. Two of these cysts were extremely large measuring 15 x 12 x 22 cm and 16 x 12 x 24 cm causing mass effect (Figure 1c) on abdominal organs and mild left hydronephrosis and upper hydroureter. b) Post splenectomy status, c) Multiple hepatic cysts (Figure 1b), d) multi-locular cysts in the left supraclavicular region – cystic hygroma (Figure 2) and e) Cysts along the right lower intercostal and bilateral internal mammary vessels, largest measuring 2 x 3 cm. 99mTc-Sulphur colloid Lymphoscintigraphy injected in the web spaces of  bilateral feet showed normal ascent of tracer through lymphatic channels of bilateral lower limbs as well as abdominal lymphatics. There was no communication with the cisterna chyli. The patient was posted for exploratory laparotomy. Drainage of the cysts with partial cystic wall excision was done (Figure 3). Cystic hygroma of the neck was aspirated under sonographic guidance post-operatively. Fluid analysis showed 569 RBCs/HPF, few neutrophils, occasional lymphocytes with few cystic macrophages against a necrotic background, and no atypical cells. HPE (Figure 4) was consistent with lymphangiomatosis. The patient tolerated the procedure well. There were no fresh complaints at the 6-month follow-up.

Lymphangiomas are uncommon, hamartomatous, congenital, focal proliferations of well-differentiated lymphatic tissue that present as multicystic or spongelike accumulations.¹ The classification of lymphangiomas lacks a standard clear definition and universal application due to the nature of lymphangiomas, which represent a clinicopathologic continuum. They can be classified as 1) Capillary (simple) lymphangiomas are slightly elevated or sometimes pedunculated, small, well-circumscribed cutaneous lesions of 1-2 cm diameter that occur in the head, neck, and axillary subcutaneous tissues. 2) Cavernous lymphangiomas i.e. Cystic hygromas are large, well-circumscribed, multiloculated cystic spaces lined by the endothelium that contain a significant intervening connective tissue stroma. They are typically found in the neck, axilla, and retroperitoneum. They can occasionally be up to 15 cm in diameter and may fill the axilla or produce gross deformities around the neck. The tumour margins are indistinct and unencapsulated, making definitive resection difficult.² Lymphangiomatosis describes the presence of multiple lymphangiomas. In more than three-quarters of cases, multiple organs are involved. Most lymphangiomas (95%) occur in the neck & axilla with abdominal lymphatic malformations estimated in ~ 5% of cases. It is seen more frequently in the mesentery, followed by retroperitoneum, abdominal viscera, and lung.^3,4