Chylous ascites developing after open thoracoabdominal aortic aneurysm repair in a patient with marfan syndrome

Abstract Chylous ascites is the pathologic accumulation of chylous fluid in the peritoneal cavity, caused by lymphomas, metastatic malignancies, and abdominal surgeries, rarely due to surgical trauma of the cisterna chyli or its major branches. A 24-year-old man with history of Marfan syndrome presented to our hospital with abdominal distention, abdominal pain, fluid in the incision region, and weakness. He had underwent an elective open aneurysm repair surgery nine days before for thoracoabdominal aortic aneurysm. Computed tomography revealed massive fluid collection in the abdominal cavity, which was drained surgically. He was diagnosed with chylous ascites and was discharged after conservative treatment.

Tratamiento endovascular exitoso de aneurisma de aorta torácica secundario a infección de catéter de arteria umbilical en un recién nacido pretérmino / Successful endovascular treatment of thoracic aortic aneurysm secondary to infection of the umbilical artery catheter in preterm infants

Los aneurismas aórticos en la población pediátrica son poco frecuentes. El uso de catéteres de arteria umbilical en neonatos se ha asociado a infección y en algunas oportunidades a formación de aneurismas aórticos. La reparación quirúrgica de estos aneurismas es una forma de terapia; sin embargo, la intervención percutánea con stents pudiese proveer una vía alternativa de tratamiento con menores complicaciones. El objetivo de este reporte es dar a conocer el alcance terapéutico de un procedimiento híbrido, en el que el desarrollo de la técnica quirúrgica y percutánea en conjunto ofrece otra alternativa terapéutica menos invasiva que la cirugía vascular abierta, para la reparación de aneurismas aórticos o de sus ramas principales. Caso clínico: Recién nacido de pretérmino, 30 semanas, peso 1.335 g. Se instaló catéter en arteria umbilicar que se retiró a los 14 días por infección. Evolucionó con sepsis a Staphylococcus aureus. Ecocardiograma y angiotac confirman AAT, se manejó mediante procedimiento híbrido, cirugía e instalación endovascular de 2 stents recubiertos (Atrium V12 XR Medical Corp, Hudson, NH). Los controles clínicos post procedimiento como la ecotomografía abdominal confirmaron el éxito del tratamiento. Conclusión: El procedimiento endovascular de reparación de aneurisma de la aorta en recién nacidos prematuros puede ser considerado en el momento de decir la terapéutica de esta enfermedad y podría evitar los riesgos asociados a cirugía abierta. Sin embargo, obliga a un seguimiento y control durante el crecimiento del paciente por la eventual necesidad de redilatar los stents implantados. Se desconoce cómo será la evolución de los procedimientos endovasculares neonatales en el futuro.

Aortic aneurysms (AA) in the paediatric population are uncommon. The use of umbilical catheters in neonates has been associated with infections and, on some occasions, the formation of aortic aneurysms. The surgical repair of these aneurysms is one type of treatment; however, percutaneous intervention with stents could provide an alternative treatment route, with fewer complications. The aim of this report is to present the therapeutic scope of a hybrid procedure, in which the combined surgical and percutaneous technique offers a less invasive alternative to open surgery for the repair of aortic aneurysms or their main branches. Clinical case: The case concerns a pre-term newborn of 30 weeks weighing 1,335 g. An umbilical catheter was introduced, which was withdrawn at 14 days due to an infection. It developed as Staphylococcus aureus with sepsis. The echocardiogram and Angio-CT confirmed AA, which were managed using a hybrid procedure of surgery and the endovascular implantation of 2 coated stents (Atrium V12 XR Medical Corp, Hudson, NH). The post-procedure clinical follow-ups, including abdominal echo-tomography, confirmed the success of the treatment. Conclusion: The endovascular aortic aneurysm repair procedure in premature newborns may be considered when deciding treatment of this disease, and could avoid the risks associated with open surgery. However, follow-up and monitoring is required while the patient grows up, due to the possibility that the implanted stents require re-dilating. The outcomes of neonatal endovascular procedures in the future are unknown.

Estudo prospectivo das complicações da Doença de Kawasaki: análise de 115 casos / Prospective study of Kawasaki Disease complications: review of 115 cases

OBJETIVO: Chamar a atenção para as complicações, que podem surgir em qualquer fase da Doença de Kawasaki (DK), para os fatores de risco que contribuem para o aparecimento dessas complicações e para as possíveis sequelas da doença, sejam elas transitórias ou permanentes. MÉTODOS: Estudo prospectivo (coorte clínica) realizado entre abril de 2002 e abril de 2009 de 115 pacientes com DK internados no serviço de Reumatologia Pediátrica do Hospital Geral do Distrito Federal. Todos os pacientes foram sequencialmente avaliados com exames clínicos e laboratoriais, ecocardiogramas com Doppler, imitanciometria, potenciais evocados auditivos, avaliação psicológica, exame oftalmológico e, em um paciente com coreia, angiorressonância magnética cerebral. Em todos os pacientes foram aplicados questionários avaliando a possível presença de dificuldades cognitivas, emocionais, comportamentais e sociais. RESULTADOS: Vinte e cinco pacientes (21,7 por cento) apresentaram aneurismas de coronárias. Trinta e oito pacientes (33 por cento) apresentaram perda auditiva neurossensorial durante a doença aguda e subaguda, e 13 pacientes (11,3 por cento) mantiveram a perda auditiva seis meses após a primeira avaliação. Outras complicações observadas foram: paralisia facial em um paciente (0,9 por cento), ataxia na fase aguda e subaguda em 11 pacientes (9,5 por cento), complicações oftalmológicas em 15 pacientes (13,2 por cento), constatando-se uveíte em 13, edema de papila em um paciente e hemorragia conjuntival em outro. Um paciente apresentou coreia (0,9 por cento) sendo que a angioressonância magnética evidenciou alterações compatíveis com isquemia cerebral. Em um paciente constatou-se a presença de aneurisma de aorta torácica (0,9 por cento), e outro apresentou vasculite necrosante que evoluiu com gangrena periférica e perda da ponta da língua (0,9 por cento). Alterações de comportamento durante a convalescença (20 por cento) foi observada em 23 crianças. CONCLUSÃO: A DK pode evoluir com complicações diversas, mesmo meses após a fase aguda da doença, eventualmente resultando em sequelas permanentes. Quanto mais precoce forem o diagnóstico e a intervenção terapêutica com a administração de IgG IV, menor será a ocorrência de complicações. Presença de trombocitose, anemia e de atividade inflamatória elevada e por tempo prolongado são fatores de risco para o aparecimento de complicações.

OBJECTIVE: To draw attention to complications that might arise in any Kawasaki disease (KD) stage, risk factors contributing to the onset of complications and possible transient or permanent disease sequelae. METHODS: Prospective study (clinical cohort) conducted between April 2002 and April 2009 of 115 patients with KD admitted to the Pediatric Rheumatology Clinic of the General Hospital of the Federal District, Brazil. All patients were sequentially assessed with clinical and laboratory examinations, Doppler echocardiography, imitanciometry, auditory evoked potentials, psychological evaluation, ophthalmologic examination and, in one patient with chorea, cerebral magnetic resonance angiography. In all patients, a questionnaire assessing the possible presence of cognitive, emotional, behavioral and social disorders was applied. RESULTS: Twenty-five patients (21.7 percent) had coronary aneurisms. Thirty eight patients (33 percent) had a sensorineural auditory loss during the acute and subacute phases of the disease and 13 patients (11.3 percent) maintained the auditory loss six months after the first assessment. Other complications observed were as follows: facial palsy in one patient (0.9 percent), ataxia in acute and subacute phases in 11 (9.5 percent); 15 patients had ophthalmologic complications (13.2 percent), with uveitis in 13, papilledema in one patient, and conjunctival hemorrhage in another patient. One patient experienced chorea (0.9 percent), with a magnetic resonance angiography showing changes consistent with cerebral ischemia. In one patient, a thoracic aorta aneurism was found (0.9 percent) and another patient had a necrotizing vasculitis progressing to peripheral gangrene and tongue tip loss (0.9 percent). Behavioral changes over convalescence were observed in 23 children. CONCLUSION: KD may progress with several complications even within months of the disease acute phase, eventually resulting in permanent sequelae. The earlier the diagnosis and therapeutic intervention with IV IgG administration are, the lower will be the occurrence of complications; the presence of thrombocytosis, anemia and elevated and extended inflammatory activity are risk factors for complication arising.

Tratamiento endovascular de transecciones agudas de la aorta descendente / Endovascular treatment of acute traumatic aortic rupture

Background: Traumatic rupture of the thoracic aorta as a result from high-speed deceleration injury is associated with a mortality rate of 80 percent to 90 percent at the scene of the accident. Survivors usually have life-threatening injuries to other organ systems. Standard open repair is associated with a high penoperative morbidity and mortality. Endografting offers a less invasive alternative to open surgical repair. Aim: To evaluate results of endovascular management of acute traumatic descending thoracic aortic ruptures. Methods: Between August 2002 and March 2010, patients treated for this trauma were reviewed. Results: 16 patients (fourteen males mean age 42.7 +/- 15.8 years, range 24-74) underwent endovascular treatment of an acute aortic rupture. Associated traumas in fifteen patients were: severe brain (7), spleen (4), liver (1), kidney (3) and large bone (9) injuries. Motor vehicle accidents caused 13 of the injuries and fall from height 3. Rupture was diagnosed with admission CT sean and confirmed by intraoperative angiogram. Patients were treated with thoracic aortic endograft, in 11 cases the left subclavian artery was covered with no need for further revascularization. Technical success was 100 percent, no procedure-related mortality or paraplegia was observed. One patient died 5 days after the procedure due to severe associated injuries. During a mean follow-up of 30.8 months (range 1-80), no deaths, complications or need for further interventions presented. Conclusion: Endovascular treatment of acute traumatic aortic isthmic rupture is encouraging and compares favorably to open surgical approach with low morbidity and mortality rates.

Los accidentes por desaceleración súbita se asocian a transección de la aorta torácica, falleciendo 85-90 por ciento de ellos en el sitio del suceso. Los que sobreviven presentan habitualmente politraumatismo asociado grave, teniendo la reparación quirúrgica convencional de la aorta una alta morbimortalidad. La reparación endovascular es una alternativa menos invasiva y de menor morbi-mortalidad. Objetivo: Analizar los resultados de la reparación endovascular de transecciones agudas de la aorta torácica. Pacientes y Métodos: Se revisan los antecedentes de los pacientes tratados entre agosto de 2002 y marzo de 2010. Resultados: Tratamos 16 pacientes (14 hombres, 42,7 +/- 15,8 años, extremos 24-74) con transección traumática aguda de aorta torácica descendente. Quince presentaban lesiones asociadas: traumatismo encéfalo-craneano (7), fractura de huesos largos (9), laceración esplénica (4), hepática (1), renal (3). La causa del accidente fue vehicular en 13 casos y caída de altura en 3. El diagnóstico fue realizado mediante tomograña axial computada al ingreso y luego angiograña en la sala de operaciones. El tratamiento consistió en la cobertura de la zona de transección mediante el implante de una endoprótesis, siendo necesario cubrir la arteria subclavia izquierda en 11 casos, sin requerir revascularización de la extremidad. El éxito técnico fue 100 por ciento, no hubo mortalidad relacionada al procedimiento. No hubo paraplejia. Un paciente de 60 años fallece al 5to día por lesiones asociadas graves. En el seguimiento alejado a 30,8 meses (1-80) no ha habido complicaciones ni reintervenciones. Conclusión: La cirugía endovascular es una alternativa eficaz y con baja morbimortalidad para el tratamiento de transecciones de la aorta torácica.

Giant aneurysm of thoracic and proximal abdominal aorta in a patient with common variable immunodeficiency

Common variable immunodeficiency [CVID] is the most common symptomatic primary immunodeficiency disease, predisposing the patients to various tissue involvement and organ damage. Here a 16-year-old boy is presented who was referred to our center with cough, dyspnea, cyanosis, and history of recurrent pneumonia. The diagnosis of CVID was made according to reduction all serum immunoglobulin levels, normal numbers of T, B and NK lymphocyte subpopulations, poor antibodies responses. Considering abnormality in heart examination and chest X-ray, echocardiography and computed tomography angiography were performed which showed large thoraco-abdominal; aortic aneurysm in this patient. Although there are some reports of cardiovascular disease associated with primary antibody deficiencies, this is the first time that such large thoraco-abdominal aortic aneurysm is reported in CVID. This may be secondary to recurrent pulmonary infections or an unknown mutation process. Cardiovascular abnormalities are an entity that should be kept in mind in patients with primary immunodeficiency diseases

Fatal traumatic rupture of ascending aortic aneurysm having idiopathic cystic medial necrosis: An autopsy case.

This report describes an autopsy case pertaining to death due to traumatic rupture of aortic aneurysm. A 21 year old deaf and dumb male was assaulted with kicks over the chest. Autopsy revealed external injuries over the body. Internally, a fusiform aneurysmal dilatation of the lumen of the aortic root and tubular segment of the ascending aorta were observed, with tear of the anterior wall of the ascending aorta and resultant haemopericardium. Histologically, the wall of the aneurysm revealed cystic medial necrosis, which appears to idiopathic in nature. A Common complication of aortic aneurysms is dissection, with subsequent spontaneous rupture. In this case, there was no evidence of dissection and the rupture was traumatic in nature. Death was certified as due to cardiac tamponade. The extent of trauma to which the victim was subjected to, appears to be such as would have been insufficient to cause death in an otherwise normal individual. An account of the findings, along with a discussion of the pathology of aneurysms and cystic medial necrosis as also of the mechanism of rupture of aneurysms is provided.

One stage hybrid repair of aortic arch aneurysms with antegrade endovascular graft deployment

Despite advances in surgical technique and intensive care medicine, the mortality and morbidity of standard single or two staged approaches for the management of arch aortic aneurysms remain extremely high. With the elephant trunk technique, some patients never proceed to completion of treatment either because of worsening co-morbidity or interim rupture. Retrograde thoracic endografting is not always feasible and its complications are well known. This study examines the use of a hybrid approach for treating arch aneurysms in one stage. The technique combines open aortic arch debranching with antegrade stent repair of these aneurysms. In this series three women and nine men with aneurysms involving the arch, ascending and descending thoracic aorta were treated. Their median age was 66 years. Most aneurysms were degenerative in aetiology [50%. The rest were either dissecting or secondary to Marfan's disease. One patient had the ascending aorta replaced prior to presenting with the arch problem. All other patients were put on cardiopulmonary bypass and had their ascending aorta replaced with or without further concomitant cardiac procedures. Cerebral perfusion was maintained antegrade at 24[degree sign] C through right axillary artery cannulation. This was followed by debranching of the arch of aorta and closure of the origins of the innominate, left common carotid and left sub-clavian arteries. The thoracic endografts were then introduced antegrade through a side arm connected to the body of the main aortic graft. No attempt was made to revascularise the left sublcavian artery. All patients were followed-up with CT scans. No patients was lost to follow-up. It was possible to complete the procedure in a single stage in all patients. The mean operative time was 5.7 hours. There was no intraoperative mortality. There were no intra or postoperative coagulation problems. Two patients had immediate type II endoleak that disappeared completely in one month. One patient died postoperatively having developed acute renal failure and chest infection. Acute renal failure requiring temporary dialysis occurred in three other patients. One patient developed delayed paraparesis that recovered with CSF drainage. One patient suffered with lower medial quadranopia in the right eye. There were no strokes or permanent spinal neurological deficits. Two patients with history of COPD required prolonged respiratory support. They were no long term complications secondary to occlusion of the left subclavian artery. The median length of ITU stay was 5.1 days. The mean length of hospital stay was 15.8 days. During a median follow-up duration of 8.8 months two patients were successfully treated for type II endoleak and one patient developed type 1 endoleak that required extension of the bottom end of the endografts. All aortic debranching grafts remain patent to last follow-up. One stage hybrid repair of aneurysms involving the aortic arch is a feasible technique. The preliminary results of this series show low mortality and complications rate. Larger series with longer follow-ups are underway

Tratamiento endovascular del aneurisma de aorta torácica descendente / Endovascular treatment of descending thoracic aorta aneurysm

BACKGROUND: The natural history of aneurysms ends in rupture and death. In 1990 the first endovascular exclusion of an aneurysm, using an endoluminal graft implanted through the femoral arteries was performed. More recently, the same procedure has been used for aneurysms of the thoracic aorta. AIM: To report our experience with endovascular treatment of thoracic aorta aneurysms. MATERIAL AND METHODS: Analysis of 14 patients (nine male), aged 30 to 79 years, treated between May 2001 and August 2002. RESULTS: The mean diameter of the aneurysms was 6.9 cm. The etiology was atherosclerotic in nine patients. The Excluder device (Gore) was preferentially used. There was no operative mortality or paraplegia. One patient had a transient leg monoparesis that reverted completely. No patient had type I endoleaks. Two patients had type II endoleaks on discharge, that sealed spontaneously. In a follow up, ranging from 2 to 17 months, one patient died of a bronchopneumonia and no aneurysm rupture has been detected. CONCLUSIONS: The short term results of endoluminal treatment of thoracic aorta aneurysms are excellent. This treatment is less invasive and has less complications than conventional surgery.

Pseudoaneurisma de la aorta torácica de origen traumático / Pseudoaneurysm of the thoracic aorta of traumatic origin

Se presenta el caso de un paciente con ruptura traumática de aorta descendente y con la formación tardía de un pseudoaneurisma, el cual fue reparado mediante la utilización de un parche de duramadre.

Dissecção da aorta associada a lupus eritematoso sistêmico / Aortic dissection associated with systemic lupus erythematosus

Mulher de 33 anos, com história de hipertensão arterial sistêmica (HAS) há 4 anos e diagnóstico de lupus eritematoso sistêmico (LES) há 3 anos, em uso de doses elevadas de corticóides, há 8 meses apresentando infecções respiratórias de repetição e insuficiência cardíaca congestiva. O estudo hemodinâmico confirmou o diagnóstico de insuficiência aórtica (IA o) com dilatação aneurismática do seio posterior de Valsalva, aorta ascendente de calibre normal e artérias coronárias normais. Foi levada à cirurgia onde foi encontrada dissecção da aorta com desabamento das cúspides aórticas levando à IAo, corrigido através de implante de tubo de pericárdio bovino e suspensão da valva aórtica. No pós-operatório (PO) evoluiu com convulsão focal em hemicorpo esquerdo seguido por hemiparesia à esquerda e infecção respiratória, recebendo alta no 25° dia PO com discreta hemiparesia à E e do ponto de vista cardiovascular em classe funcional I (NYHA), em uso de medicação. Enfatizamos a necessidade de se pensar no diagnóstico de dissecção de aorta em pacientes portadores de LES e IAo, principalmente aqueles que tenham história de HAS e uso de corticosteróides por tempo prolongado

A 33 year-old female patient, with a 4-year history of hypertension plus a 3-year history of systemic lupus erythematosus, who had been taking high dosages of corticosteroids, has shown repetitive respiratory infections and congestive heart failure for the past 8 months. Angiocardiography confirmed the diagnosis of aortic insufficiency with aneurismatic dilation of Valsalva's posterior sinus, ascending aorta of normal diameter and normal coronary arteries. Aortic dissection causing aortic insufficiency due to collapse of aortic leaflets was spotted during the surgery and was corrected by a bovine pericardial tube and suspension of aortic valve. The postoperative (PO) period was complicated by leftsided seizures followed by left hemiparesis and respiratory infection. She was discharged on the 25th PO day with mild left hemiparesis and in functional class I (NYHA), using medicines. We emphasize the need to consider the diagnosis of aortic dissection in patients with systemic lupus erithematosus and aortic insufficiency, specially in those who have a history of systemic arterial hypertension and long-term corticosteroid therapy