Four patients with generalized lymphangiomatosis presenting with chylothoraces are described. All four had bone involvement, two had involvement of the spleen, and one of the pericardium. The diagnosis was confirmed by typical radiology, histology, and in three patients by immunohistochemistry. Treatment was mainly palliative. Three patients died within 1/2 to three years of presentation.
Single incision stoma siting proves a ready, robust and reliable technique for diversion ileostomy and colostomy with a minimum of operative trauma for the patient.
The development of intramural intestinal gas may indicate a serious postoperative complication and therefore any radiological indication of such "pneumatosis intestinalis" (PI) in an unwell patient after surgery should put the clinical team on high-alert. However immediate recourse to relook laparotomy may not be always necessary and, further, in some cases may possibly accelerate the deterioration especially if it proves to be non-therapeutic. Careful and close clinical monitoring, as is described in this clinical report, may allow discriminative identification of those in whom this finding is in fact transient and therefore benign and who therefore can be successfully treated without operative re-intervention. We describe the presenting features and background scenario of PI early after laparoscopic total colectomy for medically refractory, severe ulcerative colitis and detail the critical postoperative decision pivots.
The diagnosis of lymphangiomatosis can often be made on clinical grounds with the aid of plain radiography. In children with intractable chylothorax computed tomography (CT) of the thorax may reveal the presence of a mediastinal mass but frequently in lymphangiomatosis will not demonstrate any specific features. Abdominal CT, on the other hand, may suggest the diagnosis by the association with intraabdominal lymphangioma.
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