Simple lymphangioma to generalized lymphatic anomaly: role of imaging in disclosure of a rare and morbid disease.

Manoj Joshi,Dilip S Phansalkar

doi:10.1155/2015/603859

Manoj Joshi, Dilip S Phansalkar

Open Access

https://doi.org/10.1155/2015/603859

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Abstract

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.

Highlights

Generalized lymphatic anomaly (GLA), earlier referred to as diffuse lymphangiomatosis, is a rare congenital lymphatic malformation originating from persistence of dilated lymphatics at 14–20th week of life
Later, parents decided against further treatment. Rodenberger first described this condition as generalized cystic lymphangiomatosis in 1828 [3]
It has been mentioned as generalized lymphatic anomaly in literature and its imaging features have been differentiated with other complex lymphatic anomalies, like Gorham-stout disease (GSD) and Kaposiform lymphangiomatosis (KLA) [4]

Summary

Introduction

Generalized lymphatic anomaly (GLA), earlier referred to as diffuse lymphangiomatosis, is a rare congenital lymphatic malformation originating from persistence of dilated lymphatics at 14–20th week of life. 65% of affected patients are infants and young children [1]. Prognosis is poor and major cause of morbidity and mortality is deterioration of lung function and infection [2]. Exact incidence of this condition is not known due to its rarity

Case Presentation

Discussion