Association of severe myoclonic epilepsy of infancy (SMEI) with probable autoimmune lymphoproliferative syndrome-variant.

The paper reported on a case of severe myoclonic epilepsy of infancy (SMEI) associated with a probable autoimmune lymphoproliferative syndrome variant (Dianzani autoimmune lymphoproliferative disease) (DALD). A male patient with typical features of SMEI and a SCN1A gene variant presented in the first year of life with multiple lymph nodes, palpable liver at 2 cm from the costal margin, neutropenia, dysgammaglobulinemia, relative and sometimes absolute lymphocytosis. Subsequently the patient presented with constantly raised IgA in serum and positive antinuclear and thyroid antimicrosomal antibodies. The diagnosis of probable autoimmune lymphoproliferative syndrome was made; arthritis, skin and throat blisters, which appeared subsequently led to the diagnosis of linear IgA disease. On the basis of these unique associations, the Authors hypothesized that autoimmunity may be partly responsible of the severe epileptic symptomatology, perhaps mediated by autoantibodies against sodium channels or by accompanying cytotoxic T-lymphocytes. Corticosteroid treatment ameliorated the epilepsy and laboratory tests. Future studies will be necessary to evaluate the relevance of autoimmunity in SMEI.

Severe necrotic and septic pancreatitis. Indications to endoscopic, surgical, and nutritional therapy.

Severe acute pancreatitis (SAP) management has changed over the last fifteen years, and from too aggressive behaviour, we moved to a cautious one. In every case, we can appreciate defect of extremist conceptual position. We reviewed our strategy on disease treatment, and we analyzed treatment of single cases. We collected 4 SAP cases from January 2009 to January 2010. All patients were septic, and we adopted the same approach for all of them, avoiding surgery without peritoneal infection. In all patients we placed jejumostomy and, after cleaning of septic site, we started immediate enteral nutrition (EN). Antibiotic therapy against Gram+, Gram- and antifugal drug had been started. No one died and all patients were back to an active life even if social costs are considerably high especially due to very long hospital stay.

Microcrystalline cellulose as reinforcing agent in silicone elastomers.

Cellulose is commonly used as filler for the reinforcement of polymer materials but data in the case of silicones remain rare. In this work we report the modification of microcrystalline cellulose (MCC) fibers from cotton linters by propargyl bromide, in aqueous medium without alteration of the crystalline domains. The analysis evidenced the efficient grafting of alkyne functions at the surface of the fibers, the DS being 0.5. The resulting MCC-Alkyne fibers were introduced within a bi-component reactive silicone formulation (up to 20wt%), allowing the formation of network through hydrosilylation reaction in which MCC-Alkyne played the role of a reactive fillers. Comparison between the properties of composites prepared with unmodified MCC and MCC-Alkyne highlighted a densification of the network and an enhancement of mechanical and thermal properties when coupling reactions occurred. Mechanical properties of silicone elastomers were better if the load of MCC-Alkyne remains low.

Fetus in fetu removal in a 47-year-old man.

A case of removal of a fetus in fetu in a 47-year-old man is reported. The patient had an upper abdominal mass since birth that had never caused any subjective symptoms. A preoperative computed tomographic scan was useful to confirm the diagnosis. The operative specimen consisted of a cystic mass about 20 cm in diameter, situated in the upper retroperitoneal space. The cyst was full of a yellowish fluid and hairs. A bony structure, about 10 cm in diameter, contained a vertebral axis connected to the ribs and was adherent to the cystic wall. To our knowledge this is the first reported case of fetus in fetu described in an adult man. The tumor, present for 47 years, did not grow or cause any complications and did not show any sign of malignancy.

Early degeneration of the cerebellar cortex, particularly the granular cells.

An 8 month old infant, who died of severe gastroenteritis, presented a degeneration of the cerebellar cortex involving cells arising from the outer granular layer as well as Purkinje and Golgi II cells. Residual Purkinje cells showed vacuolar change of the cell body and dendritic abnormalities. Related lesions were atrophy of the inferior olives and degeneration of the mossy fibers.

Does extended pancreaticoduodenectomy increase operative morbidity and mortality vs. standard pancreaticoduodenectomy?

The poor prognosis of pancreatic carcinoma after resection is related to distant metastases and local recurrence that is characterized by a strong tendency to infiltrate the retroperitoneal tissue and spread along the neural plexuses and lymph nodes. Thorough clearance of these tissues around the celiac and mesenteric axes, aorta, and inferior vena cava from the diaphragm to the inferior mesenteric artery (extended pancreaticoduodenectomy may lower the rate of local recurrence, but the procedure has been criticized for its higher morbidity and mortality. Our aim was to compare extended pancreaticoduodenectomy (EPD) with standard pancreaticoduodenectomy (SPD) in terms of postoperative morbidity and mortality. Data from 47 patients who underwent either EPD (n=24) or SPD (n=23) between November 1992 and October 1995 were retrospectively analyzed. Preoperative laboratory findings, operative risk (according to the American Society of Anesthesiologists classification), type of operation (classic Whipple vs. pylorus-preserving Whipple), operative time, intraoperative blood and plasma transfusion, postoperative morbidity and mortality, and postoperative hospital stay were scrutinized. The results showed that all of the parameters considered were similar in the EPD and SPD groups (intraoperative blood transfusion 800+/-490 ml vs. 700+/-586 ml, postoperative mortality 0% vs. 4.3%, overall morbidity 45.8% vs. 47.8%, surgical morbidity 37.5% vs. 34.7%, and postoperative hospital stay 16+/-8.1 days vs. 17+/-13.1 days. These two groups differed only in the operative time, which was significantly longer for EPD than for SPD (360+/-68.9 minutes vs. 330=66.9 minutes, P=0.02). Although the operative time is increased with EPD, there does not appear to be an increase in intraoperative complications, postoperative morbidity and mortality, or postoperative hospital stay with this procedure. However, definitive confirmation of these results can only be provided by a prospective randomized study.

Repeated hepatic resection.

Sixteen patients underwent repeated hepatic resections over a 16 year period. The cases were divided into two groups: group A, non-planned repeated resections (14 cases), and group B, planned repeated resections (two cases). Group A is composed of patients requiring re-resection as a result of the hepatic re-recurrence of the neoplasia (three hepatocellular carcinomas, nine metastases from colorectal carcinoma, and two metastases from carcinoid tumor). Group B is composed of two cases (hepatocellular carcinoma and metastases of leiomyosarcoma) where the extent of the disease was incompatible with radical resection in a single time thus making necessary to plan for repeated operations. The need for correct preoperative assessment of hepatic performance using CT, US and Tc 99m HIDA scan, as well as intraoperative ultrasonography is stressed.

[Diagnosis and treatment of traumatic diaphragmatic hernia with delayed presentation]. / Diagnosi e trattamento dell'ernia traumatica del diaframma a tardiva presentazione.

AIM: To evaluate the clinical picture, diagnostic techniques and most appropriate treatment in traumatic diaphragmatic hernia with delayed presentation on the basis of personal experience and in the light of other published studies. EXPERIMENTAL DESIGN: Review of cases treated. SETTING: Patients treated in University General Surgery wards. PATIENTS: Those patients in whom diagnosis was made some time after trauma and after the acute event were selected from a group of patients with traumatic diaphragmatic hernia. SURGERY: All patients underwent surgery to reduce hernia and repair the diaphragmatic lesion. MEASUREMENTS: All clinical findings were examined together with the tests performed and the type of treatment carried out. RESULTS: The diagnosis was made between 3 months and 3 years after the injury. Three patients presented manifest symptoms of high intestinal occlusion on entry. Radiological alterations were present in standard chest X-rays in all patients and digestive tract contrast radiography was positive for the diagnosis of hernia in 3 out of 4 cases in which it was performed; a preoperative diagnosis of hernia was obtained in 4 cases. Patients were operated using a thoracotomy (3 cases) or combined laparothoracotomy access (2 cases); the diaphragmatic lesion, localised in all cases in the cupula of the left hemidiaphragm, was repaired using separate sutures in non-reabsorbable material without the use of grafts. One patient died postoperatively owing to septic complications. CONCLUSIONS: traumatic diaphragmatic hernia with delayed presentation involves severe complications that increase morbidity and operating mortality.

[Butterfly mesh for the treatment of hernia]. / Una rete a papillon per il trattamento dei laparoceli.

Incisional hernias (IH) occur with an incidence of 2 to 11% after laparotomy and represent a huge social and economical problem. Polypropylene meshes remarkably decreased the incidence of recurrence after first repair. This paper reports a personal method that allows a strong, safe and quick replacement of the abdominal wall with a double layer of polypropylene mesh. This method is reliable for IHs bigger than 4 cm. The sac and the surrounding fascia are cleaned from fat and scarred tissue: peritoneum is dissected up to at least 3 cm all around the edges of the hernial sac and under the fascia, as far as possible. Two sheets of PM, 3 cm larger than the defect, are sutured together with non-absorbable running suture (polypropylene 2-0) from the center to 2 cm from the extremities at the bank. The inferior mesh is extended and fixed under the fascia with polypropylene mattress stitches. When the peritoneum is not present, the edges of the mesh are refolded and sutured to the fascia in order to avoid trauma to the loops. The superior mesh is sutured directly onto the fascia. Closed aspiration drainage is positioned. We performed this method on 20 IHs (from 5 to 25 cm long). At 24 months follow-up we never observed recurrence. This method is similar to abdominal wall suture, and permits tension free repair.

[Hepatocellular adenoma and focal nodular hyperplasia]. / Adenoma epatocellulare ed iperplasia focale nodulare.

The diagnostic findings of hepatocellular adenoma and focal nodular hyperplasia became more frequent in the last years in our as in western experience. The improvement in diagnostic technique, a correct pathological identification and the diffusion of oral contraceptives explain this increase of incidence. In our series were present 11 hepatocellular adenomas and 19 focal nodular hyperplasias: all the HCA cases were radically resected, while only 15 FNH were resected, only two biopsied and two submitted to periodical follow-up. The tendency to spontaneous bleeding and the presence of diagnostic uncertainty versus well-differentiated hepatocellular carcinoma are mandatory indications for radical resections in all the HCA cases. FNH resection is reserved to symptomatic cases and wide wedge biopsy is at least required in presence of diagnostic doubts: the asymptomatic FNH ("hot spot" on Tc99m-HIDA scintigraphy) may be followed-up only.

[Reoperation in necrotizing acute pancreatitis: evaluation of physiopathology and surgical treatment]. / I reinterventi per pancreatite acuta necrotizzante: valutazioni di fisiopatologia e di trattamento chirurgico.

We reviewed our experience of PAN cases operated for complications after a first laparotomy over the period 1992-1994. Over 29 PAN cases, 7 (24%) had been submitted to a second laparotomy or more. Total mortality rate of PAN was 10.3%, while mortality rate of relaparotomy was 14.2%. Haemorrhage and intra-abdominal sepsis were the main cause of relaparotomy (42.8% of the re-operations in both cases). Abdominal wall abscesses (14%) were treated locally; enteric or pancreatic fistulas (34%) were successful treated by drugs, such as somatostatin and octreotide, and / or by total parenteral nutrition. The main diagnostic tools to evaluate clinical course of the patients were computed tomography scan, that seems to gain serial staging of the necrosis and the septic collections. Arteriography is necessary to identify the bleeding source and to perform temporary embolization in the massive arterial haemorrhage before surgical treatment. Moreover, we need radiological exploration to explain fistulas pathways. According to circumstances, we can perform surgically the definitive hemostasis, the pancreatojejunostomy in pancreatic fistulas, and the digestive reconstruction in enteric fistulas. At all events the debridement of necrosis and septic collection is necessary. Up to date, there are not prognostic differences between "closed laparotomy" and "open laparotomy", and we think that the choice is determined only by individual believing of the surgeon.

[The therapeutic strategy in benign liver tumors: a 20-year experience]. / La strategia terapeutica dei tumori epatici benigni: esperienza di 20 anni.

Therapeutic choices for benign liver tumours have changed over the last 20 years. From 1975 to December 1993, we observed 145 hemangiomas (HMG) (57.2% females-mean age 47.3 years, 42.8% males-mean age 50.4 years): we resected 42 symptomatic hemangiomas: mortality rate was 2.3%. 93 HMG without symptoms were only followed-up: 5 of these increased in size and were resected. 27 symptomatic cases over 50 focal nodular hyperplasia (FNH) were resected, 7 cases were resected and 3 biopsied during laparotomy performed for other pathology. Postoperative mortality was nil. 13 cases were only followed-up after diagnosis by imaging techniques and fine needle biopsy: over a mean period of 23 months. No variations have been recorded. Increases in GGT and ALP were present respectively in 34% and 22% of FNH-cases. Scintigraphic techniques were the most diagnostically accurate (96.2%). All 16 hepatocellular adenomas (HCA) were removed (11 females, 5 males), postoperative mortality was nil: oestrogen administration was present in 36.4% of female cases, histological diagnosis v/s well differentiated hepatocellular carcinoma was difficult in 2 cases, whilst 3 cases had spontaneous rupture. We resected also 8 cases of biliary adenomas in order to determine a precise diagnosis v/s liver metastases, and 4 biliary cystadenomas for their malignant potential. Asymptomatic HMG and FNH for their low tendency to increase, can be only observed, whilst HCA must be fully resected for risk of bleeding and misdiagnosis v/s well differentiated hepatocellular carcinoma.

[Iatrogenic rupture of the esophagus: presentation of several cases]. / Le rotture iatrogeniche dell'esofago: presentazione di alcuni casi.

Four cases of iatrogenic rupture of the oesophagus are presented. The site of the lesion was in the cervical tract in 1 case and in the thoracic tract in the other 3 cases. Their etiology was pneumatic endoscopic dilatation for achalasia in 2 cases, endoscopic insertion of a Celestin tube for carcinoma of the thoracic tract of the oesophagus in 1 case, and diagnostic endoscopy in the last one. Instrumental findings were relevant in all cases. All patients underwent surgery. In the patients suffering from achalasia, the rupture was repaired by a patch of the gastric fundus. The patient suffering from carcinoma underwent an oesophageal resection, the one with cervical perforation underwent a mediastinal drainage. There were no deaths or considerable post operating complications. In a patient suffering from achalasia gastro-oesophageal reflux was demonstrated after some months following the operation.

[Trans-sphincteric approach by the Mason method in the local excision of neoplasms of the medium third of the rectum]. / L'accesso trans-sfinterico secondo Mason nell'asportazione locale delle neoplasie del III medio del retto.

The authors report on the trans-sphincteric access to the rectum according to Mason's technique for the local excision of rectal tumors. On the basis of their experience, they confirm that this technique is easy to perform and that it is reliable as regards preservation of anal continence.

[Hepatic angiomyelolipoma (considerations on a case brought to our attention)]. / Angiomyelolipoma epatico (considerazioni su un caso giunto alla nostra osservazione).

Liver lipomas are exceedingly rare: the authors present a case of angiomyelolipoma of the liver in a 65-year-old woman resected by right hemihepatectomy. Radiologic findings and indications to surgical treatment are discussed.

[Retroperitoneal tumors. Tactics, technics and surgical results]. / I tumori retroperitoneali. Tattica, tecniche e risultati chirurgici.

Out of 33 cases of retroperitoneal tumours (TRP), collected throughout 11 years (1970-1981), 8 (24%) resulted benign, 25 (76%) malignant. Liposarcoma resulted the most frequent tumour. In 11 of the 25 cases of malignant TRP (44%), a radical removal of the tumour was performed; in 8 (32%), a palliative demolition, and in the remaining 6 (24%) a simple diagnostic laparotomy. In 10 of the 19 demolitive operations (53%) the removal was extended to contiguous organs: kidney, adrenal glands, colon, pancreas, spleen, bladder, stomach. The postoperative mortality was 6%. The cases of recurrence after operations considered radical were 5 (45%). Out of the 8 patients suffering from benign neoformations, 7 underwent simple removal, and result recovered. The global survival for malignant TRP was 20% after 5 years and 8% after 10 years; as related to the cases subjected to radical removal, it results 45% and 18% respectively. The Authors maintain the essential function of computerized axial tomography (TAC) in the preoperative anatomo-topographic outlining of the retroperitoneal mass and in the early recognition of the remote recurrences. An aggressive surgical behaviour seems to be presently the primary therapeutical solution; nevertheless, the encouraging results obtained through the complementary treatment (radiotherapy and chemotherapy) command the necessity of a pluridisciplinary management of the treatment of TRP as an essential condition for the improvement of the remote results.

[Distal termino-lateral spleno-renal anastomosis, using the Warren technic]. / L'anastomosi spleno-renale termino-laterale distale sec. Warren.

The authors describe 23 cases of distal splenorenal derivation after Warren. Of these patients, 17 received a complete followup clinical hematological and angiographic examination at an average remove of 10 months after surgery. The authors discuss their results and submit that the Warren operation, while really selcetive in most cases when performed, in the long run tends to shung rather too much blood away from the liver; in other words, the effectiveness of the shunt tends to exceed the intended drainage,which should be restricted to the gastro-esophageal vascular bed.

[Anatomo-surgical notes on splanchnicectomy: original research on 15 autopsy observations]. / Premesse anatomo-chirurgiche alla splancnicectomia: ricerca originale su 15 osservazioni autoptiche.

Surgical splanchnicectomy for the relief of neoplastic pain is a palliative strategy in cases of unremovable pancreatic cancer. The first step in the achievement of satisfactory and long-lasting relief of pain is the correct identification of semilunar ganglia and splanchnic nerves during laparotomy. In this light, we tried to estimate the exact location, number, shape, and length of splanchnic nerves and ganglia in 15 corpses (mean age 39.9 years, range 21-74, F/M/ = 5/10). Right and left splanchnic nerves always pierce the diaphragm laterally to the crus. On the right side, the splanchnic nerve always enters the abdomen posterior to the inferior vena cava, on the right edge in 10%, on the middle in 73%, on the left in 17% of the cases. On the left side, the splanchnic nerve pierces the diaphragm strictly thickened to the left edge of the aorta in 66.6% of the cases, close to the left edge in 26.6%, and close to the right edge of the left adrenal gland in 6.8%. The right splanchnic nerve slides almost horizontally on the diaphragmatic bundles, and reaches an area delimited by the coeliac trunk and the superior mesenteric artery. The length of the right splanchnic nerve is 41 mm of the mean (range 20 to 55 mm): the thickness is between 4 and 6 mm. The left splanchnic nerve is shorter (mean 24 mm, range 15; 30 mm). The right splanchnic nerve varies from 2 to 6 ganglionar bodies and varies in size from 4.5 mm to 30 mm; the left nerve varies form 2 to 4 (sizes between 4 mm to 26 mm).(ABSTRACT TRUNCATED AT 250 WORDS)

[Vascular problems in destructive pancreatic surgery]. / Problemi vascolari nella chirurgia demolitiva pancreatica.

Obstacles of vascular type may hinder or prevent pancreatic destruction for tumours of the pancreas or periampullary tumours. Out of 72 major pancreatic demolitions (cephalic duodenopancreatectomy or subtotal pancreatectomy from rt to lt) there were vascular difficulties on 26 occasions (36%), of which 15 were in existence prior to development of the neoplasia (anomalies of rise and course of peripancreatic vessels, stenosis/obliteration of the common hepatic artery in pancreatic head or periampullary tumours) and 11 due to tumoral growth (direct involvement of the mesenteric-portal tract, the hepatic artery and the superior mesenteric artery). The technical solutions used are discussed individually in the light of the physiopathological repercussions consequent on any temporary or permanent interruption of important hepatic and splanchnic vascularisation vessels (hepatic artery and various mesenteric a.), in relation to operating mortality and the prospects of long-term survival. In the most complex cases of direct vascular involvement by the neoplasia, the Author's trend was orientated to a position which, while accepting demolition decisions useful for the widening of the surgical radicality margins, nevertheless avoids extensive demolition solutions imposed by conditions of necessity or considerations of principle that probably do not bring significant advantages as regards long-term prognosis for these tumours.

[Vascular problems in pancreatic demolitory surgery]. / Problemi vascolari nella chirurgia demolitiva pancreatica.

Obstacles of vascular type may hinder or prevent pancreatic destruction for tumours of the pancreas or periampullary tumours. Out of 72 major pancreatic demolitions (cephalic duodenopancreatectomy or subtotal pancreatectomy from rt to lt) there were vascular difficulties on 26 occasions (36%), of which 15 were in existence prior to development of the neoplasia (anomalies of rise and course of peripancreatic vessels, stenosis/obliteration of the common hepatic artery in pancreatic head or periampullary tumours) and 11 due to tumoral growth (direct involvement of the mesenteric-portal tract, the hepatic artery and the superior mesenteric artery). The technical solutions used are discussed individually in the light of the physiopathological repercussions consequent on any temporary or permanent interruption of important hepatic and splanchnic vascularisation vessels (hepatic artery and various mesenteric a.), in relation to operating mortality and the prospects of long-term survival. In the most complex cases of direct vascular involvement by the neoplasia, the Author's trend was orientated to a position which, while accepting demolition decisions useful for the widening of the surgical radicality margins, nevertheless avoids extensive demolition solutions imposed by conditions of necessity or considerations of principle that probably do not bring significant advantages as regards long-term prognosis for these tumours.