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Mondor's disease of the breast resulting from jellyfish sting.

OBJECTIVE: To present two cases of Mondor's disease of the breast resulting from jellyfish stings in Western Australia. CLINICAL FEATURES: A 30-year-old Caucasian woman presented with a palpable thickened cord in her right breast. The straightness of the cord suggested a thrombosed lymphatic. A 50-year-old Caucasian woman presented with an obvious palpable cord extending most of the length of her left breast. Mammography demonstrated no abnormality. Both women reported having been stung by jellyfish a month earlier. INTERVENTION AND OUTCOME: As Mondor's disease is a benign, self-limiting disease, the patients were reassured and reviewed routinely. In each case, the condition settled spontaneously over a period of several weeks. CONCLUSION: Jellyfish stings should be recognised as an unusual variant of the numerous causes which have been described for Mondor's disease.

Adult↗

PFAPA syndrome: a rare cause of periodic fever.

PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, adenopathy) is characterized by abrupt onset of fever, malaise, aphthous stomatitis, tonsillitis, pharyngitis and cervical adenopathy. The age of onset of the disease is four years, with a range of 6 months to 7 years. The syndrome is sporadic and nonhereditary. Long-term sequelae do not develop. A nine- year-old boy presented with sore throat, fever and oral aphthae. After taking a throat culture, he was prescribed oral antipyretic and was called for a follow-up visit the next day. As the culture result was negative, he was given a single dose prednisolone with the suspected diagnosis of PFAPA. Twenty-four hours later his temperature was 36.8 degrees C, with all his complaints regressed. Twenty-two days later the patient was again admitted to our hospital with the same complaints. Again, single dose oral prednisolone was given after a throat culture. On the next day the patient was free of all symptoms and the culture was again normal. To our knowledge this is the first PFAPA case report from Turkey in the literature.

Abnormalities, Multiple↗

[Congenital pulmonary lymphangiectasis].

Congenital pulmonary lymphangiectasis is a rare cause of severe respiratory distress in the newborn period and most of these patients die, usually within the first 24 hours of life. It may present as an isolated anomaly or it may be associated with other congenital abnormalities, most commonly cardiac in origin, as is the case of total obstruction of the pulmonary venous drainage. Congenital pulmonary lymphangiectasis may be also associated with lymphatic anomalies of other portions of the body or with other non-lymphatic, non-cardiac congenital malformations. Chest radiographs show more commonly that the lungs are hyperexpanded and present a diffuse interstitial reticulonodular pattern. The differential diagnosis from the radiological standpoint should include total anomalous pulmonary venous drainage and pneumonia. We present the case of a newborn infant with congenital pulmonary lymphangiectasis who died within the first 24 hours of life, emphasizing clinical, radiological and pathological findings.

Female↗

[Are lateral neck cysts true derivatives of cervical lymph nodes?].

For decades the origin of the branchial cyst has been attributed to an abnormal involution of derivatives of the second branchial cleft. More recently the cyst has been thought to be the result of tonsillitis and lymphadenitis, stimulating a lymphatic spread of squamous epithelium from the tonsil to the regional lymph nodes. We reviewed the histopathologic reports of 81 branchial cysts and 19 fistulas and re-examined 30 cysts and 5 fistulas by light microscopy. Similarities of morphological features between the epithelium of tonsillar crypts and of the branchial cysts were striking and emphasize the close relationship of the tonsils (2nd branchial pouch) to branchial cysts and fistulas. These observations and a review of the literature support the well-known branchiogenic origin of branchial cysts.

Branchial Region↗

Immunobiological and pharmacological properties of thymus factor X (TFX). I. Biological and pharmacological activity.

Detailed investigations on pharmacological properties of biologically active thymus extract were performed. Its acute and chronic toxicity was determined. Influence on the circulatory and the respiratory systems as well as on the parenchymatous and the smooth muscle organs, development of fetuses and reproductive functions were evaluated. Preparation TFX proved to be well tolerated and its acute action was manifested only at high doses injected intravenously. Its effect on the smooth muscle organs and its teratogenic activity were determined in the long-term experiments. It was assumed that it can be introduced in clinical use excluding women in the reproductive period.

Abnormalities, Drug-Induced↗

Isosulfan blue causes factitious methemoglobinemia in an infant.

A 9 month-old girl was given subcutaneous isosulfan blue to outline lymphatic channels during surgery for thoracic duct ligation. Her pulse oximetry values rapidly declined to a nadir of 85%, 35 min after dye injection. Arterial blood gases revealed methemoglobinemia ([MetHb] = 6.5%). Although abnormal pulse oximetry has already been reported in association with isosulfan blue, methemoglobinemia has not previously been reported. The absorption spectrum for isosulfan blue was determined and when superimposed on that of methemoglobin it was found to have an overlying peak. Interference by the dye was postulated to have caused the abnormal methemoglobin result. The phenomenon was simulated in vitro by adding isosulfan blue to whole blood, and analysing it in the same blood gas analyser as was used for the case, as well as another for comparison. One blood gas analyser reported elevated methemoglobin concentration and the other did not. The samples were sent to a reference laboratory using a chemical method to detect methemoglobin to confirm that the elevated methemoglobin level was spurious.

Coloring Agents↗

Antibody guided lymphangiography in the staging of cervical cancer.

Iodine-123-labelled tumour associated monoclonal antibody HMFG2 was administered intralymphatically at a time that cannulation of pedal lymphatic vessels was performed for standard lymphangiography in 6 patients with cervical cancer. Gamma camera images were taken at 2 h and 24 h after injection of antibody and at a similar time that X-ray lymphangiography was performed. Five out of the 6 standard lymphangiograms were reported as normal whilst one showed definite evidence of metastasis. Antibody guided analysis of the abnormal lymphangiogram confirmed the presence of abnormality. Also, marked non-specific uptake of antibody was seen on all lymphangiograms. It is concluded that, in order for monoclonal antibody guided lymphangiography to become a useful adjunct to standard lymphangiography, further improvements are needed to reduce non-specific uptake by normal lymphatics.

Adult↗

The terminal pathway of the lymphatic system of the human heart.

Aantomical dissections in 9 human cadavers revealed the terminal pathway of the lymphatic system of the left ventricle to be constituted mainly by channels emptying into the right angulus venosus (junction of the internal jugular and subclavian veins) at the base of the right side of the neck. This observation has clinical implications because it has been shown that a sampling of cardiac lymph provides the best method for analyzing myocardial metabolic abnormalities and that drainage of cardiac lymph alleviates the myocardial changes produced by ischemic injury.

Adult↗

Physiology and pathophysiology of pleural fluid turnover.

Tight control of the volume and composition of the pleural liquid is necessary to ensure an efficient mechanical coupling between lung and chest wall. Liquid enters the pleural space through the parietal pleura down a net filtering pressure gradient. Liquid removal is provided by an absorptive pressure gradient through the visceral pleura, by lymphatic drainage through the stomas of the parietal pleura, and by cellular mechanisms. Indeed, contrary to what was believed in the past, pleural mesothelial cells are metabolically active, and possess the cellular features for active transport of solutes, including vesicular transport of protein. Furthermore, the mesothelium was shown, on the basis of recent experimental evidence, both in vivo and in vitro, to be a less permeable barrier than previously believed, being provided with permeability characteristics similar to those of the microvascular endothelium. Direct assessment of the relative contribution of the different mechanisms of pleural fluid removal is difficult, due to the difficulty in measuring the relevant parameters in the appropriate areas, and to the fragility of the mesothelium. The role of the visceral pleura in pleural fluid removal under physiological conditions is supported by a number of findings and considerations. Further evidence indicates that direct lymphatic drainage through the stomas of the parietal pleura is crucial in removing particles and cells, and important in removing protein from the pleural space, but should not be the main effector of fluid removal. Its importance, however, increases markedly in the presence of increased intrapleural liquid loads. Removal of protein and liquid by transcytosis, although likely on the basis of morphological findings and suggested by recent indirect experimental evidence, still needs to be directly proven to occur in the pleura. When pleural liquid volume increases, an imbalance occurs in the forces involved in turnover, which favours fluid removal. In case of a primary abnormality of one ore more of the mechanisms of pleural liquid turnover, a pleural effusion ensues. The factors responsible for pleural effusion may be subdivided into three main categories: those changing transpleural pressure balance, those impairing lymphatic drainage, and those producing increases in mesothelial and capillary endothelial permeability. Except in the first case, pleural fluid protein concentration increases above normal: this feature underlies the classification of pleural effusions into transudative and exudative.

Humans↗

Back pain--a presentation of metastatic testicular germ cell tumours.

9 patients (21%) from a total of 42 young men with testicular germ cell malignancy had back pain as a major presenting symptom; 2 had back pain without unilateral testicular enlargement. Time to correct diagnosis from first visit to a doctor was significantly longer in those with back pain than in those with testicular symptoms. All patients with back pain had para-aortic node metastases. Young men with back pain should be examined for testicular disease and should also be asked about past testicular abnormalities.

Adult↗

[Clinical amyotrophic lateral sclerosis syndromes in gammapathies: clinical, electrophysiologic and histoimmunologic aspects].

Eleven patients with motor neuron disease associated with a monoclonal gammopathy were studied. One patient had a previously known multiple myeloma. In the other patients neurological symptoms preceded diagnosis of the gammopathy by one month to 8 years. They were 5 multiple myeloma, 1 macroglobulinemia, 1 chronic lymphatic leukemia, 1 lymphoma and 3 benign monoclonal gammopathies. EMG and motor conduction velocities results were consistent with anterior horn cell disease but sensory conduction studies were abnormal in seven cases out of eight. Superficial peroneal nerve biopsy performed in all patients confirmed peripheral nerve involvement. The relations between gammopathy, clinical symptoms and electrophysiological data are discussed. The interest of sensory conduction studies in every patient with motor neuron disease is emphasized.

Aged↗

[Lymphography in childhood (author's transl)].

Fifty-one lymphograms were performed in children; the indications, technique and results are discussed. Indications are the search for retroperitoneal involvement in lymphogranulomatosis, lympho-sarcoma and reticulum cell sarcoma; in the search for metastases from malignant tumours, particularly abdominal neuroblastoma, soft tissue sarcomas of the abdomen and lower extremities, testicular tumours and malignant melanomas and finally, for primary lymph-oedema and lymphangiomas. Technique is the same as for adults, but requires particular manual dexterity. Children under six years require general anaesthesia. Amongst 28 children with malignant lymphomas, pathological changes in the retroperitoneal lymph nodes were found in seven. In six, this resulted in a change of the staging. Five out of 16 lymphograms in children with malignant tumours showed evidence of lymph node metastases. All six lymphangiograms in children with lymphoedema and lymphangiomas were abnormal.

Abdominal Neoplasms↗

Chylous pleural effusions simulating leukemic infiltrate associated with thoracoabdominal disease and surgery in infants.

Surgical procedures and diseases in childhood involving damage to or obstruction of the thoracic duct and related lymphatic channels can cause pleural effusions that are often chylous and can be massive. The morphology of fluid cytology in these cases can prompt a mistaken diagnosis of a hematolymphoid malignancy. We describe 6 infants, aged 3 days to 5 months, in whom thoracoabdominal disease and/or surgical procedures interfered with lymphatic drainage of the thorax and pleural space. In 5 cases, the clinically significant chylous pleural effusion was postoperative; in 1 case, it was related to congenital abnormalities. After chest tube drainage, the effusion resolved in all cases. Fluid cytology revealed a predominant population of small to medium immature-appearing cells with moderate amounts of basophilic cytoplasm without granules. The nuclei were often irregular or slightly lobulated, with evenly dispersed chromatin and occasionally prominent nucleoli. Flow cytometry performed on these cases revealed a heterogeneous population of lymphoid cells, most of which showed phenotypic markers typical for mature T cells. A complete patient history and ancillary studies in such cases can correctly characterize the cells to reach an accurate diagnosis.

Abnormalities, Multiple↗

Autopsy case of congenital pulmonary lymphangiectasis.

Congenital pulmonary lymphangiectasis (CPL) is a rare anomaly. We report a female infant born at 39 weeks of gestation who was found to have CPL. Cyanosis and tachypnea were noted immediately after birth, and, at room air, PaO2 was 30.7 mmHg, PaCO2 was 82.5 mmHg and pH was 7.12. The infant's symptoms did not improve even with the initiation of artificial ventilation. Chest X-ray film showed cotton-like infiltrates in both lungs and an air-leak surrounding the cardiac shadow. Echocardiography study showed no abnormality. The neonate died 3 days after birth due to hypoxemic cardiac failure. At autopsy, the pleural surface contained numerous dilated vessels that had the appearance of lymphatics. Microscopic features of the lungs were marked lymphatic dilatation of the perivascular, subpleural and interlobular areas. Lymphangiectasis was found in the liver, kidney, pancreas, thyroid and alimentary canals, such as the esophagus, stomach and rectum. Patients with lymphatic dilatations in extrapulmonary organs have mild pulmonary involvement and symptoms and a better prognosis. However, a few cases of CPL with lymphatic dilatations in extrapulmonary organs and an aggressive course, such as the present case, have been reported. The clinical behavior and prognosis of CPL depend on the extent of pulmonary involvement of the lymphatic dilatations regardless of systemic lymphatic dilations.

Fatal Outcome↗

Non-parasitic chyluria (presenting with urinary retention).

Two cases of non-parasitic chyluria are presented each presenting with retention of urine. Abnormal communications in both cases have been demonstrated lymphangiographically. The thoracic ducts were patent and there was no evidence of filariasis. Surgical removal of the anomalous dilated lymphatics was carried out, the patients being symptom-free at 7 and 10 years. The two cases seem to have lymphatic malformations which may be the basis of the disorder.

Adult↗

Isolated primary chylopericardium.

A 16-year-old man was found to have an enlarged cardiac silhouette. Primary chylopericardium was diagnosed when pericardiocentesis yielded the characteristic milky-white fluid. The thoracic duct was easily identified by giving milk and butter and an injection of ethylene blue immediately before the operation. Intraoperative thoracic ductography showed no abnormal findings. Mass ligation of the thoracic duct above the diaphragm and partial pericardiectomy were successfully performed through a right thoracotomy approach. In addition, many of the lymphatics were ligated above the diaphragm. The right thoracotomy approach was a useful method for resection and ligation of the thoracic duct just above the diaphragm. Follow-up showed no accumulation of pericardial fluid or pleural effusion.

Adolescent↗

Physiotherapy results in a baby with congenital lymphedema: a follow-up study.

Physiotherapy results of a 6.5-month-old baby with congenital lymphedema in the lower limbs are presented in this study. Her motor developmental level and reflexes were evaluated and test results did not show any abnormal findings. Assessment of limb volume included circumferential and volumetric measurements, and photographs showing the changes in macroscopic view are presented. The physiotherapy program consisted of manual lymphatic massage, remedial exercises, multilayered inelastic compression bandaging, meticulous skin care and education of parents. The treatment lasted for 2.5 months, five days per week. Evaluations were done immediately after treatment and six months after treatment. The evaluations showed reduction in swelling after the treatment and this reduction continued during the follow-up period. It can be concluded that this physiotherapy program reduces the swelling in a baby with congenital lymphedema. Further studies are required in order to see the effectiveness of this therapy program in a greater number of subjects.

Female↗