The role of splenectomy in myelofibrosis with myeloid metaplasia.

AIM: In this paper we retrospectively analyzed prospectively-collected data on our myelofibrosis with myeloid metaplasia (MMM) patients who underwent splenectomy. The aim was to ascertain the hematological response and any resolution of symptoms existing prior to splenectomy; redefining timing and role of splenectomy in the treatment of MMM. METHODS: This prospective study considered 31 patients with MMM who underwent splenectomy for transfusion-dependent anemia, thrombocytopenia, abdominal swelling and pain. Postoperative work-up consisting in laboratory tests and clinical evaluation performing a quality of life (QoL) test based on EORTC QLQ-C30 questionnaire. Follow-up data were collected for one year after surgery. Statistical analysis used Student's t-test, the Mann-Whitney rank sum, Fisher's exact test, the Friedman test and the Wilcoxon test. RESULTS: Mortality was 3.2%. Respiratory symptomatic complications occurred in 35.4%. In all patients the need for blood transfusions was significantly reduced (P=0.005). An improvement in the painful symptoms was reported and a significant improve of postoperative quality of life was observed at one year after surgery. CONCLUSION: In our experience splenectomy is associated with limited perioperative mortality and morbidity. Acute complications are almost exclusively limited to respiratory tract. The removal of spleen seems can be recommended to increase the QoL and to palliate hematological disorders in patients no more responder to chemotherapy.

[Internal carotid artery aneurisms: two cases]. / Anévrismes de l'artère carotide interne extra-crânienne: à propos de 2 cas.

INTRODUCTION: Aneurysms of the extracranial portion of the internal carotid artery (ICA) are rare (accounting for only 0.1-2% of all surgical procedures affecting the ICA, 0.4-1% of all arterial aneurysms, and 4% of all aneurysms involving peripheral arteries), but they are nonetheless clinically significant because of the high related risk of cerebral thromboembolism. Given the rarity of these lesions, it seems worthwhile to report on two extracranial ICA aneurysms, one of atherosclerotic, the other of fibrodysplastic etiology that came under our observation. PATIENTS AND METHODS: Our experience concerns just two cases, treated at the Department of Surgical and Gastroenterological Sciences of the Policlinico G.B. Rossi in Verona, presenting with very different clinical and instrumental findings, and requiring a different surgical treatment. The former underwent resection of the aneurysm and end-to-end reconstruction; in the latter, we performed a carotid transposition with internalization of the external carotid artery. RESULTS: Neither patient suffered from any major or minor neurological complications during or after surgery, and the follow-up confirmed a normal extracranial carotid patency. CONCLUSIONS: Based on our, albeit limited experience and an analysis of the literature, we make a few points concerning the diagnostic approach (which differs from the case of stenosing carotid lesions), the indications and type of treatment for extracranial ICA aneurysms.

[Treatment of celiac trunk aneurysms: personal experience and review of the literature]. / Traitement des anévrismes du tronc coeliaque: expérience personnelle et revue de la littérature.

INTRODUCTION: Celiac trunk aneurysms represent 4% of all splanchnic artery aneurysms. These lesions are thus extremely rare but yet have a significant clinical importance. Mortality, mainly related to site characteristics, is a significant risk (14%) in the event of rupture. PATIENTS AND METHODS: We put forward our experience in both diagnosis and treatment in three patients, two women and one man (average age 55.3 years, range 35-74), presenting aneurysms involving the celiac trunk. The preoperative diagnosis was established successively with ultrasonography, CT scan and angiography. Two patients were treated via an open surgical approach while endovascular percutaneous treatment was performed for the third patient. RESULTS: Mortality was null at 13 days on average from admission for the surgical patients and 4 days for the patient treated endovascularly. Postoperative complications were modest: pulmonary thickening with pleural effusion for the two surgical patients (spontaneous resolution), while for the third patient treated with an endovascular method, the stent migrated to a splanchnic arterial branch, with no consequence for the spleen. The average follow-up was 19 months (range 14-24). Full exclusion of the aneurysm was maintained at four months for the aneurysm treated percutaneously. A patent celiac was also maintained for the patients treated surgically. CONCLUSIONS: Considering the largely unforeseeable outcome and the high risk of rupture, we suggest that all the patients presenting this type of aneurysmal lesion should be treated. This attitude is widely advocated in the literature. Moreover, we noted null mortality in our small series, with only one percutaneous "re-do" case; resolutive at last control. With the present improvement in stent technology, endovascular treatment should be preferred. Patients should be treated surgically only if a percutaneous procedure would be risky or technically unfeasible due to the size of the aneurysm or its anatomic features.

[Endoscopic diagnosis of the lacrimal drainage system]. / L'endoscopie lacrymale à visée diagnostique.

Endoscopic study of the lacrimal drainage system is the only way to directly observe the lacrimal sector of the lacrimal drainage system. The first experiments on this subject date from 1979. Recent technological progress has allowed us to test the latest instruments. These experiments have demonstrated the unquestionable progress of endoscopes and their potential contribution to diagnosing lacrimal pathology. However, certain factors still limit their everyday use. Image improvements and low cost will no doubt facilitate its integration in the diagnosis and therapy of the lacrimal drainage system.

Treatment of an aneurysm of the celiac artery arising from a celiomesenteric trunk. Report of a case.

INTRODUCTION: Visceral artery aneurysms (VAA) are rare, frequently present as a life-threatening emergency and are often fatal. The celiacomesenteric trunk (CMT), a common origin of the celiac trunk (CT) and the superior mesenteric artery (SMA) from abdominal aorta, is quite rare. Aneurysms that involve this celiomesenteric anomaly are even rarer and in the last 32 years have been reported in only 20 cases in the literature. PRESENTATION OF CASE: We describe a case with 30mm aneurysm arising from a CMT. In general, an aneurysm that is 20mm or greater in size is considered to be significant enough to warrant treatment. Abdominal VAA sometimes can be treated with low-invasive procedures: our patient required open surgical repair with the celiac artery replanted on to the aorta. DISCUSSION: The clinical course was complicated only by an increase of hepatic cytolysis enzymes, and by a low output pancreatic fistula, treated conservatively. The patient was discharged on the fifteenth postoperative day. One month after discharge, imaging revealed a good patency of all reconstructed arteries. In the subsequent 36-month follow-up period, the patient reported no clinical episodes. CONCLUSION: Our finding of a very rare case of a celiomesenteric anomaly with a concurrent aneurysm is extremely rare (20 cases in word literature in the last 32 years). The feasibility of the endovascular approach for aneurysms originating from the common celiomesenteric trunk depends mainly on aneurysmal location, diameter and neck size. In case of specific unfit anatomy, a careful surgical treatment can ensure the best results.

Italian multicenter study on the safety and efficacy of coenzyme Q10 as adjunctive therapy in heart failure. CoQ10 Drug Surveillance Investigators.

Digitalis, diuretics and vasodilators are considered the standard therapy for patients with congestive heart failure, for which treatment is tailored according to the severity of the syndrome and the patient profile. Apart from the clinical seriousness, heart failure is always characterized by an energy depletion status, as indicated by low intramyocardial ATP and coenzyme Q10 levels. We investigated safety and clinical efficacy of Coenzyme Q10 (CoQ10) adjunctive treatment in congestive heart failure which had been diagnosed at least 6 months previously and treated with standard therapy. A total of 2664 patients in NYHA classes II and III were enrolled in this open noncomparative 3-month postmarketing study in 173 Italian centers. The daily dosage of CoQ10 was 50-150 mg orally, with the majority of patients (78%) receiving 100 mg/day. Clinical and laboratory parameters were evaluated at the entry into the study and on day 90; the assessment of clinical signs and symptoms was made using from two-to seven-point scales. The results show a low incidence of side effects: 38 adverse effects were reported in 36 patients (1.5%) of which 22 events were considered as correlated to the test treatment. After three months of test treatment the proportions of patients with improvement in clinical signs and symptoms were as follows: cyanosis 78.1%, oedema 78.6%, pulmonary rales 77.8%, enlargement of liver area 49.3%, jugular reflux 71.81%, dyspnoea 52.7%, palpitations 75.4%, sweating 79.8%, subjective arrhytmia 63.4%, insomnia 662.8%, vertigo 73.1% and nocturia 53.6%. Moreover we observed a contemporary improvement of at least three symptoms in 54% of patients; this could be interpreted as an index of improved quality of life.

A clinical assessment of the potential for pharmacological interaction between nimesulide and digoxin in patients with heart failure.

The potential interaction between nimesulide, a nonsteroidal anti-inflammatory drug, and digoxin was studied in 9 patients [6 males, 3 females; mean age 67 (range 57 to 70) years] with mild heart failure. All patients were receiving maintenance therapy with digoxin (0.25 mg/day, orally) and were treated with oral nimesulide 100mg twice daily for 7 days. Blood samples were collected at 8am and 6pm for 4 days before and throughout the nimesulide treatment period for determination of serum digoxin concentrations. Physical health, electrocardiographic recordings and blood and urine samples were also monitored. Mean serum digoxin concentrations remained within the normal therapeutic range throughout the study despite large interindividual variation. Furthermore, there were no significant differences between the morning and afternoon serum digoxin concentrations and there was no major change in the clinical condition of any patient. These results indicate that short term administration (7 days) of conventional therapeutic doses of nimesulide (100mg twice daily) does not modify the serum digoxin profile in patients with low class heart failure treated with a maintenance dose (0.25 mg/day) of this cardiac glycoside.

Dopaminergic control of serum potassium.

Metoclopramide, a dopaminergic inhibitor, injected in 9 normal volunteers, was followed by a prompt decrease of serum potassium (10--20 min; p less than 0.01) and by an increase of plasma aldosterone (p less than 0.01). Renin slightly increased at 45 min (p less than 0.05); insulin and cortisol did not show any significant increase. The urinary excretion of potassium rose after metoclopramide (p less than 0.05). A bolus of aldosterone (250 micrograms i.v.) in 4 normal subjects was not followed by any modification of serum potassium, but increased urinary potassium excretion (p less than 0.05); the injection of metoclopramide in two patients with an aldosterone-secreting adenoma of the adrenal and in one patient with Addison's disease induced a decrease of serum potassium in absence of any modification of plasma aldosterone. The decrease of serum potassium after metoclopramide is not explained by changes of aldosterone or insulin, considered the most important hormonal controls of potassium. The rapidity of potassium decrease implies a change of distribution of potassium between extra- and intracellular compartments, which, in turn, may stimulate aldosterone secretion. It is conceivable that the dopaminergic system has a role in the control of serum potassium.

Outpatient surgery of varices of the lower limbs: experience of 2,568 cases at four universities.

Outpatient surgery of varices of the lower limbs is currently considered a viable alternative to traditional surgery with hospitalization. This paper reports the experience of 4 university groups (Padua, Modena, Verona, Milan), where outpatient treatment has been used since 1987. From June 1987 to June 1992, 2,568 lower limb varices were treated in this way. Different techniques of anaesthesia were used (local infiltration, combined local and general, general, subarachnoid). In all cases, crossectomy was combined with short or long saphenous stripping. There were no intra- or perioperative deaths, and only limited morbidity. Postoperative hospitalization was required in only 2 cases: for hemorrhaging of the inguinal wound in one case, and headache 2 days after spinal anaesthesia in the other. In 2 separate samples of 100 patients, 88 and 89 indicated satisfaction with the surgical treatment. In conclusion, outpatient surgery of varices can be based on the same techniques as in-patients treatment. The risks of surgery and anaesthesia in specialised centres are very limited, with scope for a variety of anaesthetic techniques according to facilities available. Patients satisfaction is high.

[Significance of certain neoplastic markers in a series of patients with lung cancer]. / Significato di alcuni marcatori di neoplasia in una casistica di pazienti affetti da carcinoma polmonare.

Perchloric acid soluble proteins (PPS) and their content of N-acetylneuraminic acid (NANA) may serve as valuable tumor markers for a variety of malignant neoplasms. To evaluate their clinical significance, PPS, NANA and carcino-embryonic antigen (CEA) were measured in 32 patients with lung cancer. High PPS (greater than or equal to 0,73 mg/ml) and NANA (greater than or equal to 96 micrograms/ml) levels occurred in 8 of 50 (16%) healthy volunteers and respectively in 22% and 54% of patients. CEA levels were high (greater than or equal to 3 ng/ml) in 1 out of 21 (5%) healthy volunteers and in 83% of the patients; 84% of the patients showed an elevation of NANA and of CEA. The highest values of the three markers seem to be associated with extensive disease but no statistically significant difference has emerged from the comparison of patients with limited disease with the ones with extensive involvement. Changes of the tumor mass correlate with changes of serum levels of PPS, NANA and CEA. It is concluded that CEA determination is clinically valuable in lung cancer, while PPS and NANA do not provide greater information.

[Carotid lesions and vascular risk factors]. / Lesioni carotidee e fattori di rischio vascolare.

BACKGROUND AND AIMS: Recent studies have underlined a significant incidence of peripheral arterial occlusive disease (PAOD) of the lower limbs in the general population which is often wrongly diagnosed. The "classic" risk factors--like dyslipidemia--are not always present in significant percentages in patients suffering from PAOD of the lower limbs. The aim of this study was to evaluate the incidence of the most common vascular risk factors (smoking, hypertension, hyperglycemia, dyslipidemia) in patients suffering from stenosing lesions of the extracranial carotid axes, comparing the data with similar findings in lower limbs. Moreover, the authors evaluated the association between these risk factors, carotid atheromatous lesions and ischemic cardiomyopathy (CI). METHODS: A retrospective study was performed to evaluate the data from 1643 patients examined consecutively. A total of 636 (age > 40, carotid stenosis > 40%, presence of risk factors) were considered eligible. RESULTS: The results showed that, contrary to the findings in patients suffering from PAOD, diabetes was not among the most frequently associated risk factors, whereas a relatively large number of patients had a history of smoking, including both smokers and ex-smokers. CONCLUSIONS: The difference in the most frequent risk factors identified for PAOD and carotid lesions suggests different etiopathogenetic mechanisms for the two districts.

[Unidentified arteriopathy: an epidemiological study. The preliminary results]. / L'arteriopatia misconosciuta: studio epidemiologico. Risultati preliminari.

The importance of the social and medical impact of arteriosclerotic disease fully justifies a series of studies focused on improving knowledge of this pathology before symptoms become manifest, so as to orientate treatment increasingly towards prevention. The aim of this study-of which the authors now report the preliminary results-was to establish the incidence of unidentified lower limb arteriopathy in the general population and to evaluate the relations between this and the possible presence of risk factors. The authors therefore decided to evaluate patients attending outpatient General Surgery clinic for symptoms manifestly not related to lower limb vascular pathology (arterial or venous). Patients suffering from any arterial disease were likewise excluded from the study. The importance of performing a large-scale epidemiological study (sample size, stratification of population studied, vast geographic area, etc.) persuaded the authors to adopt a multi-centre structure. Twelve General Surgery departments at hospitals in the Veneto area were involved: each was required to evaluate an average of 150 patients, giving a total of 1950 subjects. The method of study chosen was the residual pressure index (RPI) measured using Doppler velocimetry. Patients were divided into two study groups: patients in Group A (with pathological RPI) were re-evaluated after correction for risk factors, integrated or not by medical treatment. Preliminary results relating to 46% of the total sample show that 26.7% of the subjects present pathological RPI. The evaluation of risk factors and the comparison of their presence in the two groups (A--pathological patients, B--non-pathological subjects) reveals significant differences regarding diabetes and hypertension.

[Morgagni's hernia in infancy]. / Ernia di Morgagni in età infantile.

Morgagni's hernia is an uncommon type of diaphragmatic hernia fairly exceptional in pediatric age. A case of Morgagni's hernia, observed by chance in a 15-month-old infant, is presented. The authors present the fundamental embryogenic stages of the diaphragm and stress the clinical manifestations and diagnostic procedures to reach the diagnosis, as well as the advantages of early treatment in order to prevent possible complications.

[Surgical treatment of upper lid trichiasis with partial tarsal rotation: a new procedure]. / Traitement du trichiasis localisé par rotation tarsale segmentaire: une nouvelle technique chirurgicale.

The authors' report a new surgical technique for partial isolated upper lid trichiasis. This ten minutes' time consuming procedure is easy to perform. It allows a fair cosmetic appearance of the operated eyelid and permits a reliable uneventful outcome. The technique consists to severe the lid margin and the tarsal plate on a distance of 3 to 4mm, at each part of it's trichiased area, in order to allow the pathological part of the eyelid to rotate like a shutter. The rotation of the ill eyelid segment is strengthen by a partial resection of the anterior lamella localizated here and there of the lid crease above the trichiased part of the eyelid, which allows to place everting sutures between the superior part of the tarsus and the two edges of the anterior lamella incision.

[Surgical treatment of congenital eyelid malpositions in children]. / Chirurgie des malpositions palpébrales congénitales de l'enfant.

Congenital eyelid malpositions can be isolated or associated with other structures such as the eyeball, ocular muscles, and lacrimal pathways. It is important to separate eyelid malpositions, which are topographical disorders, from eyelid malformations, which are constitutional morphological disorders. Only eyelid malpositions will be described in this paper, with a distinction made between static and dynamic disorders. Static disorders include epiblepharon, congenital ectropion and entropion, epicanthus, telecanthus, and centurion syndrome. Dynamic disorders group ptosis and congenital eyelid retractions. Epiblepharon is characterized by the absence of adhesion between the lower eyelid retractors and the orbicularis-skin layer, which allows the anterior lamella to roll over. Congenital entropion and ectropion are very rare. They most often require surgical treatment. Epicanthus involves a semi-lunar fold of skin extending from the upper eyelid across the medial canthal area to the margin of the lower eyelid. Four types (supra-ciliaris, palpebralis, tarsalis and inversus) of epicanthus are described. Treatment requires surgery. Telecanthus is defined as an increased distance between canthi. Primary telecanthus results from attenuation of the medial canthal tendons and is usually associated with other soft tIssue abnormalities such as epicanthus or blepharophimosis, or is seen after trauma. Secondary telecanthus is caused by underlying bony malpositions with an abnormal separation between the orbits because of an increased thickness of the interorbital bones such as that seen in hypertelorism or in other complex craniofacial syndromes. Euryblepharon is distinguished by an enlargement of the horizontal palpebral fissure associated with enlarged eyelids. The etiology is unknown and patients may benefit from surgery. Congenital ptosis is characterized by a deficiency of the levator muscle. They are most often unilateral and isolated. When associated with other abnormalities such as squint, synkinesis, blepharophimosis, or craniofacial syndromes, surgical treatment may require several-stage procedures. On the other hand, isolated congenital ptosis is usually treated in childhood (3-6 years) in a single stage. Congenital eyelid retractions may affect either the lower or the upper eyelid. They make up a rare condition and most cases are associated with craniofacial syndromes such as Crouzon or Apert syndromes. Upper eyelid retractions may spontaneously improve in some cases, but lower eyelid retractions do not. Treatment requires surgery, depending on the corneal consequences.

[Examination and surgical indications for blepharoplasty]. / Examen et indications opératoires des blépharoplasties.

Cosmetic blepharoplasty has become very popular these last years and can easily be performed with local anesthesia. Its benefits are numerous and side effects minimal. Nevertheless, the different types of eyelid impairment are numerous and the patient is often not aware of them. This paper discusses the importance of the clinical examination targeting proper diagnosis of the type of eyelid impairment so as to recommend the most adapted surgical technique. The different types of cosmetic eyelid impairment are presented.

[Postoperative ptosis: etiopathogenesis, clinical analysis, and therapeutic management. Apropos of a series of 43 cases]. / Les ptosis postopératoires: étiopathogénie, analyse clinique et prise en charge thérapeutique. A propos d'une série de 43 cas.

PURPOSE: Acquired postoperative ptosis (PP) are difficult to situate in the current classification of ptosis. Assessement of the mechanisms, the clinical features and the possible treatments of these PP would suggest a new classification of ptosis. MATERIAL AND METHODS: Among 260 cases of surgically corrected ptosis, 43 cases of PP (16.5%) were detected and analyzed. RESULTS: Forty cases of PP were eligible for this study. Their responsible mechanisms were aponeurotic (57.5%), mixed (aponeurotic and/or myogenic and/or neurogenic) (27.5%) and myogenic (15%). PP was assessed in most cases as being mild (77.5%) and the levator's muscle contraction was most often mildly impaired (77.5%). In these cases, surgical procedure was performed: levator aponeurosis disinsertion repair (85%), Fasanella-Servat procedure (2.5%), frontalis sling (2.5%) and other surgical procedure with synthetic materials (10%). Postoperative complications included 1 case of persistent lid edema and 4 cases of spontaneous suture rupture. Six patients (15%) were secondarily reoperated: 2 for overcorrection (5%) and 4 for undercorrection (10%). The general outcome was good in 90% of cases, insufficient in 5% of cases and unsatisfactory in 5% of cases. CONCLUSION: This study confirms the previously described features of the PP: onset after anterior surgery procedures of usually moderate ptosis, induced by an aponeurotic defect mechanism in most cases. The treatment was exclusively surgical: anterior reinsertion of the levator aponeurosis. For better management, we suggest a new ptosis classification: aponeurotic, myogenic, neurogenic and mixed (aponeurotic and/or myogenic and/or neurogenic) and false or pseudo-ptosis.

[A new instrument in eyelid surgery: ptosis forceps with a protected lock]. / Un nouvel instrument en chirurgie palpébrale: la pince à ptosis à cliquet protégé

We report on a new instrument in eyelid surgery: the ptosis forceps with a protected lock. This new device helps the surgeon to dissect the levator muscle to facilitate any type of levator surgery. It is particularly useful in the adjustment of the superior lid margin level towards the corneoscleral area in ptosis surgery with levator resection.

[Etiologic causes of ptosis about a serie of 484 cases. To a new classification?]. / Etiopathogénie des ptôsis à propos d'une série de 484 cas. Vers une nouvelle classification?

We have analyzed the etiological causes of ptosis in a retrospective study of 484 cases undergoing operation. This study has confirmed how difficult it is to classify ptosis despite the different existing classifications. Some authors divide ptosis into two groups: congenital and acquired. These classifications seem to have been abandoned now for classifications based on the mechanism that instigates ptosis rather than the moment when the deficit developed. We chose to classify ptosis into five subgroups: myogenic (42% of the cases studied), aponeurotic (35.3%), neurogenic (6.8%), mixed (15.9%), and pseudoptosis (enophthalmos, eyelid tumor, hypotropia, etc.), the latter of which were removed from this series. This classification is based on clinical and surgical criteria. It has the advantages of unity, simplicity, and practicality in terms of establishing a treatment plan for a given ptosis patient. Indeed, each subgroup requires a particular clinical examination and a more stereotyped surgical treatment.

[Analysis of the efficacy of early probing in the treatment of symptomatic congenital lacrimal duct obstruction in infants. Apropos of 92 cases]. / Analyse de l'efficacité du sondage précoce dans le traitement de l'obstruction congénitale des voies lacrymales symptomatique du nourrisson. A propos de 92 cases.

INTRODUCTION: Nasolacrimal duct obstruction (NLDO) is a common clinical problem (5 to 6% of newborns). Dacryostenosis is a partial or complete block in the nasolacrimal duct. PATIENTS AND METHODS: The records of 68 patients (92 cases) of NLDO with/without mucocele were reviewed. The probing was performed early and according to the same technique. RESULTS: The age at the time of probing ranged from 0 to 7 months (mean 4.6 months). The cure rate was 91.3% and the failure rate was 8.70%. DISCUSSION: Our results are consistent with other reports in the literature. The cure rate was higher with a earlier probing and was good after a second tentative of probing. A multiple obstruction of nasolacrimal system was cause of a failure. This was not the case with mucocele. The optimal timing of the probing is discussed in this report and we preferred to perform the probing to 4 at 6 months. CONCLUSION: We showed a high cure rate with early probing in our series. These results should be confirmed by a prospective randomised study (in process in our institution).