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

Monday, December 31, 2012

A case of enteric muco-submucosal elongated polyp.

A case of enteric muco-submucosal elongated polyp.



Department of Gastroenterology and Hepatology, Yamaguchi University Graduate School of Medicine.


A woman in her seventies was admitted because of black stool. Esophagogastroduodenoscopy and colonoscopy revealed no bleeding lesions. Balloon endoscopy showed a long and slender polyp. The polyp was covered with normal mucosa and accompanied with ulcerations. The long polypoid lesion was found to be located in the duodenojejunal flexure by a small bowel series. We performed endoscopic polypectomy. The polyp was composed of edematous mucosa and submucosa with dilated blood vessels and lymphangiectasia. The histologic findings of resected specimen were compatible with colonic muco-submucosal elongated polyp. The polyp was considered to be classified as enteric muco-submucosal elongated polyp.

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Hennekam Syndrome  


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Wednesday, December 26, 2012

Hennekam lymphangiectasia syndrome

Hennekam lymphangiectasia syndrome

Oct-Dec 2011 - Just released PubMed


Key Words:  Facial and dental anomalies, renal lymphangiectasia, Hennekam lymphangiectasia syndrome

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 Hennekam lymphangiectasia syndrome with anodontia to be reported from India.


Hennekam Lymphangiectasia syndrome is a rare disorder characterized by presence 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. This syndrome thought to be due to developmental disorder of the lymphatics. The synonym for this disease is multiple congenital anomaly/mental retardation (MCA/MR) syndrome.

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Liquid Nitrogen Cryotherapy for Conjunctival Lymphangiectasia: A Case Series

Liquid Nitrogen Cryotherapy for Conjunctival Lymphangiectasia: A Case Series

Dec 2009 - Just released



To report a case series of conjunctival lymphangiectasia treated with liquid nitrogen cryotherapy.


A 1.5-mm Brymill cryoprobe was applied in a double freeze-thaw method after an incisional biopsy of a portion of the conjunctiva in patients with conjunctival lymphangiectasia. Freeze times were 1 to 2 seconds with thawing of 5 to 10 seconds between treatments. Patients were reexamined at 1 day, 2 weeks, 3 months, 6 months, and yearly following cryotherapy.


Five eyes of 4 patients (3 male and 1 female) with biopsy-proven conjunctival lymphangiectasia underwent liquid nitrogen cryotherapy. The average patient age was 53 years. Ocular examination revealed large lymphatic vessels that were translucent and without conjunctival injection. Subjective symptoms included epiphora, ocular irritation, eye redness, and occasional blurred vision. After treatment with liquid nitrogen cryotherapy, the patients’ symptoms and signs resolved within 2 weeks. Lymphangiectasia recurred twice in one patient, at 1 and 3 years postoperatively. In another patient, lymphangiectasia recurred at 6 months. The average time to recurrence in these 3 eyes was 18 months. Average length of follow-up was 24.5 months for all subjects.


Liquid nitrogen cryotherapy may be an effective surgical alternative in the treatment of conjunctival lymphangiectasia. Cryotherapy may need to be repeated in some instances.
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Friday, December 14, 2012

Surgical Therapy of Segmental Jejunal, Primary Intestinal Lymphangiectasia.

Surgical Therapy of Segmental Jejunal, Primary Intestinal Lymphangiectasia.

Dec 2012

[Article in German]


Klinik und Poliklinik für Allgemein- und Abdominalchirurgie, Universitätsmedizin der Johannes-Gutenberg-Universität, Mainz.


Key Words: primary intestinal lymphangiectasia - chylaskos - protein-losing gastroenteropathy - small bowel resection

Primary intestinal lymphangiectasia (PIL) is a protein-losing, exsudative gastroenteropathy causing lymphatic obstruction. Diagnosis depends on clinical examination and histological findings. Conservative treatment modalities include a low-fat diet and enteral nutritional therapy in order to reduce enteric protein loss and to improve fat metabolism. Other treatment options consist of administration of antiplasmin or octreotide to lower lymph flow and secretion. We report on a 58-year-old patient who underwent exploratory laparotomy due to a worsening physical status, recurrent chylaskos and leg oedema under conservative dietary therapy. Intraoperative findings showed a typical PIL of the jejunum about 20 cm distal to the Treitz's ligament. Histological examinations confirmed this diagnosis. One year after segmental small bowel resection (105 cm) with end-to-end anastomosis the patient is healthy, free of symptoms, has gained weight and his serum protein level has increased. Intraabdominal ascites and leg oedema (lymphedema) have not reoccurred since.

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Sunday, December 02, 2012

Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia.

Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia.

Nov 2012


Siham Al Sinani, Yusria Al Rawahi, Hamed Abdoon, Child Health Department, Sultan Qaboos University Hospital, Muscat 123, Sultanate of Oman.


A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE. Few syndromes are associated with PIL; Hennekam syndrome (HS) is one of them. The principal treatment for PIL is a high protein, low fat diet with medium chain triglycerides supplementation. Supportive therapy includes albumin infusion. Few publications have supported the use of octreotide to diminish protein loss and minimize hypoalbuminemia seen in PIL. There are no publications on the treatment of PIL with octreotide in patients with HS. We report two children with HS and PLE in which we used octreotide to decrease intestinal protein loss. In one patient, octreotide increased serum albumin to an acceptable level without further need for albumin infusions. The other patient responded more dramatically with near normal serum albumin levels and cessation of albumin infusions. In achieving a good response to octreotide in both patients, we add to the publications supporting the use of octreotide in PIL and suggest that octreotide should be tried in patients with PIL secondary to HS. To the best of our knowledge, this is the first case report on the use of octreotide in HS-associated PIL.

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