Information on all the types of lymphangiectasia, including intestinal, pulmonary, renal, cutaneous (skin). Sponsored by Pat O'Connor

Sunday, October 26, 2008

Lymphangiectasia of small intestine presenting as intussusception.

Lymphangiectasia of small intestine presenting as intussusception.
Indian J Pathol Microbiol. 2008 Jul-Sep

Katoch P, Bhardwaj S.
Department of Pathology, Government Medical College, Jammu, Jammu and Kashmir, India.
pervezkatoch@rediffmail.com.

Intussusception is defined as telescoping of a segment of gastrointestinal tract into an adjacent one. In small children, it is the commonest cause of intestinal obstruction. More than 90% of childhood intussusceptions are idiopathic. We report a rare case of localized small intestinal lymphangiectasia, presenting as intussusception in a 6-month-old male child. The child presented with features of acute intestinal obstruction for which he was later operated. The gross examination of excised ileocecal mass revealed intussusception. Histopathologic examination revealed lymphangiectasia of small intestine, which acted as a lead point for ileocecal intussusception. Postoperative period was uneventful.

Pathology and Microbiology

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Survival in primary congenital pulmonary lymphangiectasia with hydrops fetalis

Survival in primary congenital pulmonary lymphangiectasia with hydrops fetalis

Fetal Diagn Ther. 2008

Laje P, Wilson RD, Guttenberg M, Liechty KW.
Department of General Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
laje@email.chop.edu

The recent advances in neonatal and pediatric intensive care have changed the outcome of patients with congenital pulmonary lymphangiectasia of different types, including those cases with early neonatal symptoms. However, the patients who present the most severe form of the disease, manifested by in utero hydrops (including those treated by in utero thoracoamniotic shunting to relieve the mediastinal compression), have had an unvaryingly fatal ending in all published reports, with most cases dying before birth, and the few born alive dying during the first days of life. We present a patient with primary congenital pulmonary lymphangiectasia complicated by hydrops fetalis, who was treated in utero, survived the neonatal period after intensive medical and surgical support, and was discharged home at the age of 2 months. She is currently 6 months old, and has minimal signs or symptoms of chronic lung disease. The different aspects of the management of congenital pulmonary lymphangiectasia are discussed in this report, together with a review of the literature. Copyright 2008 S. Karger AG, Basel.

Karger

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