Anesthetic management in a child with moya-moya disease and sickle cell anemia: case report / Tratamiento anestésico en niño con enfermedad de moya-moya y anemia falciforme: caso clínico

Objective. The objective of this case report is to describe the anesthetic management in a child with moya-moya disease and sickle cell anemia provided in a tertiary hospital. Case. A 6 year-old patient, diagnosed with moya-moya disease and sickle cell anemia, both conditions associated with a greater incidence of intracranial ischemic events, with a history of two strokes of the ischemic subtype, was submitted to general anesthesia for the execution of multiple cranial burr holes in order to produce the neovascularization in poorly perfused regions. There were no complications in the perioperative period and the child was discharged from the hospital on the second postoperative day. Conclusion. Although scarcely described in the medical literature, the anesthetic management in a patient with moya-moya disease must ensure the maintenance of cerebral blood flow, normocapnia and the appropriate mean arterial pressure. In a patient with sickle cell disease, an adequate tissue perfusion, adequate oxygenation and hydration and strict pain control are to be primarily expected. The anesthesiologist is expected to know the physiopathology of both conditions to provide the best outcome for these patients (AU)

Objetivo. El objetivo de este caso clínico es el de describir el tratamiento anestésico en un niño con la enfermedad de moya-moya y anemia falciforme en un hospital terciario. Caso. Se trataba de un paciente de 6 años de edad, diagnosticado de enfermedad de moya-moya y anemia falciforme. Ambas condiciones se asocian a una elevada incidencia de episodios de isquemia cerebral. El paciente presentaba como antecedentes médicos 2 accidentes cerebrovasculares isquémicos. Se procedió a la administración de la anestesia para la realización de múltiples orificios craneales, y así favorecer la neovascularización en regiones cerebrales previamente hipoperfundidas. Conclusión. Aunque escasamente descrito, el procedimiento anestésico aplicado en el paciente con enfermedad de moya-moya debe asegurar la estabilidad del flujo de sangre en el cerebro, normocapnia y el apropiado control de la presión arterial. En un paciente con anemia falciforme, la adecuada perfusión tisular, oxigenación e hidratación y el estricto control del dolor son los primeros objetivos a conseguir. El anestesiólogo debe conocer la fisiopatología de ambas condiciones para proveer el mejor cuidado de este tipo de pacientes (AU)

Anesthetic management in a child with moya-moya disease and sickle cell anemia: Case report.

OBJECTIVE: The objective of this case report is to describe the anesthetic management in a child with moya-moya disease and sickle cell anemia provided in a tertiary hospital. CASE: A 6 year-old patient, diagnosed with moya-moya disease and sickle cell anemia, both conditions associated with a greater incidence of intracranial ischemic events, with a history of two strokes of the ischemic subtype, was submitted to general anesthesia for the execution of multiple cranial burr holes in order to produce the neovascularization in poorly perfused regions. There were no complications in the perioperative period and the child was discharged from the hospital on the second postoperative day. CONCLUSION: Although scarcely described in the medical literature, the anesthetic management in a patient with moya-moya disease must ensure the maintenance of cerebral blood flow, normocapnia and the appropriate mean arterial pressure. In a patient with sickle cell disease, an adequate tissue perfusion, adequate oxygenation and hydration and strict pain control are to be primarily expected. The anesthesiologist is expected to know the physiopathology of both conditions to provide the best outcome for these patients.

Extraintestinal infection by group C Salmonella: a case report and review of the literature.

Infected or mycotic aneurysms of the aorta are not very frequent but they are associated with high morbidity and mortality rates. Vascular infections due to Salmonella are not very frequent, but in recent years the reports of infections of this type have been on the increase. The authors report their experience with a case of aneurysm of the abdominal aorta infected by group C Salmonella and go on to review the literature on the subject.

Contained rupture of an abdominal aortic aneurysm presenting as obstructive jaundice: report of a case.

Chronic rupture of an abdominal aortic aneurysm (AAA) is a rare occurrence, the presentation of which is unusual, often mimicking other conditions such as femoral neuropathy or radicular compression syndrome. We report herein the case of an 87-year-old woman found to have a contained rupture of an AAA after presenting with back pain and obstructive jaundice. A computed tomography (CT) scan revealed the contained rupture of the aneurysm with a large hematoma compressing the common bile duct. Surgical repair of the aortic aneurysm was successfully performed and the patient remains well.

[Chronic rupture of abdominal aortic aneurysms. (Report of 3 cases)]. / Rottura cronica degli aneurismi dell'aorta addominale. (Su tre casi osservati).

Rupture of an abdominal aortic aneurysm often presents with an abdominal pain, hypotension and a pulsatile abdominal mass. In the last years same clinical reports describe patients with less apparent clinical signs who were found later in their evaluation to have a contained rupture of an abdominal aortic aneurysm. The diagnosis may be delayed by consideration of other disease causing similar symptoms (herniated disc, renal colic). In these patients with confusing abdominal symptoms CT scan provides a rapid and noninvasive diagnosis. We report three cases of contained rupture of an abdominal aortic aneurysm evaluated by computed tomography with different clinical presentation: back pain for erosion into the lumbar vertebral bodies, lower extremity neuropathy and obstructive jaundice. All patients were operated on within 24 hours on admission; there was no operative mortality and survival was 100% at one year.

Occlusion of the common iliac artery secondary of fungal endocarditis: report of a case.

Fungal endocarditis becomes complicated by peripheral arterial embolization of the lower limbs in 33%-75% of cases. Although the prognosis of patients with fungal endocarditis has improved somewhat over recent years, it remains poor, especially when the disease is associated with peripheral arterial embolization. We report herein our experience of treating a patient in whom occlusion of the right common iliac artery developed secondary to endocarditis caused by Candida parapsilosis, and review the literature on this subject.

[The surgery of abdominal aortic aneurysms synchronous with neoplastic lesions (apropos of 18 personal observations)]. / La chirurgia degli aneurismi dell'aorta addominale sincroni a lesioni neoplastiche (a proposito di 18 osservazioni personali).

The management of simultaneously occurring abdominal aortic aneurysm and malignancy is controversial. It is unclear whether to treat the aneurysm first or the malignancy, or both simultaneously. If the malignancy is resected first there is a risk of postoperative rupture of the aneurysm. If simultaneous surgery is performed there is a risk of prosthetic graft infection. This condition leads to many therapeutic problems which, by the light of 18 personal cases occurred in almost ten years and the recent literature, are discussed in this paper.

[Echo-guided compression in the treatment of femoral pseudoaneurysms secondary to cardiological interventional procedures]. / La compressione eco-guidata nel trattamento degli pseudoaneurismi femorali secondari a procedure interventistiche cardiologiche.

PURPOSE: To analyze perspectively a set of 13 patients affected by femoral artery pseudoaneurysm treated with Us-guided compression. MATERIAL AND METHODS: From January to December 1997 we observed 13 patients (7 females and 6 males; mean age: 67.4 years old) affected by femoral artery pseudoaneurysm which were treated with Us-guided compression. The compression was repeated for 15 minutes up to complete closure of the pseudoaneurysm. Every patient was followed up 24 hours after the procedure, before the discharge and every 3 months. RESULTS: In 6 cases the cardiologic procedure had diagnostic purposes while in 7 cases had interventional purposes. The pseudoaneurysm was located in the common femoral artery in 10 cases, in the superficial femoral artery in 2 cases and in the profunda femoris in the last case. The fistula between the aneurysmatic chamber and the arterial lumen had a mean length of 1 centimeter (range: 0.5-2.5 cm) and the mean diameter of the pseudoaneurysm was 3 centimeters (range: 1.5-6 cm). The mean length of the compression was 34 minutes (range: 10-120 min). The technical success rate was 92.3% with one case of failure in the unique localization in the profunda femoris. The procedure was well tolerated from the patients, with minimal discomfort. After the treatment the mean hospital stay was 2 days and to date we have had no recurrences after a mean follow-up of 18 months. CONCLUSIONS: In accordance with the data of the literature, Us-guided compression is the treatment of choice of femoral pseudoaneurysms after cardiac catheterization. The procedure is well tolerated in almost all the patients and it deters the need of surgery with reduction of complications, costs and hospital stay.

Usefulness of lipids, lipoprotein(a) and fibrinogen measurements in identifying subjects at risk of occlusive complications following vascular and endovascular surgery.

The study was designed to establish the usefulness of measuring lipoprotein(a) [Lp(a)], total cholesterol, triglycerides, low-density lipoprotein [LDL]-cholesterol, high-density lipoprotein [HDL]-cholesterol, total-to-HDL-cholesterol ratio and fibrinogen in identifying subjects at risk of occlusive complications following vascular and endovascular surgery, including primary successful ileofemoral percutaneous transluminal angioplasty, infrainguinal and aortic bypass graft and carotid endarterectomy. A total of 68 volunteers subjected to vascular and endovascular surgery were recruited to the study. Six months after successful interventions, no occlusive complications verified by angiography were observed in 45 patients (66%; No-restenosis group), whereas significant restenosis or reocclusion occurred in 23 patients (34%; Restenosis group). Significant lower concentrations of Lp(a) (p=0.032), total cholesterol (p<0.0001), LDL-cholesterol (p=0.001) and total-to-HDL-cholesterol ratio (p<0.0001) and higher concentrations of HDL-cholesterol (p=0.048) were observed in the No-restenosis group compared to the Restenosis group. The concentrations of triglycerides (p=0.080) and fibrinogen (p=0.510) did not differ significantly between groups. In multivariate discriminant analysis, the best predictors of restenosis or reocclusion were in decreasing order: LDL-cholesterol, Lp(a), total-to-HDL-cholesterol ratio, HDL-cholesterol and total cholesterol. A statistical difference of particular interest was observed in the overall distribution of Lp(a) concentrations between groups (p<0.0001), occlusive complications being unlikely to occur in patients with Lp(a) concentrations below 50 mg L(-1). The potential interference from a concurrent acute phase response, the most common source of elevation of Lp(a) in humans, was less likely in view of the absence of differences in erythrocyte sedimentation rate between the No-restenosis and Restenosis groups (p=0.463). In conclusion, the results of the present investigation point to a definite role of the combined measurements LDL-cholesterol, Lp(a), total-to-HDL-cholesterol ratio, HDL-cholesterol and total cholesterol in the identification of subjects at risk of occlusive events following vascular and endovascular surgical procedures.

[True aneurysm of the profunda femoris artery. Case report and literature review]. / Aneurisma vero dell'arteria femorale profonda. Caso clinico e revisione della letteratura.

Aneurysms of the profunda femoris artery rarely occur as an isolated lesion because of anatomical position and characteristics of wall tissue. The case of a 79-year-old man admitted to the hospital because of acute pain and swelling of the right leg with pulsating tumor in the groin is reported. Duplex scanning and angiography, performed in order to establish the diagnosis and to plan the operation, demonstrated a right large aneurysm of the deep femoral artery (8 x 12 cm). At operation, the aneurysm was resected associated to a PTFE Stretch graft replacement because the autogenous saphenous vein was not available. A review of the literature on the clinical manifestation and management in emergency or elective surgery of the isolated true atherosclerotic aneurysm of the deep femoral artery, is presented.