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1.
Medicine (Baltimore) ; 99(34): e21849, 2020 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-32846835

RESUMO

INTRODUCTION: Patent ductus venosus (PDV) is a rare and critical disease, and the majority of patients present with pulmonary arterial hypertension (PAH) or hepatopulmonary syndrome due to congenital portosystemic shunt. We reported that both PAH and hypersplenism were major complications of PDV in this case. This case report can assist the treatment and recovery of the patients with similar symptoms. PATIENT CONCERNS: A 4-year-old male patient presented to our institution with a history of recurrent respiratory infections accompanied by leukocytopenia, thrombocytopenia and presented with tachypnoea. upon mild exertion. DIAGNOSIS: A wide communication, 10 mm in diameter, between the portal vein and inferior vena cava was identified in the subcostal echocardiogram and computed tomography images. Echocardiography showed an estimated systolic pulmonary artery pressure of 106 mm Hg. Right-sided cardiac catheterization indicated a mean pulmonary arterial pressure of 30 mm Hg and a pulmonary vascular resistance of 3 Wood units. Chest X-ray revealed cardiomegaly with a prominent pulmonary segment. INTERVENTIONS: The patient was treated with combination pharmacotherapy of bosentan and tadalafil and PDV ligation. OUTCOMES: A year later, the boy showed normal exercise tolerance and weight gain. Liver and spleen parameters, liver function, blood cells and the general condition of the boy improved. CONCLUSION: Initial combination therapy of bosentan and tadalafil is safe and effective in children with PAH associated with PDV. When PDV banding test shows normal portal pressure, PDV ligation is considered acceptable in children with PAH and hypersplenism associated with PDV.


Assuntos
Hiperesplenismo/etiologia , Ligadura/métodos , Veia Porta/anormalidades , Hipertensão Arterial Pulmonar/etiologia , Malformações Vasculares/cirurgia , Assistência ao Convalescente , Anti-Hipertensivos/administração & dosagem , Anti-Hipertensivos/uso terapêutico , Bosentana/administração & dosagem , Bosentana/uso terapêutico , Cardiomegalia/diagnóstico por imagem , Pré-Escolar , Terapia Combinada/métodos , Ecocardiografia/métodos , Humanos , Masculino , Veia Porta/diagnóstico por imagem , Veia Porta/cirurgia , Hipertensão Arterial Pulmonar/fisiopatologia , Radiografia Torácica/métodos , Tadalafila/administração & dosagem , Tadalafila/uso terapêutico , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Malformações Vasculares/complicações , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/tratamento farmacológico , Resistência Vascular/fisiologia , Vasodilatadores/administração & dosagem , Vasodilatadores/uso terapêutico
3.
Asian Cardiovasc Thorac Ann ; 28(8): 463-469, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32659103

RESUMO

BACKGROUND: Pulmonary artery sling is commonly associated with tracheal stenosis and intracardiac anomalies. While surgical repair is standardized, coexistent anomalies often determine outcomes. With the paucity of risk stratification, this study aimed to review our experience and stratify risk factors for the surgical outcome of complex pulmonary artery sling repair in the presence of airway or intracardiac lesions. METHODS: Seventy-nine consecutive children with pulmonary artery sling were evaluated retrospectively following surgical repair. Median age at surgery was 5 months (interquartile range 3-9). Surgical approaches included pulmonary artery sling alone (n = 10), pulmonary artery sling with tracheoplasty (n = 41), and pulmonary artery sling with both intracardiac and tracheal surgery (n = 28). RESULTS: There were 7 early (8.8%) deaths. Two patients after left pulmonary artery reimplantation needed revision of the anastomosis. The median intensive care and hospital stay were 11 (interquartile range 9.2-24.8) and 17.9 (interquartile range 4.3-19.8) days, and considerably longer when associated tracheal surgery (p = 0.002). Follow-up was complete in 66/69 and 3 (3.8%) children died late: 2.7, 10.2, and 17 months after surgery. Univariate analysis showed abnormal lung and coexisting structural heart disease as risk factors. Multivariate analysis revealed total cardiopulmonary bypass time as an independent predictor of overall mortality. CONCLUSION: Complex pulmonary artery sling repair can be performed with a good surgical outcomes even when associated with airway malformations or structural heart diseases. Lung abnormality and longer cardiopulmonary bypass time as a surrogate marker of complex surgery, are possible risk factors.


Assuntos
Anormalidades Múltiplas , Cardiopatias Congênitas/cirurgia , Artéria Pulmonar/cirurgia , Reimplante , Estenose Traqueal/cirurgia , Malformações Vasculares/cirurgia , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/mortalidade , Humanos , Masculino , Complicações Pós-Operatórias/mortalidade , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Reimplante/efeitos adversos , Reimplante/mortalidade , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Estenose Traqueal/diagnóstico por imagem , Estenose Traqueal/mortalidade , Resultado do Tratamento , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/mortalidade
5.
J Card Surg ; 35(7): 1743-1745, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32485051

RESUMO

The association of absent right superior vena cava and persistent left superior vena cava draining into unroofed coronary sinus with common atrium and the atrioventricular septal defect is an extremely rare form of the congenital cardiac disorder with only one case reported so far, hence, can be missed preoperatively if not carefully looked for. Failure to detect absent right superior vena cava beforehand may otherwise pose difficulties in carrying out invasive surgical or medical interventions.


Assuntos
Anormalidades Múltiplas/cirurgia , Procedimentos Cirúrgicos Cardiovasculares/métodos , Seio Coronário/anormalidades , Seio Coronário/cirurgia , Defeitos dos Septos Cardíacos/cirurgia , Malformações Vasculares/cirurgia , Veia Cava Superior/anormalidades , Veia Cava Superior/cirurgia , Pré-Escolar , Ecocardiografia , Ecocardiografia Transesofagiana , Feminino , Átrios do Coração/anormalidades , Átrios do Coração/cirurgia , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Humanos , Resultado do Tratamento
6.
Transplant Proc ; 52(5): 1544-1546, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32505503

RESUMO

BACKGROUND: Double kidney transplantation allows the use of marginal kidneys with a significant improvement in the recovery of renal function expected after transplantation, although with a greater anesthesiologic and surgical risk. One-sided positioning, more cautious in the event of functional exhaustion, can be complex due to vascular anomalies. MATERIALS AND METHODS: We report the case of 2 double unilateral kidney transplants with vascular reconstructions. The first is a double kidney transplant from a 83-year-old donor. Both kidneys (score 5) had 2 arteries and the arterial patch was not usable. A cryopreserved arterial graft was used for the packaging of an arterial axis with which a single T-L anastomosis was performed; the 2 veins were also joined with the packaging of a single anastomosis. The second case is a double kidney transplant from a cadaveric donor performed on a recipient suffering from severe diffuse atheromasia. The right kidney had 2 arteries and the left kidney had 3 arteries (both score 5). The aortic patches and veins of the 2 kidneys were joined together and a single arterial and venous anastomosis was performed. RESULTS: The course has been uneventful. In both cases there were no perioperative vascular complications. CONCLUSIONS: The use of marginal organs is an increasingly common reality. Bench vascular reconstructions can further increase donation resources, safely enhancing the transplantation of already marginal organs that would otherwise not be usable and allowing the contralateral vascular axis to be kept intact.


Assuntos
Transplante de Rim/métodos , Rim/irrigação sanguínea , Rim Único/cirurgia , Transplantes/irrigação sanguínea , Malformações Vasculares/cirurgia , Procedimentos Cirúrgicos Vasculares/métodos , Idoso , Idoso de 80 Anos ou mais , Anastomose Cirúrgica , Feminino , Humanos , Rim/cirurgia , Masculino , Pessoa de Meia-Idade , Reoperação/métodos , Transplantes/cirurgia
7.
Medicina (Kaunas) ; 56(5)2020 May 22.
Artigo em Inglês | MEDLINE | ID: mdl-32456057

RESUMO

Vascular malformations (VMs) are a wide vascular or lymphatic group of lesions common on the head and neck. The objective of this study was to assess the efficacy and morbidity of sclerotherapy for the treatment of VMs in the oral and perioral area. Special attention was given to factors that may contribute to minimizing postoperative morbidity. Data from 25 patients (32 lesions) with oral VMs submitted to sclerotherapy with monoethanolamine oleate (EAO) were included. A structured form was used to collect data. An arbitrary score was determined to evaluate postoperative morbidity. Each of the following signs or symptoms received one point: pain, swelling, hematoma, ulceration, erythema, transient numbness, and transient itching. Pain and swelling were further divided into mild to moderate (1 point) and severe (2 points). Theoretically, the score was in the range of 0-9. Calculated scores ranged 0-4. The patients were further divided into two groups with scores of 0-1 denoting minimal morbidity (MIN) and 2-4 denoting significant morbidity (SIG). The number of lesions in each morbidity-score group were comparable (MIN 17and SIG 15). There were no statistically significant differences between the groups regarding age, number of applications, or average injection volume per mm lesion. Statistically significant differences were noted regarding gender (p = 0.05), lesion diameter (p = 0.030), total volume of first (p = 0.007) and second application (p = 0.05), and total injected volume (p = 0.03). Factors contributing to the risk for significant morbidity included being male, lesion diameter > 5 mm, volume > 0.3 mL per application, and total injected volume > 0.3 mL. A waiting time of 12 weeks prior to additional EAO application was required in 12 out of 29 lesions for clinical observation of complete regression. It was concluded that sclerotherapy with EAO as monotherapy is easy to apply, safe, and effective within a small number of sessions. Application of <0.3 mL EAO per session, and a waiting time of 12 weeks prior to the second application, would significantly minimize morbidity.


Assuntos
Boca/fisiopatologia , Complicações Pós-Operatórias/etiologia , Escleroterapia/métodos , Malformações Vasculares/cirurgia , Adolescente , Adulto , Feminino , Humanos , Israel , Masculino , Pessoa de Meia-Idade , Boca/lesões , Complicações Pós-Operatórias/epidemiologia , Melhoria de Qualidade , Estudos Retrospectivos , Escleroterapia/normas , Escleroterapia/estatística & dados numéricos , Resultado do Tratamento
8.
J Craniofac Surg ; 31(5): e445-e448, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32371683

RESUMO

Hemangiomas are benign vascular lesions characterized by endothelial vascular proliferation and may demonstrate aggressive clinical features. Intraosseous hemangiomas are uncommon and the maxillary location rare, with few cases described in the literature. The treatment of these lesions varies, however they have a better prognosis when a surgical treatment through resection is performed. The aim of this study is to report a case of right maxillary cavernous intraosseous hemangioma treated by surgical resection with previous embolization. The diagnosis was made through incisional biopsy and immunohistochemical examination. An aggressive lesion profile was observed through the Ki67 marker. During surgery, a temporary ipsilateral external carotid artery ligation was performed to minimize possible bleeding. Weber-Fergson surgical access was performed and total surgical resection through hemi-maxillectomy. The patient is stable after 30 months of follow-up without relapses. In suspected cases of intraosseous hemangiomas, accurate diagnosis and total surgical resection of the lesion is essential. The use of therapeutic mammoths that minimize sagging during and after the procedure such as embolization and arteriography should always be performed.


Assuntos
Hemangioma Cavernoso/cirurgia , Neoplasias Maxilares/cirurgia , Crânio/anormalidades , Coluna Vertebral/anormalidades , Malformações Vasculares/cirurgia , Craniotomia , Embolização Terapêutica , Hemangioma Cavernoso/diagnóstico por imagem , Humanos , Masculino , Neoplasias Maxilares/diagnóstico por imagem , Crânio/diagnóstico por imagem , Crânio/cirurgia , Coluna Vertebral/diagnóstico por imagem , Coluna Vertebral/cirurgia , Malformações Vasculares/diagnóstico por imagem , Adulto Jovem
9.
Int J Pediatr Otorhinolaryngol ; 134: 110074, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32416530

RESUMO

OBJECTIVE: Retropharyngeal lymphatic malformations (LMs) are uncommon congenital anomalies. Open surgery, sclerotherapy and various pharmaceutical agents have been described to manage these lesions. However, when such lesions involve the retropharyngeal space with evidence of airway compromise, none of the above aforementioned modalities has shown superior result without repeated imaging and procedures. We describe the use of radiofrequency ablation (Coblation) in an endoscopic-assisted, transoral approach as a one-off treatment for pediatric retropharyngeal lymphatic malformations. METHODS: Six patients with retropharyngeal lymphatic malformations, presented with airway compromise, were treated with endoscopic-assisted, transoral submucosal coblation in our unit between April 2015 to August 2018. We retrospectively reviewed their medical records, imaging and endoscopic findings. RESULTS: Five out of our six patients had LMs involving only the retropharyngeal space, who received the coblation as their sole treatment. One patient with extensive LMs involving multiple neck compartments received coblation to the retropharyngeal part as an adjunct procedure. The average operation time was 62.8 min, and the average intraoperative blood loss was 3.8 ml. All patients were safely extubated immediately after the surgery. None required admission to pediatric intensive care unit (PICU), nor prolonged dysphagia reported. They were observed for 2-5 days post operatively. The average follow up was 28 months (1-5 years) and none required re-operation to date. CONCLUSIONS: We presented our approach and outcome of 6 children with endoscopic-assisted, transoral submucosal coblation to retropharyngeal lymphatic malformation. We believe it is a safe and effective primary treatment to these patients, and a possible adjunct to complex vascular malformation involving multiple sites.


Assuntos
Ablação por Cateter/métodos , Anormalidades Linfáticas/cirurgia , Doenças Faríngeas/cirurgia , Pré-Escolar , Endoscopia , Feminino , Humanos , Anormalidades Linfáticas/diagnóstico por imagem , Masculino , Doenças Faríngeas/diagnóstico por imagem , Faringe/cirurgia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Malformações Vasculares/cirurgia
10.
Ann Vasc Surg ; 68: 201-208, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32439531

RESUMO

BACKGROUND: The need for major amputations in patients with vascular malformations is rare. This study reviews our contemporary experience with major amputations in patients with vascular malformations. METHODS: A retrospective review from April 2014 to November 2018 identified 993 patients undergoing management of a vascular malformation involving the upper or lower extremity at a tertiary center. This population was analyzed to identify those requiring either a transfemoral or transhumeral amputation. This cohort was investigated for clinical course, surgical procedures, and outcomes. RESULTS: Five patients (0.5%) underwent major amputation, including 3 transhumeral and 2 above-knee amputations. The median age was 37.8 years (interquartile range (IQR): 25.4-40.2), and 2 (40%) were male. Four (80%) patients had high-flow arteriovenous malformations, including 1 (20%) with Parkes-Weber syndrome. One (20%) patient had a low-flow venous malformation associated with Klippel-Trénaunay syndrome. All patients had malformation extending into the chest or pelvis, with the amputation being at the level of residual malformation. As such, amputation had been initially felt to be high risk because of the proximal extent of the lesions. Before amputation, a median of 11 procedures (IQR: 4-39) were performed per patient. This included 29 transarterial embolizations, 4 transvenous embolizations, 20 direct stick embolizations, 3 debulking procedures, 38 debridements, 6 skin grafts or muscle flaps, and 4 minor amputations. The median time course of treatment before amputation was 117 months (IQR: 44-171). Indications for major amputation included chronic pain and recurrent bleeding in all 5 (100%) patients, loss of function in 2 (40%), nonhealing wounds in 2 (40%), and sepsis in 1 (20%) patient. There were no perioperative deaths. The median blood loss was 1,000 mL (IQR: 650-2,750). All patients required transfusion of packed red blood cells with a mean of 1.6 units (standard deviation: 0.54). Transhumeral amputation was facilitated by transcatheter embolization in 1 (33%) and an occlusion balloon within the subclavian artery in 2 (66%) patients. The median length of stay was 6 days (IQR: 5-13). The median length of follow-up was 132 months (IQR: 68-186) from initial intervention and 12 months (IQR: 8-31) from amputation. Two patients (40%) who had undergone transhumeral amputation required revision of the amputation site for recurrent ulceration at 2 and 38 months. Of these, 1 patient underwent 3 transcatheter embolization procedures before revision and 1 underwent 1 embolization after revision. CONCLUSIONS: Although rare, successful amputation at the level of residual malformation can be performed in select patients with refractory complications of vascular malformations including intractable pain, bleeding, or nonhealing wounds. Specific preoperative and intraoperative measures may be critical to achieve satisfactory outcomes, and endovascular techniques continue to play a role after amputation.


Assuntos
Amputação , Extremidade Inferior/irrigação sanguínea , Extremidade Superior/irrigação sanguínea , Malformações Vasculares/cirurgia , Adulto , Amputação/efeitos adversos , Feminino , Humanos , Salvamento de Membro , Masculino , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Malformações Vasculares/complicações , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/fisiopatologia , Cicatrização
12.
Cardiovasc Intervent Radiol ; 43(6): 858-865, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32236672

RESUMO

PURPOSE: To retrospectively assess the technical feasibility, safety and clinical efficacy of percutaneous MR-guided cryoablation of low-flow vascular malformations (LFVM). MATERIALS AND METHODS: Between July 2013 and May 2019, 9 consecutive patients (5 male; 4 female; mean age 39.4 ± 15.3 years, range 15-68) underwent MR-guided cryoablation of LFVM. Patients were treated due to pain in all cases. Procedural data, complications and clinical results were analyzed. RESULTS: Technical success defined as complete coverage of the LFVM by the iceball without involvement of nearby non-target thermal-sensitive structures was achieved in 9/9 (100%) cases. Mean procedure time was 122 ± 20 min (range 90-150); 2-6 cryoprobes (mean 3.7 ± 1.2) and 2-4 freezing cycles (mean freezing time 19.8 ± 11.8 min; range 4-40) were applied. No complications were noted. Mean time from the first treatment to the last follow-up was 548 days (range 30-1776). Persistent/recurring pain was noted in 3/9 cases (33%) 30, 133 and 639 days after cryoablation, respectively, and was related in all cases to MR-confirmed local residual/recurring disease. A second cryoablation treatment was performed in these 3 cases with complete pain control at the last available follow-up (153, 25, 91 days, respectively). In the whole population, at mean 161 days (range 25-413) after the last treatment, on the numerical pain rate scale, pain significantly dropped from mean 6.4 ± 2.1 (range 3-9/10) before CA to mean 0.3 ± 0.9 (range 0-3/10) after (p = 0.009). CONCLUSIONS: Percutaneous MR-guided cryoablation is technically feasible, safe and effective for the treatment of symptomatic LFVM. LEVEL OF EVIDENCE: Level 3b, retrospective cohort study.


Assuntos
Criocirurgia/métodos , Imagem por Ressonância Magnética Intervencionista/métodos , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/cirurgia , Adolescente , Adulto , Idoso , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
13.
Ann Vasc Surg ; 68: 569.e9-569.e11, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32278874

RESUMO

BACKGROUND: Vascular malformations of the hand are rare vascular malformations that are challenging to treat. METHODS: We present a case of a large vascular malformation with left hand pain and decreased sensation of the small and ring fingers. The lesion was treated operatively with surgical excision. RESULTS: The malformation was successfully removed surgically, and pain resolved and numbness recovered by 2 weeks after surgery. CONCLUSIONS: This is a rare case of large vascular malformation in the hand with compromised neurologic status. Surgical treatment provided complete relief of the disease, and the patient returned to normal daily activities.


Assuntos
Mãos/irrigação sanguínea , Mãos/inervação , Síndromes de Compressão Nervosa/etiologia , Nervo Ulnar/fisiopatologia , Malformações Vasculares/complicações , Feminino , Humanos , Pessoa de Meia-Idade , Síndromes de Compressão Nervosa/diagnóstico , Síndromes de Compressão Nervosa/fisiopatologia , Recuperação de Função Fisiológica , Resultado do Tratamento , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/cirurgia
14.
Vet Surg ; 49(5): 958-970, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32246785

RESUMO

OBJECTIVE: To identify prognostic factors for short-term survival of dogs that experience seizures within 7 days after surgical correction of single congenital extrahepatic portosystemic shunts (cEHPSS). STUDY DESIGN: Multi-institutional retrospective study. SAMPLE POPULATION: Ninety-three client-owned dogs. METHODS: Medical records at 14 veterinary institutions were reviewed to identify dogs that underwent surgical attenuation of a single cEHPSS from January 1, 2005 through February 28, 2018 and experienced postattenuation seizures (PAS) within 7 days postoperatively. Logistic regression analysis was performed to identify factors associated with 1-month survival. Factors investigated included participating institution, signalment, shunt morphology, concurrent/historical conditions, presence of preoperative neurologic signs, presence of preoperative seizures, aspects of preoperative medical management, surgical details including method and degree of shunt attenuation, type of PAS (focal only or generalized ± focal), drugs administered as part of the treatment of PAS, and development of complications during treatment of PAS. RESULTS: Thirty (32.3%) dogs survived to 30 days. Seventy-six (81.7%) dogs experienced generalized PAS. Factors positively associated with short-term survival included having a history of preoperative seizures (P = .004) and development of focal PAS only (P = .0003). Most nonsurvivors were humanely euthanized because of uncontrolled or recurrent seizures. CONCLUSION: Dogs that experienced PAS that had a history of preoperative seizures and those that experienced focal PAS only had significantly improved short-term survival. CLINICAL SIGNIFICANCE: The results of this study provide information that will help in the counseling of owners who seek treatment for PAS after surgical correction of cEHPSS. © 2020 The American College of Veterinary Surgeons.


Assuntos
Doenças do Cão/cirurgia , Sistema Porta/anormalidades , Derivação Portossistêmica Cirúrgica/veterinária , Complicações Pós-Operatórias/veterinária , Convulsões/veterinária , Animais , Cães , Feminino , Humanos , Masculino , Sistema Porta/cirurgia , Período Pós-Operatório , Estudos Retrospectivos , Fatores de Risco , Convulsões/etiologia , Resultado do Tratamento , Malformações Vasculares/cirurgia , Malformações Vasculares/veterinária
15.
World J Pediatr Congenit Heart Surg ; 11(3): 372-373, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32294008

RESUMO

An eight-year-old boy with tricuspid atresia was found to have atretic coronary sinus ostium during cardiac catheterization. Single-stage extracardiac fenestrated Fontan operation was performed with surgical unroofing of the coronary sinus into the left atrium to avoid the risk of cardiac congestion.


Assuntos
Seio Coronário/anormalidades , Técnica de Fontan , Átrios do Coração/cirurgia , Atresia Tricúspide/complicações , Malformações Vasculares/complicações , Cateterismo Cardíaco , Criança , Humanos , Masculino , Atresia Tricúspide/cirurgia , Malformações Vasculares/cirurgia , Veia Cava Superior/cirurgia
16.
Cardiol Young ; 30(4): 585-587, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32172701

RESUMO

BACKGROUND: Patients with univentricular heart disease may undergo a superior cavopulmonary anastomosis, an operative intervention that raises cerebral venous pressure and impedance to cerebral venous return. The ability of infantile cerebral autoregulation to compensate for this is not well understood. MATERIALS AND METHODS: We identified all patients undergoing a superior cavopulmonary anastomosis (cases) and compared metrics of cerebral oxygenation upon admission to the ICU with patients following repair of tetralogy of Fallot or arterial switch operation (controls). The primary endpoint was cerebral venous oxyhaemoglobin saturation measured from an internal jugular venous catheter. Other predictor variables included case-control assignment, age, weight, sex, ischemic times, arterial oxyhaemoglobin saturation, mean arterial blood pressure, and superior caval pressure. RESULTS: A total of 151 cases and 350 controls were identified. The first post-operative cerebral venous oxyhaemoglobin saturation was significantly lower following superior cavopulmonary anastomosis than in controls (44 ± 12 versus 59 ± 15%, p < 0.001), as was arterial oxyhaemoglobin saturation (81 ± 9 versus 98 ± 5%, p < 0.001). Cerebral venous oxyhaemoglobin saturation correlated poorly with superior caval pressure in both groups. When estimated by linear mixed effects model, arterial oxyhaemoglobin saturation was the primary determinant of central venous oxyhaemoglobin saturation in both groups (ß = 0.79, p = 3 × 10-14); for every 1% point increase in arterial oxyhaemoglobin saturation, there was a 0.79% point increase in venous oxyhaemoglobin saturation. In this model, no other predictors were significant, including superior caval pressure and case-control assignment. CONCLUSION: Cerebral autoregulation appears to remain intact despite acute imposition of cerebral venous hypertension following superior cavopulmonary anastomosis. Following superior cavopulmonary anastomosis, cerebral venous oxyhaemoglobin saturation is primarily determined by arterial oxyhaemoglobin saturation.


Assuntos
Pressão Sanguínea/fisiologia , Circulação Cerebrovascular/fisiologia , Derivação Cardíaca Direita/métodos , Oxigênio/sangue , Veias Pulmonares/cirurgia , Malformações Vasculares/cirurgia , Veia Cava Superior/cirurgia , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Consumo de Oxigênio , Veias Pulmonares/diagnóstico por imagem , Estudos Retrospectivos , Malformações Vasculares/sangue , Malformações Vasculares/fisiopatologia , Veia Cava Superior/diagnóstico por imagem
17.
J Pediatr Surg ; 55(9): 1897-1902, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32067808

RESUMO

OBJECTIVE: Distinguishing retrohepatic end-to-side portocaval shunts (ES-PCS) and side-to-side portocaval shunts (SS-PCS) can be difficult, but it is essential for determining the treatment strategy. Our experience with retrohepatic PCS is analyzed. METHODS: Since 2007, 9 children (5/9 ES-PCS and 4/9 SS-PCS) were surgically treated. Radiology studies included Doppler-ultrasound, CT/MRI and angiography/occlusion test (8/9). RESULTS: CT in 5/5 ES-PCS revealed the portal vein (PV) entering the left side of the vena cava with a uniform shape. 4/4 SS-PCS showed aneurysmal PV containing the origin of the main intrahepatic portal branches (IHPB) entering the cava anterior aspect or slightly to the right with a variable length (from long to short/wide). ES-PCS anatomy showed caudate lobe absence with the fistula entering the left cava aspect free of parenchyma, but anterior through the caudate lobe in SS-PCS. With the angiography/occlusion test, the IHPB was undeveloped in ES-PCS (portal pressure > 38 mmHg) and hypoplasic in SS-PCS (portal pressure < 25 mmHg). ES-PCS treatment included: 1/5 hepatectomy and 4/5 definitive banding (one by laparoscopy); while in SS-PCS: 1/4 liver transplantation, 2/4 1-step closure (one by laparoscopy), and 1/4 definitive banding. CONCLUSION: Fistula shape, cava relationship, IHPB and portal pressures differ between the two shunt types. A question arises regarding the need for secondary complete closure after banding. LEVEL OF EVIDENCE: Level IV.


Assuntos
Veia Porta , Malformações Vasculares , Veia Cava Inferior , Adolescente , Criança , Pré-Escolar , Feminino , Fístula/diagnóstico por imagem , Fístula/cirurgia , Humanos , Masculino , Veia Porta/anormalidades , Veia Porta/diagnóstico por imagem , Veia Porta/cirurgia , Estudos Retrospectivos , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/cirurgia , Veia Cava Inferior/anormalidades , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/cirurgia
18.
World J Surg Oncol ; 18(1): 32, 2020 Feb 07.
Artigo em Inglês | MEDLINE | ID: mdl-32028965

RESUMO

BACKGROUND: Unilateral absence of pulmonary artery (UAPA) is a rare congenital disease of pulmonary circulation, which is often accompanied by other cardiovascular anomalies. Infrequently, it may remain undiagnosed until adulthood. More rarely, it is to be found with lung cancer in the ipsilateral or contralateral lung simultaneously. CASE PRESENTATION: A 56-year-old man with complaints of intermittent left chest pain for 2 months was referred to our hospital. Chest computed tomography(CT) revealed a cavitary lesion measuring 5.5 cm × 5.7 cm in the superior segment of the left lower lobe. Absence of left pulmonary artery and right-sided aortic arch were found on chest computed tomography angiography (CTA). The tumor was successfully removed via left pneumonectomy, and postoperative histopathology showed that the tumor was a squamous cell carcinoma (T2bN1). At a postoperative 24-month follow-up, the patient was free of disease and no evidence of recurrence or metastasis. Based on literature review, this is the ninth case of lung cancer in UAPA patients. CONCLUSIONS: Lung cancer and UAPA occurred ipsilaterally in 66.7% of these cases (6/9), including the present case. For those patients who occurred contralaterally, surgical treatment may be more challenging. CT and CTA could provide an accurate diagnosis for this disease entity. Identification and recognition of this rare and special disease entity may facilitate timely diagnosis and appropriate treatment.


Assuntos
Anormalidades Múltiplas/patologia , Pneumopatias/congênito , Pneumopatias/patologia , Neoplasias Pulmonares/patologia , Pulmão/anormalidades , Artéria Pulmonar/patologia , Malformações Vasculares/patologia , Anormalidades Múltiplas/cirurgia , Humanos , Pulmão/patologia , Pulmão/cirurgia , Pneumopatias/complicações , Pneumopatias/cirurgia , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/cirurgia , Masculino , Pessoa de Meia-Idade , Pneumonectomia , Prognóstico , Artéria Pulmonar/cirurgia , Malformações Vasculares/complicações , Malformações Vasculares/cirurgia
19.
J Vasc Interv Radiol ; 31(5): 788-794, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32107126

RESUMO

PURPOSE: To assess the utility of preoperative venography in evaluating and managing patients with congenital portosystemic shunts (CPSSs). MATERIALS AND METHODS: A retrospective study was performed of 42 patients (62% female; median age, 4.1 years) diagnosed with a CPSS from 2005 to 2018. Preoperative venography (n = 39) and balloon occlusive pressure measurements (n = 33) within the mesenteric venous system guided treatment. Primary outcome was serum ammonia levels at 1 month after shunt closure. Management strategies included single (n = 12) or staged (n = 18) operative ligation, endovascular occlusion (n = 8), combined surgical and endovascular closure (n = 2), and observation (n = 2). RESULTS: At 1 month, serum ammonia levels decreased from 82.5 ± 10.3 µmol/L to 38.4 ± 4.6 µmol/L (P < .001). No difference was observed in the decrease between patients treated surgically or endovascularly (P = .91). Mean occluded to non-occluded pressure gradients were significantly lower for endovascular closure (5.3 ± 1.8 mmHg) than for surgical closure (12.3 ± 3.3 mmHg, P = .02). Shunts were classified as extrahepatic in 29 patients and as intrahepatic in 13 patients; all shunts demonstrated filling of the portal system with occlusive venography. Broad and short shunts were closed surgically; narrow and long shunts were closed endovascularly. Shunts were closed in a single session (n = 20) if the pressure gradient was less than 10 mmHg and the occluded mesenteric pressure was less than 25 mmHg. CONCLUSIONS: Preoperative venography delineates shunt morphology, and balloon occlusion simulates closure hemodynamics. This information is necessary to determine whether definitive closure should be performed through endovascular or surgical methods and whether closure should be performed in a single or staged setting.


Assuntos
Flebografia , Veia Porta/diagnóstico por imagem , Veia Porta/cirurgia , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/cirurgia , Procedimentos Cirúrgicos Vasculares , Adolescente , Criança , Pré-Escolar , Bases de Dados Factuais , Procedimentos Endovasculares , Feminino , Humanos , Lactente , Recém-Nascido , Ligadura , Circulação Hepática , Masculino , Pressão na Veia Porta , Veia Porta/fisiopatologia , Valor Preditivo dos Testes , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Malformações Vasculares/fisiopatologia , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Adulto Jovem
20.
Ann Vasc Surg ; 66: 671.e15-671.e18, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32018020

RESUMO

Fibro-adipose vascular anomaly (FAVA) is a painful complex vascular malformation, characterized by muscle fibrofatty infiltration, usually in lower limb, associated with contracture of the ipsilateral extremity. This article describes the first case of FAVA reported in Portugal successfully treated with surgery. A 9-year-old female was admitted complaining of a painful mass in the right leg. The MRI scan showed the presence of a 5 × 4 × 4 cm mass in the right leg consistent with FAVA located in the peroneus longus muscle. The patient underwent resection of the involved muscle. She had 6 months of follow-up without any relevant clinical event. The authors also highlight the difference between FAVA and venous malformation. Early surgery is a treatment option that can prevent long-term consequences, as contracture and movement limitation.


Assuntos
Tecido Adiposo/patologia , Músculo Esquelético/irrigação sanguínea , Malformações Vasculares/patologia , Criança , Feminino , Fibrose , Humanos , Extremidade Inferior , Músculo Esquelético/patologia , Músculo Esquelético/cirurgia , Resultado do Tratamento , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/cirurgia
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