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Intestinal lymphangiectasia is one of the protein-losing enteropathies. Herein, a case with intestinal lymphangiectasia responded well to diet and octreotide is presented.
We study two authentic cases of protein-losing enteropathy, the diagnosis of which was facilitated using Given M2A videocapsule endoscopy. The first case corresponded to a primary intestinal lymphangiectasia confirmed by jejunum biopsies and the second one to a protein-losing enteropathy with lymphatic abnormalities secondary to a chronic constrictive pericarditis. In the first case, the mucosa of jejunum presented with a diffuse oedematous aspect, whitish villi, white curved lines probably related to submucosal dilated lymphatics and lacteal juice. In the second case, capsule endoscopy showed oedematous aspect of jejunum mucosa associated with white curved lines similar to those observed in the first case. Videocapsule endoscopy is useful in cases of protein-losing enteropathy to identify presence of intestinal lymphangiectasia and to specify their localisation after ruling out other disorders liable to induce protein-losing gastrointestinal syndrome.
Misdiagnosis of sexual abuse may arise in cases of vulvar and/or perianal diseases such as lichen sclerosus et atrophicus, Behcet's syndrome, bullous diseases, contact dermatitis, or neoplastic lesions. We present the case of a 7-year-old girl who was referred by her general paediatrician to the local hospital for perianal fissures and swelling of the labia majora. A report to the judicial authorities was made, for suspected sexual abuse, and the patient was transferred to the department of paediatric surgery. Here, histopathologic examination of vulvar and rectal biopsies revealed multiple non-caseating and non-confluent epithelioid-gigantocellular granulomas, consistent with a diagnosis of Crohn's disease, with cutaneous involvement of the genitalia. In cases of suspected sexual abuse, examination of children should be performed by a specialist in legal medicine in collaboration with a gynaecologist or paediatric surgeon. If the patient is hospitalised and the question of protection does not arise, physicians should exclude dermatological diseases before reporting to the judicial authorities.
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BACKGROUND: Lipedematous alopecia and lipedematous scalp are two similar unusual conditions mostly affecting healthy black women. OBJECTIVE: The purpose of our study was to report three cases of this condition with emphasis on clinical and histologic findings, and to review the literature on the subject. METHODS: The study includes clinical, echographic, and histologic findings of 3 patients, complemented with a literature review. RESULTS: Two cases of lipedematous alopecia and one of lipedematous scalp in 3 white women had echographic confirmation of an increased subcutaneous layer. The presence of ectatic lymphatic vessels in the two cases with hair loss was particularly emphasized. CONCLUSIONS: Our findings suggest a lessened role of racial factors, but confirms the sex implications in these related conditions, and stress the potential significance of lymphangiectatic vessels in the development of alopecia in these patients.
A 55-year-old white man with a history of hypertension, fibromyalgia, and colonic polyps presented with unrelenting plantar warts on his hands and feet for the past 4 years. He was otherwise healthy and without a history of recurrent infections. Physical examination was unremarkable except for extensive warts on his hands and feet. Pertinent laboratory findings included hypoalbuminemia, hypogammaglobulinemia, and lymphopenia most severely affecting CD4(+) T cells. Testing for HIV infection was negative. This clinical and laboratory presentation suggested a combined humoral and cellular immunodeficiency syndrome that could be best explained by loss of lymphocytes, immunoglobulins, and other serum proteins. Additional immunologic testing revealed a marked reduction in peripheral blood naive (CD4(+)CD45RA(+)) T cells. A 24-hour stool collection showed a markedly elevated alpha(1)-antitrypsin level. These findings were most consistent with the diagnosis of intestinal lymphangiectasia, a type of protein-losing enteropathy associated with hypoalbuminemia, hypogammaglobulinemia, and lymphopenia, characterized by a preferential loss of naive CD4(+) T cells into the gastrointestinal tract. This case illustrates the importance of considering intestinal loss of immunoglobulins and lymphocytes in the differential diagnosis of the adult patient who presents with laboratory evidence of a combined humoral and cellular immunodeficiency. It also underscores the diagnostic utility of the clinical immunology laboratory and how flow cytometry, in particular, can contribute to an understanding of pathogenic mechanisms.
Making a correct diagnosis of a primary immunodeficiency disease is crucial for the selection of proper therapy. Although many cases go undiagnosed, there are also many instances of incorrect diagnosis that result in years of inappropriate treatment and failure to implement beneficial treatment. This article summarizes 2 actual cases in which incorrect diagnoses led to recommendations of unwarranted high-risk or costly treatments. Had the physicians chosen tests of immune function rather than relying on immunoglobulin levels or cell counts, they would have arrived at the true diagnoses.
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Isolated fetal hydrothorax is an uncommon finding on routine prenatal ultrasound and is associated with a variable prognosis. We believe this is the first report of a patient whose antenatal hydrothorax was associated with both congenital diaphragmatic hernia and congenital pulmonary lymphangiectasia. This rare combination caused significant problems in diagnosis and subsequent treatment.
INTRODUCTION: Primary intestinal lymphangiectasia (Waldmann's disease) lead to a protein-losing enteropathy due to lymph leak into intestinal tract. EXEGESIS: A 28-year-old woman presented a bilateral lower limb lymphedema. Laboratory examination showing lymphopenia, hypoalbuminemia, hypogammaglobulinemia suggested the diagnosis of primary intestinal lymphangiectasia. Gastroscopy was normal and second duodenum biopsies were negative. Videocapsule endoscopy gave evidence of intestinal lymphangiectasia of the small bowel. CONCLUSION: Videocapsule endoscopy may be proposed to confirm intestinal lymphangiectasia and to precise their localization when gastroscopy is not conclusive.
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Postoperative chylothorax compromises nutrition, immune function, coagulation, and fluid status. We report rapid short-term suppression of chylothorax by octreotide in an infant after surgery for complex congenital heart disease complicated by lymphangiectasia.
BACKGROUND: Pulmonary venous obstruction (PVO) induces pulmonary arterial hypertension, as well as pulmonary venous hypertension, and jeopardizes the repair of cardiac lesions. METHODS: Four cases of congenital mitral stenosis and 4 cases of cor triatriatum (Lucas type A), ages ranging from 2 months to 16 years, were histologically examined on pulmonary vasculature. Histometrical analysis was performed on medial thickness and intimal changes of both pulmonary arteries and veins. For comparison, the examination of pulmonary vasculature in ventricular septal defect (VSD) cases was also performed. RESULTS: Medial thickening and intimal fibrosis, in both pulmonary arteries and veins with widespread lymphangiectasia, were characteristic vascular changes of PVO cases. Medial thickness of pulmonary arteries was correlated with preoperative pulmonary arterial pressure (PAP) (r = 0.77, p = 0.03 for systolic PAP), and greater than that of VSD cases. Medial thickness of pulmonary veins was also greater in PVO cases. Intimal fibrosis of pulmonary arteries and veins was seen extensively at the advanced ages, whereas no plexiform lesions or more advanced stages of pulmonary vascular disease were present. CONCLUSIONS: Congenital PVO induced progressive medial thickening and intimal fibrosis in pulmonary arteries and veins accompanied by lymphangiectasia. However, no plexiform lesions or more advanced stages of pulmonary vascular disease were present, which may explain the reversibility of pulmonary hypertension due to congenital PVO.
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Intestinal lymphangiectasia is a disorder presenting as enteric protein loss through the dilated lymphatics without mucosal ulceration. To determine the double-contrast radiographic features and to assess the significance of them, five patients with intestinal lymphangiectasia were examined using single- and double-contrast small intestinal studies. The double-contrast examinations demonstrated clearly the main radiographic findings of smooth nodular protrusions, thickening of the mucosal folds, with no evidence of mucosal ulceration. Compared with the single-contrast study, smooth nodular protrusions were seen more often and in more widespread segments, particularly in the duodenum, on double-contrast study. Thickening of the mucosal folds was revealed similarly by both methods. Double-contrast study appears to be worthwhile to demonstrate the characteristic radiographic findings of this disease.
We describe the CT appearances of lymphangiectasia in the pelvis of a 21-year-old male who presented with fever, abdominal pain and swelling of the right thigh. Previous histological examination of a biopsy specimen from the patient's thigh had demonstrated lymphangiectasia.
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