Hennekam lymphangiectasia syndrome.

2011

Lakshminarayana G, Mathew A, Rajesh R, Kurien G, Unni VN.

Source

Department of Nephrology, Amrita Institute of Medical Sciences & Research Center, Kochi, India.

Abstract

Hennekam lymphangiectasia syndrome is a rare disorder comprising of intestinal and renal lymphangiectasia, dysmorphic facial appearance and mental retardation. The facial features include hypertelorism with a wide, flat nasal bridge, epicanthic folds, small mouth and small ears. We describe a case of a multigravida with bad obstetric history and characteristic facial and dental anomalies and bilateral renal lymphangiectasia. To our knowledge this is the first case of Hennekamlymphangiectasia syndrome with anodontia to be reported from India.

Full text article

Indian Journal of Nephrology

Ocular coherence tomography in lymphangiectasia.

Oct 2011

Daya SM, Papdopoulos R.

Source

Corneo-Plastic Unit and Eye Bank, Queen Victoria Hospital, East Grinstead, West Sussex, United Kingdom. sdaya@centreforsight.com

Abstract

PURPOSE:

To report the aid of ocular coherence tomography (OCT) in diagnosing conjunctival lymphangiectasia and correlate clinical and pathological findings.

METHODS:

Single interventional case report. A 64-year-old man presented with a 2-year history of ocular discomfort, tearing, and a gradually enlarging lesion on the conjunctiva of his left eye.

RESULTS:

Slit-lamp biomicroscopy revealed localized conjunctival swelling temporally in the left eye, with the lesion protruding between the upper and lower eyelids. Visante (Carl Zeiss-Meditec, Jena, Germany) OCT revealed clear fluid-filled spaces demarcated by septae within the elevated conjunctiva. The lesion was excised, and histopathology of the specimen showed features consistent with lymphangiectasia.

CONCLUSIONS:

OCT, a valuable tool in imaging of the anterior segment, is also useful in evaluation of conjunctival pathology.

PubMed

Conjunctival lymphangiectasia: a report of 11 cases and review of literature.

Mar 2012

Welch J, Srinivasan S, Lyall D, Roberts F.

Source

Department of Ophthalmology, Ayr Hospital, Ayrshire and Arran National Health Service, Ayrshire, Scotland, United Kingdom.

Abstract

Conjunctival lymphangiectasia is an uncommon clinical condition in which there is dilatation of lymphatic channels in the bulbar conjunctiva. Conjunctival lymphangiectasia is a rarely appreciated ocular surface disorder that typically occurs as a secondary phenomenon in response to local lymphatic scarring or distal obstruction.

Conjunctival lymphangiectasia can either be unilateral or bilateral with focal or diffuse bulbar chemosis. We present 11 cases of biopsy-proven conjunctivallymphangiectasia. Of the 11 cases, 3 presented with bilateral diffuse bulbar chemosis, 1 had diffuse unilateral chemosis, and the remaining 7 presented with focal (<90°) bulbar chemosis.

Three of these cases had co-existing pterygium, and one case presented with focal bulbar chemosis and a conjunctival keratin horn. All underwent surgical excision of the involved conjunctiva, either with no graft (n = 6), combined with amniotic membrane transplant (n = 3), or combined with conjunctival autograft (n = 2).

Survery of Ophthalmology

Pediatric Renal Lymphangiectasia: Importance of Recognition and Accurate Renal Imaging. Pediatric Renal Lymphangiectasia: Importance of Recognition a

Pediatric Renal Lymphangiectasia: Importance of Recognition and Accurate Renal Imaging.

Feb 2012

Vasquez E, Kallen RJ, Shore RM, Lindgren BW.

Source

Department of Urology, Loyola University Stritch School of Medicine, Maywood, Illinois.

Abstract

Renal lymphangiectasia is a rare diagnosis. It is also referred to as renal lymphangioma, renal lymphangiomatosis, peripelvic lymphangiectasia, renal peripelvic multicystic lymphangiectasia, and hygroma renale. The presentation varies, and the diagnosis depends on accurate radiologic interpretation. We present the radiologic images of a pediatric patient who was diagnosed with renal lymphangiectasia after being evaluated for suspected nonaccidental trauma to emphasize the importance of identifying this entity by the characteristic radiologic findings.

Elsevier