Surgical Therapy of Segmental Jejunal, Primary Intestinal Lymphangiectasia.

Dec 2012

[Article in German]

Kneist W, Drescher DG, Hansen T, Kreitner KF, Lang H.

Source

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

Abstract

 

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.

Thieme Journal

Lympangiectasia of the vulva accompanying congenital lymphedema

Lympangiectasia of the vulva accompanying congenital lymphedema
April 2009

M Ihsan Okur1, Rüştü Köse2, A Mustafa Yıldırım1, Bengü Çobanoğlu3

Dermatology Online Journal 15 (4): 13 1. Assistant Professor, Department of Plastic and Reconstructive Surgery, Fırat Medical Center, Firat (Euphrates) University, Elazig, Turkey2. Assistant Professor, Department of Plastic and Reconstructive Surgery, Harran University Medical Center, Şanlıurfa, Turkey. rkose@harran.edu.tr3.
Assistant Professor, Department of Pathology

Abstract

Lymphangiectasia is a benign condition with multiple dilated lymph vessels in the dermis. Vulvar localization is rare and mostly depends on disturbing the lymphatic flow. We present a patient with vulvar lesions and ipsilateral congenital lower extremity lymphedema. Surgical excision eliminated the lymphangiectasia and improved the appearance of the edematous vulva.

Introduction

Vulvar lymphangiectasia is not usually seen as a result of congenital lymphedema. Lymphangioma circumscriptum, a subtype of lymphangioma, is mainly a congenital malformation that presents in infancy but may be seen at any age. An acquired lesion, especially one related to the obstruction of lymphatic circulation, is defined as lymphangiectasis; however it is histologically indistinguishable from lymphangioma circumscriptum.
Lymphangiectasia of the vulva is a rare disease and usually reported following surgery and radiotherapy, performed because of a genital malignant tumor [1]. Long-standing Crohn disease with fistulae and tuberculosis lymphadenitis are the other associated conditions [1, 2, 3, 4]. Primary lymphedema is an unusual cause of vulvar lymphangiectasia. We present a case of vulvar lymphangiectasia with ipsilateral congenital lower extremity lymphedema.

Case Report

A 27-year-old woman was referred for treatment of a skin lesion on her right labium majus. The plaque had appeared initially at the age of 19. Sometimes vesicles arose on the surface from which clear and occasionally bloody fluid oozed. She underwent a few bouts of vulvar cellulitis. Four years prior, she had been seen in another dermatology clinic and was given the clinical diagnosis of "infected lymphangioma of the vulva," which was treated with antibiotics. Congenital lympedema along the entire right leg was noted. The lymphedema (Fig. 1) was apparent soon after birth and she regularly used a pressure garment. Genital examination revealed a red and skin-colored plaque with shining, clustered papules on the right labium majus and ipsilateral vulvar edema (Fig. 2). This edema deformed the appearance of the vulva, which was the main complaint of the patient. The growth was excised, including the edematous subcutaneous tissue, under general anesthesia. A negative pressure drain was placed and tissues were sutured.

The histopathological examination of the excised tissue showed many thin-wall lumenal structures with irregular size and shape, lined by a monolayer of endothelium in the upper dermis (Fig. 3). Postoperative recovery was uneventful and the surgical wound healed well.

After the surgery, the symmetry of the vulva was improved. Although a thin scar remained along the labium majus, the aesthetic outcome was good (Fig. 4) and the patient was satisfied. The patient was followed up for 31 months. Recurrence and infection were not observed during this period.

Comment

Vulvar lymphangiectasia is mainly associated with obstruction of the lymphatic flow. The lesions typically arise approximately 7-15 years after lymph node dissection and radiotherapy of the genitalia [1, 5, 6, 7, 8]. De novo acquired lesions without lymphatic disturbance are less frequently seen. In some cases, lymphangiectasia appears following lymphedema of the vulva or lower extremity [3, 4, 5, 6, 9]. However, the condition may arise without adjacent lymphedema [3].

Although deep lymphangiomas are seen in primary lymphedema cases, reports detailing subsequent lymphangiectasia of vulva are very few [2, 10]. Buckley and Barnes report a 35-year-old patient who had lymphedema since the age of 14 and underwent two cellulitis attacks. Thereafter, lymphangiectasia on the vulva appeared in the same year [10]. Another patient with late onset lymphedema in the right leg also presented with lymphangiectasia on the labium majus [2]. The lesions emerged three years after the lymphedema.

The mechanism behind development of the vulvar lymphangiectasia in our patient accompanied by congenital lymphedema is probably similar. A 13-year-old boy has been described who presented exhibited scrotal lymphangiectasia with congenital contralateral leg lymphedema [11].

He also experienced recurrent scrotal infections after the lymphangiectasia appeared. Our patient had congenital lymphedema and suffered episodes of cellulitis before and after the vulvar lymphangiectasia appeared.

Lymphangiectasia may be actually expected in primary lymphedema patients because lymphatic obstruction is an important etiologic factor in its etiology. The cause of the higher rate in acquired lymphatic damage may be because of the rapidly increasing pressure when there is a sudden blockage of the superficial lymphatic system. In addition, recurrent cellulitis further damages lymphatic vessels, increases lymphatic pressure, and induces formation of lymphangiectasia in lymphedema patients.

Lymphangiectasia on the vulva can be easily misdiagnosed, especially as viral warts [1-5, 7, 8, 12]. Oozing vesicules or papules and recurrent infections should bring into mind the possibility of lymphangiectasia.

Excisional surgery and carbon dioxide laser are two major treatment modalities in addition to cryotherapy, electrocoagulation, and sclerosing agent injection [13]. Whereas carbon dioxide laser only vaporizes the lesion superficially, it may seal the underlying lymph vessels and diminish recurrence [9, 14]. The laser therapy can be repeated if any recurrences occur. Delayed wound healing, scars, and even keloid formation may be seen after laser therapy [1, 14]. Excisional surgery eliminates the abnormal subcutaneous lymph vessels and cisterns, and corrects of the aesthetic appearance of the edematous vulva. It is the only plausible choice in the therapy of advanced disease [13].

DermatologyOnline

A case of Hennekam syndrome presenting with massive pericardial effusion.]

A case of Hennekam syndrome (lymphangiectasia) presenting with massive pericardial effusion.
Turk Kardiyol Dern Ars. 2008 Jul
Nişli K, Oner N, Kayserili H, Ertuğrul T.
Department of Pediatrics, Istanbul Medicine Faculty of Istanbul University, Istanbul, Turkey. kemalnisli@yahoo.com.

Hennekam syndrome is an autosomal recessive disease characterized by intestinal lymphangiectasia accompanied by severe lymphedema of the limbs, genitalia, and face, and learning difficulties. A 38-month-old boy was admitted with breathing difficulty. He had facial abnormalities and preputial hyperplasia consistent with Hennekam syndrome. Lymphangiography showed lymphedema in the left eye and right foot. Teleradiography showed cardiomegaly and echocardiography showed massive pericardial effusion. He first underwent pericardiocentesis for the removal of pericardial effusion, but pericardial tube drainage was required upon recurrence of effusion. On the fifth day, the drain was removed because of significant decrease in the drainage.

Full Text Article PDF

Acquired lymphangiectases and breast cancer

Acquired lymphangiectases and breast cancer
Actas Dermosifiliogr. 2007 Jun

Valdés F, Peteiro C, Toribio J.
Unidad de Dermatología. Hospital da Costa. Burela, Lugo. Spain. Fernando.Valdes.Tascon@sergas.es.

Acquired lymphangiectases represent superficial lymphatic dilatations caused by a wide range of processes. Many cases reported in the literature develop in patients with upper limb lymphedema secondary to mastectomy, radiotherapy, keloids or scleroderma. Clinically they consist of traslucent vesicles in a chronic lymphedematous area. Histologically they are characterized by the presence of dilated spaces with flattened endotelial cells in the papillary dermis. All these cases have a good prognosis and there have not been any reports of malignant transformation. We describe a 67-year-old woman that showed multiple papules along her left upper limb associated with lymphedema. She had undergone a mastectomy followed by radiotherapy fourteen years before due to a breast cancer. Laboratory and radiological exams were within normal limits. The cutaneous lesions showed characteristic clinical and histological features of lymphangiectases and they progressively resolve in several weeks without any treatment.

Actos dermo-sifiliograficas

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. sobolos@spark.net.gr

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.

Springerlink

PMID: 11261684 [PubMed - indexed for MEDLINE]