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Berdon syndrome

Berdon syndrome is a disorder of the newborn, most prevalent in females, characterised by constipation and urinary retention, microcolon, giant bladder (megacystis), intestinal hypoperistalis, hydronephrosis, and dilated small bowel. The pathological findings consist of an abundance of ganglion cells in both dilated and narrow areas of the intestine. It is a familial disturbance of unknown aetiology. Authors disagree whether inheritance is autosomal dominant or recessive. more...

Bacterial endocarditis
Bacterial food poisoning
Bacterial meningitis
Bacterial pneumonia
Bangstad syndrome
Bardet-Biedl syndrome
Bardet-Biedl syndrome
Bardet-Biedl syndrome
Bardet-Biedl syndrome
Barrett syndrome
Barth syndrome
Basal cell carcinoma
Batten disease
Becker's muscular dystrophy
Becker's nevus
Behcet syndrome
Behr syndrome
Bell's palsy
Benign congenital hypotonia
Benign essential tremor...
Benign fasciculation...
Benign paroxysmal...
Berdon syndrome
Berger disease
Bicuspid aortic valve
Biliary atresia
Binswanger's disease
Biotinidase deficiency
Bipolar disorder
Birt-Hogg-Dube syndrome
Bloom syndrome
Blue diaper syndrome
Blue rubber bleb nevus
Body dysmorphic disorder
Bourneville's disease
Bowen's disease
Brachydactyly type a1
Bright's disease
Brittle bone disease
Bronchiolotis obliterans...
Bronchopulmonary dysplasia
Brown-Sequard syndrome
Brugada syndrome
Bubonic plague
Budd-Chiari syndrome
Buerger's disease
Bulimia nervosa
Bullous pemphigoid
Burkitt's lymphoma
Cavernous angioma

Berdon, et al. in 1976 first described the condition in five female infants, two of whom were sisters. All had marked dilatation of the bladder and some had hydronephrosis and the external appearance of prune belly. The infants also had microcolon and dilated small intestines.


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Lymphoepithelial cyst in the lung - selected reports
From CHEST, 4/1/03 by Rakesh V. Alva

A Case Report

An unusual endobronchial presentation of lympho-epithelial cysts (LECs) is described in a HIV-seropositive patient. The bilateral infrahilar cysts had followed an apparently benign course for 2 years. Bronchoscopy revealed an endobronchial mass occluding the anterior basilar segment of the left lower lobe. Biopsy resulted in emptying of the cyst and showed the typical pseudostratified columnar epithelium with intraepithelial lymphocytes of an LOC. This diagnosis should be considered in patients with HIV infection and pulmonary cysts on CT.

Key words: bronchoscopy; cavity; HIV infection; lung mass; lung nodule

Abbreviation: LEC = lymphoepithelial cyst


Lymphoepithelial cyst (LEC) refers to a cyst that is lined by metaplastic squamous or columnar epithelium permeated by lymphoid cells. The cyst wall is formed by lymphoid tissue with germinal centers. LECs occur commonly in the salivary glands (1) of adults and children with HIV infection. Less frequently, they have been described in the thymus of individuals with HIV infection. (2) The literature describes only one case of such a lesion in the lung of an adult patient with HIV infection. (3) We report an unusual endobronchial presentation of LEC associated with infrahilar masses.


A 42-year-old Hispanic woman presented with gastroenteritis following a viral prodrome and was found to have bilateral infrahilar masses on her hospital admission chest radiograph (Fig 1). She had a history of mild intermittent asthma, occasional smoking, and "benign cysts" removed from her parotid gland and axilla 2 years ago at another hospital. She had no history suggestive of opportunistic infections and tested negative for purified protein derivative. Physical examination and laboratory values were normal. Chest CT showed bilateral infrahilar multiloculated cysts measuring 5 cm and 3.8 cm, respectively, on the right and left (Fig 2, 3). Similar hypodensities were also noted in the right paraspinal region and in the left adrenal gland. Pulmonary function tests showed mild reversible obstructive disease with FE[V.sub.1] 1.57 L (75% predicted and 67% of FVC). Fiberoptic bronchoscopy revealed a smooth-walled mass occluding the anterior basilar segmental bronchus of the left lower lobe. The initial attempt to biopsy the mass resulted in the efflux of 20 mL of yellow serous fluid and disappearance of the endobronchial mass. Fluoroscopy showed the left-sided cyst to be partially collapsed. Multiple biopsy specimens were obtained from the cyst wall and the lung. Chest CT showed a decrease in size of the left-sided cyst that had developed an air-fluid level (Fig 4). Cultures of the cyst fluid for bacterial, mycobacterial, and fungal organisms and stains for acid-fast bacilli were negative. Fluid chemistry revealed an lactate dehydrogenase of 439 U/L, glucose of 17 mg/dL, and triglycerides of 7 mg/dL. Fluid cytology showed scant cellularity and no evidence of malignancy.


Histologic examination of the biopsy specimens demonstrated a fragmented cyst wall that was lined in part by pseudostratified columnar epithelium (Fig 5) and in part by metaplastic non-keratinizing squamous epithelium. There were numerous intraepithelial lymphocytes (immunohistochemically admixed CD20-positive B cells and CD3-positive T cells). The cyst wall consisted of fibrous tissue as well as reactive lymphoid tissue with germinal centers. Alveolar and bronchial tissue failed to reveal any significant lymphocytic infiltrate. These findings were consistent with an LEC.


The pathology report of the parotid gland cyst removed 2 years earlier also demonstrated cystic lymphoid hyperplasia. Imaging studies of the chest at the time that the parotid gland cyst was removed reported enlarged cystic infrahilar lymph nodes bilaterally.

The patient was subsequently tested and found to be HIV positive. Her CD4 cell count was 244/[micro]L, CD8 count was 674/[micro]L, the ratio was 0.36, and viral load was 13,982 copies per milliliter. She has since been started on combination antiretroviral treatment.


A spectrum of unusual lymphoproliferative disorders in the lung has been reported in adults with HIV infection. (4-7) LECs have been well described in the salivary glands and less commonly in the thymus, thyroid, and pancreas. The LEC seems to be a manifestation of an HIV-induced autoimmune-like syndrome. (8) LECs have been associated with the diffuse infiltrative lymphocytosis syndrome, which is characterized by bilateral parotid swelling, cervical lymphadenopathy, CD8 lymphocytosis, and infiltration of various viscera by CD8 lymphocytes. (9) In the lungs, this often takes the form of lymphocytic interstitial pneumonitis.

In our review of the literature, we found that pulmonary LECs have been described only once in an adult with HIV infection. (3) The described patient was a 32-year-old woman who presented with bilateral hilar cysts, one of which was resected. The authors suggested that the lymphoid component of the cyst developed from an intrapulmonary peribronchial lymph node or from peribronchial lymphoid tissue that is frequently present in smokers, and the metaplastic nonkeratinizing epithelium lining the cyst developed from bronchial gland epithelium. HIV p24 core protein was found to be present in most germinal centers. They speculated that the dendritic reticular cells in the germinal centers may act as a reservoir to maintain the disease and that follicular hyperplasia might be induced either directly by HIV or by other viruses such as Epstein-Barr virus or cytomegalovirus.

A right-sided suprahilar LEC has been described in a 10-year-old child with HIV infection who presented with recurrent postobstructive pneumonia. The authors suggested if the patient is asymptomatic and no complications such as pneumonia develop, then it might be prudent to observe rather than surgically remove such a lesion. (10) Our case is unique in several ways: (1) the endobronchial location of the LEC is unusual for any type of lung cyst, (2) bronchoscopy was diagnostic without the need for surgical resection, (3) the lesion followed a benign course for at least 2 years, and (4) the adrenal gland was presumably also involved.

The differential diagnosis of cysts in the lung is broad and includes bronchogenic cyst, trauma, cystic adenomatoid malformation, lymphoma, parasitic infections such as echinococcal cyst, and autoimmune disease. (11) LEC, although rare, should be considered in the differential diagnosis of lung cysts in patients with HIV infection, particularly if they have parotid cysts or other unusual lymphoproliferative disorders.

Although diagnosis of LEC is based on pathology, chest CT may suggest this diagnosis. Further clarification on the characteristics of the cyst fluid chemistry and cytology may enable us to make this diagnosis with needle aspiration--either bronchoscopic or by a transthoracic approach.

Our patient had the lesions for at least 2 years without any overt symptoms. When LECs occur in the parotid gland, aspiration often leads to recurrence of the lesions. While excision remains the definitive treatment for parotid LECs, they have been shown to respond to combination antiretroviral therapy with or without corticosteroids. (12) In patients whose disease is refractory to such medication, external radiation therapy may be an option, (13) although xerostomia and the risk of a secondary malignancy make this option unattractive. Doxycycline sclerotherapy is another conservative approach. (14)

Based on our experience and the description of LECs in other locations, it seems unlikely that LEC in the lung is a premalignant lesion, so monitoring for lymphoma is probably unnecessary. If an accurate diagnosis can be made without resection, therapy should be based on symptoms.

In summary, we describe an HIV-infected patient with pulmonary LEC presenting as infrahilar masses with an endobronchial component. This diagnosis must be considered in patients with HIV infection when cystic lesions are discovered in the hila or lung adjacent to the tracheobronchial tree, particularly in a patient with parotid cysts or other unusual lymphoproliferative disorders.


(1) Shaha AR, DiMaio T, Webber C, et al. Benign lymphoepithelial lesions of the parotid. Am J Surg 1993; 166:403-406

(2) Leonidas JC, Berdon WE, Valderrama E, et al. HIV infection and multilocular thymic cysts. Pediatr Radiol 1996; 198:377-379

(3) Marie B, Labouyrie E, Scheid P, et al. HIV type 1 in an unusual cystic lymphoepithelial lesion of the lung. Histopathology 1997; 31:83-86

(4) Ettensohn DB, Mayer KH, Kessimian N, et al. Lymphocytic bronchiolitis associated with HIV infection. Chest 1988; 93:201-202

(5) Travis WD, Cecil HF, Devaney KO, et al. Lymphoid pneumonitis in 50 adult patients infected with HIV: lymphocytic interstitial pneumonitis vs nonspecific interstitial pneumonitis. Hum Pathol 1992; 23:529-541

(6) Guillon JM, Autran B, Denis M, et al. HIV related lymphocytic alveolitis. Chest 1988; 94:1264-1270

(7) McGuinness G, Scholes JV, Jagirdar JS, et al. Unusual lymphoproliferative disorders in nine adults with HIV or AIDS: CT and pathologic findings. Radiology 1995; 197:59-65

(8) Chetty R. HIV associated lymphoepithelial cysts and lesions: morphological and immunohistochemical study of the lymphoid cells. Histopathology 1998; 33:222-229

(9) Itescu S, Brancato LJ, Buxbaum J, et al. A diffuse infiltrative CD8 lymphocytosis syndrome in HIV infection: a host immune response associated with HLA-DR5. Ann Intern Med 1990; 112:3-10

(10) Brudnicki AR, Levin TL, Slim MS, et al. HIV associated (non-thymic) intrathoracic lymphoepithelial cyst in a child. Pediatr Radiol 2001; 31:603-605

(11) Case records of the Massachusetts General Hospital. N Engl J Med 2001; 344:1701-1708

(12) Craven DE, Duncan RA, Stram JR, et al. Response of parotid lymphoepithelial parotid cysts to antiretroviral treatment in HIV infected adults. Ann Intern Med 1998; 128:455-459

(13) Beitler JJ, Smith RV, Brook A, et al. Benign parotid hypertrophy in HIV+ patients: limited late failures after external radiation. Int J Radiat Oncol Biol Phys 1999; 45:451-455

(14) Suskind DL, Tavill MA, Handler SD. Doxycycline sclerotherapy of benign lymphoepithelial cysts of the parotid: a minimally invasive treatment. Int J Pediatr Otorhinolaryngol 2000; 52:157-161

* From the Departments of Medicine (Drs. Alva, Rosenthal, and Aldrich), Radiology (Dr. Haramati), and Pathology (Dr. Madan), Montefiore Medical Center and Albert Einstein College of Medicine, Bronx, NY.

Manuscript received May 17, 2002; revision accepted August 13, 2002.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (e-mail:

Correspondence to: Rakesh V. Alva, MD, Pulmonary Medicine Division, Montefiore Medical Center, 111 East 210th St, Bronx, NY 10467; e-mail:

COPYRIGHT 2003 American College of Chest Physicians
COPYRIGHT 2003 Gale Group

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