Primary left atrial haemangioendothelioma

We describe the case of a 21-year-old female patient with epithelioid haemangioendothelioma of the roof of the left atrium. The patient underwent radical resection of the tumour with large disease-free margins, which required reconstruction of left and right atrial superior walls. The prognosis is unpredictable, but life expectancies ranging from 1 to 20 years have been described. There is no single treatment that can be prescribed but, because of their biologic behaviour, epithelioid haemangioendotheliomas must be regarded as fully malignant neoplasms and ought to be resected radically to prevent metastatic disease, and to improve life expectancy and quality of life

McCune Albright Syndrome: A Diagnosis to be Kept in Mind

Precocious puberty, defined as the development of secondary sexual characteristics before the age of 8, often leads to anxiety in patients and their families but also in clinicians searching for the final diagnosis. After adequate investigation, the majority of the cases in girls turn out to be idiopathic. The authors present a case of McCune Albright syndrome in order to call attention to a rare cause of sexual precocity and the value of ultrasound in the evaluation of these situations. 10 years old infant girl admitted in our department due to irregular menstrual bleeding. She experienced a vaginal bleeding by the age of 3 which led to the diagnosis of McCune Albright Syndrome after a complete evaluation. Pubertal assessment revealed a reversed sequence in the remaining events with adrenarche at 5 and thelarche at 8. Hormonal evaluation demonstrated low FSH and LH levels (11,2 and 6,72 respectively) with high estrogen (204). Pelvic ultrasound showed a normal sized uterus (73x 29x32 mm), endometrial thickness of 5 mm and ovaries with several microfollicles and a copus luteum measuring 23 mm in the right ovary. McCune Albright syndrome is a very uncommon cause of sexual precocity that should, however, be suspected in all infant girls who present with vaginal bleeding. It is characterized by a triad: polyostotic fibrous dysplasia, gonadotropin-independent precocious puberty and café-au-lait skin spots. Due to autonomous production of estrogen by the ovaries, ultrasound image of the female reproductive tract is inconsistent with chronologic age. Pelvic ultrasound demonstrates a normal sized uterus with a well defined cervix and clearly identified ovaries with several follicles, similar to adult women of reproductive age. Ultrasonography of the pelvis has also an important role excluding other causes of GnRH-independent precocious puberty conditions like ovarian cysts or tumors

Surgery for bronchiectasis

OBJECTIVE: The incidence of bronchiectasis has declined markedly in developed countries. However, a reasonable number of patients still need surgery, despite aggressive physiotherapy and antibiotic therapy. We have reviewed our patients to clarify the benefits from surgery and to analyse the complications. MATERIAL AND METHODS: Between 1988 and 1999, we have operated on 119 patients with bronchiectasis, 71 female and 48 male, with a mean age of 42.2 years (range 11--77 years). Surgery was indicated because of unsuccessful medical therapy in 66 patients (55%), 31 (26%) had haemoptysis, 11 (9.2%) had lung abscess, 10 (8.4%) had lung masses, and three (2.5%) had pneumothorax. The most common manifestations were cough with sputum in 90 patients (76%), haemoptysis in 45 (38%) and recurrent infections in 57 (48%). The mean duration of the symptoms was 4 years (range 1--40 years). The lower lobes were diseased in 61 patients and bilateral disease was found in ten. The mean number of involved pulmonary segments was five (range 1-15). A lobectomy was performed in 75 patients (62%), a segmentectomy in 12 (10%), a pneumonectomy in nine (7.4%) and a bilobectomy in four (3.3%). Complete resection of the disease was achieved in 108 cases (91%). RESULTS: There was no operative mortality and perioperative morbidity occurred in 15 patients (15%), including temporary broncho-pleural fistulae in 7 (5.8%), and post-operative haemorrhage and atrial arrhythmias in four (3.3%) each. After a mean follow-up was 4.5 years, 73 patients (68%) of this group were asymptomatic, and 31 (29%) had meaningful clinical improvement, while only four (3.7%) maintained or worsened prior symptoms. The best clinical improvement occurred in patients with complete resection of the disease (P=0.008). There were no differences in the respiratory function, comparing pre- and post-operative data, with a 2-year of minimum interval. The VC was 91 and 89% and the FEV1 was 83% and 81% of expected, respectively before and after surgery, (P=NS). CONCLUSION: Surgery of pulmonary bronchiectasis has few complications and markedly improves symptoms in the great majority of patients, especially when complete resection of the disease is achieved. Pulmonary resection of bronchiectasis does not alter respiratory function

Surgery of chest wall deformities

OBJECTIVE: To evaluate the medium-term results of 77 surgical corrections in patients with chest wall deformities, 53 (68.8%) with pectus excavatum and 24 with pectus carinatum, operated upon from 1985 to 1994. METHODS: The mean age of the patients was 14.7 years (4-39 years) and 77% were younger than 15 years of age. There were 59 male (76.7%) and 18 female patients. Only four had a family history of the malformation. Seven patients (9.1%) presented with asthma-like symptoms, and 13 (16.9%) referred dyspnea and tiredness for small efforts. The remainder (74.2%) were asymptomatic, but most were psychologically disturbed by the deformity and postural abnormality. Two patients had other skeletal abnormalities. The modified surgical technique used in all cases consisted of subperichondrial resection of the abnormal costal cartilages, transverse and longitudinal osteotomies of the sternum and internal stabilization with a steel rod which was generally removed between 6 and 12 months postoperatively. RESULTS: There was neither early nor late mortality. One patient had a pneumothorax which required chest tube drainage. The mean admission time was 10.5 days (8-14 days). Follow-up was complete, and 90% of the patients had increased effort tolerance. Five of the seven patients (72%) with 'asthmatic' symptoms showed a decrease in the frequency of the crises. Two patients had recurrence of the depression by 3 and 8 months, respectively. The remaining 75 patients (97.3%) were satisfied with the cosmetic result of the surgery. CONCLUSIONS: Surgical treatment of chest wall deformities using this technique leads to good cosmetic, orthopedic and psychological results. We believe that the operations should be performed at any age in patients who have at least a moderate deformity

Surgery for pulmonary aspergiloma: curative?

Introduction.In lhe last few decades, there has been an increase in fungal diseases, especially in those caused by Aspergillus.The aim of this retrospective study was to confirm or ascertain whether surgical intervention to pulmonary aspergiloma can result in a cure or long term palliative treatment with improvement of quality of life. Methods and materiais. From 1989 to 2001, 23 patients with mean age of 44.1 years (18-69 years) were submitted to pulmonary surgery for excision of aspergilloma. Sixteen patients were mate (70%). The most frequent indication for surgery was haemoptysis in 16 patients (70%) followed by abundant sputum in 3 patients (3%). Four patients (17%) were asymptomatic. Old tuberculosis lesions (87%) or pulmonary abscesses (13%) were lhe basic conditions for lhe aspergilloma. Pre-operative evaluation of respiratory function showed a mean vital capacity of 69.8% (61-84% limits) and lhe mean Fevl was 66% (53-82% limits). Results. This group of patients were submitted to 18 lobectomies (82%), 2 bilobectomies (7%), 2 wedge resections and 1 pneumonectomy (4%). There was no operative mortality and lhe morbidity in lhe post-operative period was: persistent air leak in 7 patients (30%), post-operative bleeding in 2 patients (7%) and residual cavities in 2 patients (7%). The mean time of follow-up was 7.2 years (limits 1.5-14 years) and 3 deaths were registered. Two deaths were related to intestinal neoplasia and 1 related to lhe original disease, 5 years after surgery. All surviving patients referred good improvement of symptoms and quality of life. Conclusions. The resection of pulmonary aspergilloma could be performed with a low morbidity and mortality. The patients referred good improvement of symptoms and quality of life after surgery. Consequently, we suggest that surgical therapy is an option for both symptomatic and asymptomatic patient

Two Gestational Sacs, Two Locations - Heterotopic Pregnancy - Case Report

Introduction: Heterotopic pregnancy (HP) is defined as two gestational sacs simultaneously present in two different locations, being the uterus and the fallopian tubes the more common. Sporadic HP is a very rare condition (1:30,000 pregnancies). With the use of medically assisted reproduction the prevalence is significantly higher(1:7,000). Considering spontaneous pregnancy, HP is associated with risk factors, being prior inflammatory pelvic disease the most common. The clinical presentation is similar to that of ectopic pregnancy or spontaneous miscarriage although it is usually a more late diagnosis. Case report: 25 year-old pregnant woman, OI 0000, previously healthy; admitted at the Emergency Department (ED) with acute pelvic pain mainly at the right iliac fossa and moderate vaginal bleeding confirmed by speculum examination. She was hemodynamically stable and the bimanual palpation was painful; no prior medically assisted reproduction technique had been performed. The haemoglobin value was within normal range and the serum β-hCG was 2,763mUI/mL. The ultrasonography at the ED showed an in uterus gestational sac and another one inside the right fallopian tube; in both gestational sacs cardiac activity was absent. HP diagnosis was then established and the patient was admitted at the Obstetrics Ward for surveillance and ultrasonographic/laboratorial reassessment; complete miscarriage of the uterine pregnancy occurred but methotrexate was necessary for the treatment of persistent tubarian pregnancy. Conclusion: When evaluating a pregnant woman with pelvic pain and vaginal bleeding one should always be aware of several differential diagnosis amongst which HP should be considered. If the patient has in uterus viable pregnancy the treatment of the ectopic concomitant gestational sac should be as conservative as possible; methotrexate should not be used in that situation as it leads to uterine pregnancy miscarriage in about one third of the patients

Mediastinitis after aorto-coronary bypass surgery

OBJECTIVES: To identify risk factors in 60 cases of mediastinitis amongst 2512 patients (2.3%) subjected to isolated coronary bypass surgery from March 1988 through December 1995, treated by a closed irrigation/drainage system. PATIENTS AND METHODS: The mean age of the 60 patients was 56.9 +/- 6.8 years (45-81 years) and 55 (91.6%) were male. Early mediastinal reexploration was performed in all cases immediately after the diagnosis of mediastinitis, with debridement of necrosed tissues, followed by implantation of a closed-circuit irrigation system of the mediastinum constituted by irrigation catheter and drain, closure of the sternum and skin, and specific systemic antibiotic therapy. The mean interval between the original surgery and reexploration was 9.4 days (range 6-14 days). No patient required more extensive procedures, namely omental or muscular flaps. Twenty potential risk factors in patients with mediastinitis, including diabetes mellitus, obesity, coexistence of peripheral vascular disease, decreased LV function, use of inotropes, mediastinal blood drainage and utilization of double IMA, were compared with the group without mediastinitis. RESULTS: Mean cardiopulmonary bypass time was 74.1 +/- 8.1 min, anesthetic time 3.5 +/- 0.8 h and postoperative mechanical ventilation 18 +/- 3 h. A total of 23 patients (38.3%) received one IMA and 35 (58.3%) two IMAs. In the postoperative period, 7 of the 60 patients (11.6%) had required inotropes because of low output. Mediastinal blood loss was 1112cc +/- 452cc and 9 patients (15%) were transfused. Cultures were positive in 40 cases (66.6%) and the most frequent infecting agent was the Staph. epidermidis in 25 cases (62.5%), followed by Candida albicans and Enterobacter and Serratia species (7.5% each); 1 patient (1.7%) died and 9 (15%) had renal failure. The irrigation/drainage was maintained for a mean of 9.1 days (5-83 days). Patients with mediastinitis had a significantly higher prevalence of diabetes (41.6% vs. 18.8%; P < 0.01), obesity (48.3% vs. 15.2%; P < 0.001), peripheral vascular disease (11.6% vs. 4.0%; P < 0.05), but a lower incidence of poor LV function (18.3% vs. 32.7%; P < 0.05). A double IMA was used more frequently in patients who had mediastinitis (58.3% vs. 23.5%; P < 0.001) CONCLUSIONS: Diabetes mellitus, obesity, co-existence of peripheral vascular disease and use of double IMA are risk factors for mediastinitis after coronary artery surgery. The efficacy of the closed method of treatment with a mediastinal irrigation/drainage system was increased with early diagnosis and reintervention

Diaphragmmatic eventration: long-term follow-up and results of open-chest plicature

Objective: Diaphragmmatic eventration is a relatively uncommon entity with a simple surgical correction technique - plication of the diaphragm. This study aims to assess the clinical and ventilatory impact of this technique. Materials: From April 1988 to February 2007, we operated on 20 patients (12 men) with diaphragmmatic eventration using the postero-lateral approach and correction by radial plication. The mean age of the patients studied was 56.3+/-15.6 years (range: 13-74 years). A traumatic cause was identified in 13 patients; one patient had a congenital cause and the remainder were of idiopathic origin. Chronic obstructive pulmonary disease and arterial hypertension were present in one-half of the study group, while diabetes mellitus was present in three patients. Dyspnoea was the most common complaint in 75% of the patients, and thoracic pain was present in 25%. The mean forced expiratory volume in 1s (FEV(1)) and vital capacity (VC) were 66.2+/-15.3% and 70.4+/-16% of the predicted values, respectively. Results: There was no operative mortality. Apart from a patient with moderate/severe pain and another who had pneumonia, there were no other important perioperative complications. Average drainage time was 3.3+/-1.6 days (range: 2-7 days). Hospitalisation time was 6.2+/-1.6 days (5-10 days). Follow-up was complete, for a mean of 59.6+/-55.1 months (4-206 months). There were three late deaths (one sudden, one stroke and one trauma). Eight of the 17 survivors (47%) are asymptomatic. According to the MRC/ATS grading system, the dyspnoea score was 2.06+/-0.97 preoperatively and 1.06+/-1.14 postoperatively (p=0.007). At follow-up, the FEV(1) was 76.1+/-20.1% and the VC was 78.4+/-17.3% (p>0.1). Two patients had chronic pain. Conclusion: Plication of the diaphragm is a safe and efficient procedure. Most patients experienced significant clinical improvement with enhancement of the FEV(1) and VC. Chronic surgical pain still remains a potential problem with the classical approach

Lung metastases from colorectal cancer: surgical resection and prognostic factors

OBJECTIVE: To analyse our experience with excision of lung metastases from colorectal carcinoma (CRC), and to evaluate clinically relevant prognostic factors, identifying the cluster of patients who would benefit from this procedure. METHODS: Sixty-one patients, 42 men (69%), with primary CRC who underwent 94 curative resections of pulmonary metastases were retrospectively reviewed. Age was 30-80 years (mean 61.2+/-15). Population was analysed for age, sex, disease-free interval (DFI), prethoracotomy carcinoembryonic antigen (CEA) level, location and histology of primary tumour, number of lung lesions (and size of largest resected metastasis), type of lung resection, nodal involvement (hilar/mediastinal), use of adjuvant treatment, morbid-mortality and immediate and follow-up survival. RESULTS: Mean DFI was 29+/-22 months (range 5-132 months). There was no hospital mortality and significant morbidity occurred in five patients (8.2%). Mean follow-up was 39+/-4 months (range 4-173 months). Mean overall survival and disease-free survival were 67+/-16 months and 52+/-6 months, respectively. Three-, 5- and 10-year survival rates from date of primary colorectal resection were 83%, 71% and 43%, respectively. Three-, 5- and 10-year survival rates from date of lung resection were 61%, 48% and 11%, respectively. Five-year survival was 57% in patients with normal prethoracotomy CEA levels and 18% for those with high levels (>5 ng/ml) (p=0.039). CONCLUSIONS: Pulmonary metastasectomy has potential survival benefit for patients with metastatic colorectal carcinoma. Low morbidity and mortality rates, contrasting with lack of any other effective therapy, justify aggressive surgical management. Single deposits, DFI >36 months and normal prethoracotomy serum CEA were significant independent prognostic factors

Minimal Criteria for the Diagnosis of Avulsion of the Puborectalis Muscle by Tomographic Ultrasound

Introduction and hypothesis Puborectalis avulsion is a likely etiological factor for female pelvic organ prolapse(FPOP). We performed a study to establish minimal sonographic criteria for the diagnosis of avulsion. Methods We analysed datasets of 764 women seen at a urogynecological service. Offline analysis of ultrasound datasets was performed blinded to patient data. Tomographic ultrasound imaging (TUI) was used to diagnose avulsion of the puborectalis muscle. Results Logistic regression modelling of TUI data showed that complete avulsion is best diagnosed by requiring the three central tomographic slices to be abnormal. This finding was obtained in 30% of patients and was associated with symptoms and signs of FPOP (P<0.001). Lesser degrees of trauma (‘partial avulsion’) were not associated with symptoms or signs of pelvic floor dysfunction. Conclusions Complete avulsion of the puborectalis muscle is best diagnosed on TUI by requiring all three central slices to be abnormal. Partial trauma seems of limited clinical relevance