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

Friday, March 30, 2007

Enteroclysis in older children and teenagers

Enteroclysis in older children and teenagers
Pediatr Radiol. 2007 Mar 22

Kurugoglu S,
Korman U,
Adaletli I,
Selcuk D.
Department of Radiology, Cerrahpasa Medical Faculty, Istanbul University, Kocamustafapasa, 34303, Istanbul, Turkey,

BACKGROUND: Enteroclysis (EC) has been widely and successfully used for evaluation of the small bowel in adults for about 30 years. However, despite recently improved intubation and examination techniques, in many paediatric radiology centres it is still not the preferred conventional barium study for the evaluation of small bowel pathology in children.

OBJECTIVE: To share our 10 years of experience and review the feasibility of EC in 83 older children and teenagers, in terms of both technique and pathological findings.

MATERIALS AND METHODS: Between 1996 and 2006, EC was performed by the standard technique described by Herlinger to 83 children between 7 and 18 years of age. The indication for the study was jointly decided by the paediatric radiologist and the clinician. None of the examinations was converted to follow-through studies because of patient refusal or technical failure. Morphological changes, mucosal abnormalities, luminal abnormalities, perienteric structures, the location of the disease, indirect findings regarding the bowel wall and functional information were evaluated.

RESULTS: All the children tolerated the procedure without difficulty. Out of 83 patients, 63 had abnormal findings. The spectrum of diagnoses were Crohn disease (n = 23), nonspecific enteritis (n = 10), malabsorption (n = 8), intestinal tuberculosis (n = 6), intestinal lymphoma (n = 5), Peutz-Jegher syndrome (n = 3), adhesions (n = 2), Behcet disease (n = 2), back-wash ileitis due to ulcerative colitis (n = 2), common-variable immune deficiency (n = 1) and lymphangiectasis (n = 1).

CONCLUSION: EC can easily be performed in children over 7 years of age and when performed using a correct technique it shows high diagnostic performance without any complications in the evaluation of small bowel diseases in older children and teenagers.


Labels: , , , , , ,

Wednesday, March 21, 2007

Congenital pulmonary lymphangiectasia: CT and pathologic findings.

J Thorac Imaging. 2004 Jan

Nobre LF, Muller NL, de Souza Junior AS, Marchiori E, Souza IV.

Department of Radiology, Universidade Federal de Santa Catarina, Florianopolis, Santa Catarina, Brasil.

Congenital pulmonary lymphangiectasia is a rare disease characterized by dilation of lymphatic channels without lymphatic proliferation. The disease is seen almost exclusively in infancy and early childhood.

The authors report 2 cases of pulmonary lymphangiectasia. The patients were a 12- and a 25-year-old male who presented with progressive dyspnea and hemoptysis. The diagnosis was confirmed by open lung biopsy.

The radiographic findings consisted of bilateral reticular changes, peribronchial cuffing, and bilateral pleural effusions. High-resolution CT demonstrated extensive bilateral septal and peribronchovascular interstitial thickening, areas of ground-glass attenuation, and bilateral pleural effusions. The histologic findings consisted of ectatic and tortuous lymphatic channels in the interlobular septa, bronchovascular sheaths, and pleura.

Journal of Thoracic Imaging

Labels: , , ,

Monday, March 19, 2007

End-stage renal disease in a patient with congenital lymphangiectasia and lymphedema.

End-stage renal disease in a patient with congenital lymphangiectasia and lymphedema.

Sombolos KI,
Papachillea AI,
Natse TM,
Gogos KI,
Pavlidis GO,
Barboutis KA,
Mavromatidis KS.
Renal Unit G.H. G. Papanikolaou, Thessaloniki, Greece.

Congenital lymphangiectasia with lymphedema is a disorder constituting the main defect in many different genetic syndromes. Herein we describe a 23-year-old male patient with congenital lymphangiectasia and severe lymphedema of the right leg, scrotum, and abdominal wall, who presented with end-stage renal disease, presumably due to cystic renal lymphangiectasia, and is undergoing chronic hemodialysis treatment.


PMID: 11261684 [PubMed - indexed for MEDLINE]

Labels: , ,