Tetralogia de Fallot Associada a Artéria Subclávia Direita Aberrante. Implicações Clínicas / Tetralogy of Fallot Associated with Aberrant Right Subclavian Artery. Clinical Implications

Resumo Desde a primeira descrição da tetralogia de Fallot (ToF) em 1671 por Niels Stensen e em 1888 por Étienne-Louis Arthur Fallot, vários trabalhos relataram essa anomalia juntamente com suas variantes e anomalias cardiovasculares concomitantes. A artéria subclávia direita aberrante (ASDA) é a anomalia do arco aórtico mais comum. Diferentemente da artéria subclávia esquerda aberrante, a ocorrência de ASDA em pacientes com ToF só foi relatada casuisticamente. Apresentamos dois pacientes de ToF com ASDA. É importante notar que o conhecimento da coexistência das duas anomalias tem pontos muito práticos durante correções endovasculares ou cirúrgicas de defeitos cardíacos congênitos (inclusive ToF).

Abstract Since the first description of Tetralogy of Fallot (ToF) in 1671 by Niels Stensen and in 1888 by Étienne-Louis Arthur Fallot, numerous papers have reported on this anomaly, along with its variants and concomitant cardiovascular anomalies. Aberrant right subclavian artery (ARSA) is the most common anomaly of the aortic arch. Different from the left aberrant subclavian artery, occurrence of ARSA in ToF-patients has only casuistically been reported so far. The present study reports on two ToF-patients with ARSA. It is important to note that knowledge of the coexistence of both anomalies has highly practical points during surgical or endovascular corrections of congenital heart defects (including ToF).

Removal of Esophageal Variceal Bands to Salvage Complete Esophageal Obstruction

Esophageal varices develop in almost half of the patients with cirrhosis, and variceal hemorrhage constitutes an ominous sign with an increased risk of mortality. Variceal banding is considered an effective and mostly safe measure for primary and secondary prophylaxis. Although adverse events related to banding including dysphagia, stricture formation, bleeding, and ligation-induced ulcers have been described, complete esophageal obstruction is rare, with only 10 reported cases in the literature. Among those cases, 6 were managed conservatively; 1 patient had esophageal intraluminal dissection from an attempt to remove the bands using biopsy forceps but ultimately recovered with conservative management. Three patients developed strictures following removal of the bands, requiring repeated sessions of dilation therapy. We report on a patient who developed absolute dysphagia and complete esophageal obstruction after variceal banding. We successfully used the endoloop cutter hook to release the bands intact and restore luminal integrity.

Esophageal Obstruction Caused by the Solidification of Residue of an Enteral Feeding Formula

The nutritional support of acutely ill patients is very important and early enteral nutrition is recommended. Feeding via the nasogastric route is used widely for its ease of access if the patient cannot maintain volitional food intake. If the position of the tip or side holes of the nasogastric tube (NGT) is above the gastroesophageal junction, there is the possibility of retention and solidification of the administered enteral feeding formula in the esophagus. Therefore, the tip of the NGT should be checked carefully; a chest radiograph to confirm its position can be considered, especially in patients with a higher risk of aspiration and gastroesophageal reflux. In addition, careful consideration of the risk factors that can trigger esophageal obstruction is required when feeding patients in the intensive care unit. This paper describes two unusual cases of esophageal obstruction caused by the solidification of residue of an enteral feeding formula.

Timpanismo em bovinos, secundário à obstrução esofágica por citrus limon (limão siciliano) / Bloat in cattle secondary to esophageal obstruction by citrus limon (sicilian lemon)

Timpanismo ruminal consiste na distensão acentuada do rúmen e retículo, devido a incapacidade do animal em expulsar gases produzidos durante o processo fisiológico da fermentação. O timpanismo pode ser ocasionado de forma primária, por formação de espuma estável, ou secundária, devido a anormalidades funcionais e/ou físicas que interferem na eructação. Nesse trabalho, são descritos os aspectos epidemiológicos, clínicos e anatomopatológicos da ocorrência de timpanismo secundário em bovinos, decorrente da obstrução esofágica aguda por limões sicilianos. Cinco bovinos, de um lote de 210, foram afetados. Os bovinos eram suplementados com resíduo de tangerina (Citrus reticulata) no cocho e na última carga desse subproduto, havia limões sicilianos inteiros misturados ao resíduo. Os cinco animais afetados eram da raça Aberdeen Angus e tinham entre 12-24 meses de idade. Todos apresentaram sinais clínicos caracterizados principalmente por mucosas cianóticas, grave timpanismo, desconforto abdominal, acentuada dispneia e taquicardia, atonia ruminal, desidratação, decúbito e morte. O curso clínico variou entre 24 a 48 horas. Na necropsia, os cinco bovinos apresentavam grave obstrução esofágica por limões nas porções: cranial (logo após a laringe [1/5]), porção medial (entrada do tórax [1/5]) e final (próximo ao cárdia [3/5]). Nas áreas de oclusão, observou-se extensa necrose e ulceração da mucosa esofágica. O conteúdo ruminal dos bovinos estava seco e misturado com limões inteiros. No esôfago de dois animais havia linha de timpanismo. As lesões histológicas eram visualizadas principalmente no esôfago, na região da obstrução, onde se evidenciaram alterações degenerativas, necróticas e ulcerativas acentuadas no revestimento epitelial.

Ruminal bloat (acute timpany) in ruminants is a marked rumen-reticular distension which results from more gas being produced during the physiologic process of fermentation than is eliminated by eructation. This condition may present itself as either primary timpany due to the formation of stable foam or secondary timpany resulting from functional and/or physical disturbances compromising eructation. This paper describes the epidemiological, clinical, and anatomopathological aspects of acute timpany in cattle secondary to esophageal obstruction by sicilian lemons. Five out of a herd of 210 cattle were affected. Cattle were supplemented with tangerine (Citrus reticulata) residues in a trough. In the last batch of this feed there were whole sicilian lemons mixed with the tangerine residue. The five affected cattle were 12-24 month-old Aberdeen-Angus. All of the five presented clinical signs characterized mainly by cyanotic mucous membranes, severe timpany, abdominal discomfort, marked dyspnea and tachycardia, ruminal atony, dehydration, recumbence and death. Clinical course lasted from 24 to 48 hours. Necropsy findings in the five affected cattle were similar and included complete esophageal obstruction by lemons in the cranial esophagus (immediately cranial to the larynx [1/5]) medial esophagus (at the thoracic inlet [1/5]) and caudal esophagus (close to the cardia [3/5]). At the occluded sites the esophageal mucosa was necrotic and ulcerated. Ruminal content was dried and admixed with whole lemons. In the esophagus o two affected bovine a bloat line was observed. Histological lesions were observed mainly in the esophagus at the sites of obstruction and consisted of marked degenerative, necrotic and ulcerative changes in the esophageal mucosal epithelium.

A Case of Multiple Myeloma with Multiple Plasmacytomas pesented as Upper Airway and Esophageal Obstruction / 대한혈액학회지

Herein, we report a case of multiple myeloma with multiple plasmacytomas of thyroid, lung, lymph nodes and adrenal gland, who presented as upper airway and esophageal obstruction with vocal cord palsy. An 80-year-old women complained of dysphagia, hoarseness and anterior neck mass. Ultrasonography of the neck showed huge inhomogeneous solid mass involving left thyroid lobe and isthmus with several lymphadenopathy along the both upper deep cervical chain. Chest CT scanning revealed 4x3 cm sized, low density mass in posterobasal segment of the right lower lobe with both lower paratracheal lymphadenopathy, and abdominal CT scanning revealed 4.5x4.5 cm sized round low density mass in left adrenal gland. Serum and urine electrophoresis was normal and immunofixation of serum and urine was negative. Bone marrow smear and a bone scintigram revealed no abnormality, but iliac bone marrow biopsy revealed infiltration of abnormal plasma cells. Histologic and immunohistochemical examinations of the thyroid and lung mass revealed monoclonal proliferation of plasma cells of kappa light chain. After local irradiation of the neck with parenteral dexamethasone injection, anterior neck mass was much diminished in size, but 2 weeks later she had a pneumonia with fatal outcome.

Silicone - Covered Self - Expandable Metal Stents for the Palliation of Malignant Esophageal Obstruction and Esophagorespiratory Fistulas / 대한소화기내시경학회지

BACKGROUND: Esophageal cancer can induce progressive dysphagia and occasionally develop esophagorespiratory fistulas. Surgically incurable disease is present in over 60% of patients at the time of presentation and in such a means, relief of dysphagia is one of the important treatment modes in the majority of these patients. Implantation of stents is widely used for improvement of dysphagia and sealing of esophagorespiratary fistulas. METHODS: Silicone-covered self-expandable metal stents were used in 19 consecutive patients with malignant esophageal obstruction (n=14) or esophageal obstruction with esophagorespiratory fistulas (n=5). RESULTS: The stents were successfully inserted in all patients. Dysphagia improved in 18 of 19 patients (95%). All fistulas were sealed and symptoms due to bronchial aspiration disappeared. Complications occurred during follow-up including chest pain (11 patients), aspiration pneumonia (4 patients), reflux esophagitis (2 patients), stent migration (2 patients), tumor overgrowth (2 patients) and hemorrhage (1 patient). Three of the 4 patients with aspirtion pneumonias and a patient with hemorrhage suffered from dysfunction of upper esophageal sphincter after implantation of stents in upper esophageal carcinoma. But, Tumor ingrowth and perforation did not occur. Two patients died of massive hemorrhage and respiratory failure caused by aspiration pneumonia. CONCLUSION: These results suggest that implantation of silicone-covered self-expandable metal stents is a rapid and effective procedure for the palliative treatment of malignant esophageal obstruction and esophagorespiratory fistulas.