[Warren shunt combined with partial splenectomy in children with extra-hepatic portal hypertension, massive splenomegaly and severe hypersplenism].

INTRODUCTION: Extra-hepatic portal vein obstruction (EHPVO) is one of the most often causes of portal hypertension in children. OBJECTIVE: Establishing the importance of shunt surgery in combination with partial spleen resection in selected pediatric patients with EHPVO, enormous splenomegaly and severe hypersplenism. METHODS: Distal splenorenal shunt (DSRS) with partial spleen resection was performed in 22 children age from 2 to 17 years with EHPVO. Indications for surgery were pain and abdominal discomfort caused by spleen enlargement, as well as symptomatic hypersplenism with leucopenia, thrombocytopenia and anemia. The partial spleen resection was performed by ligation of blood vessels to caudal two thirds of the spleen. After ischemic parenchymal demarcation transection with electrocautery LigaSure was performed with preservation of 20-30% of spleen tissue, and then Warren DSRS was created. Platelet and leucocytes counts and liver function tests were obtained before, one month and one year after surgery. Growth was assessed with SD scores (Z scores) for height, weight and body mass index at the time of surgery and one year later. RESULTS: In all patients postoperative period was without significant complications. Platelets and leucocytes counts were normalized. Patency rate of shunts was 100%. Two significant shunts stenosis were observed and successfully treated with percutaneous angioplasty. During the follow-up period (1 to 9 years) all patients were asymptomatic, with improved quality of life and growth. CONCLUSION: Results of our study indicate that shunt surgery with a partial spleen resection is an effective and safe procedure for patients with enormous splenomegaly and severe hypersplenism caused by EHPVO.

Warren shunt combined with partial splenectomy for children with extrahepatic portal hypertension, massive splenomegaly, and severe hypersplenism.

PURPOSE: To establish the importance of shunt surgery combined with partial resection of the spleen for selected pediatric patients with extra-hepatic portal vein obstruction (EHPVO), enormous splenomegaly and severe hypersplenism. Severe hypersplenism is often refractory to treatment with endoscopic sclerotherapy or band ligation and shunt surgery; however, to our knowledge, this is the first such study to be published. METHODS: Distal splenorenal shunt with partial resection of the spleen was performed in 16 of 60 children treated for EHPVO in the Gastroenterology Department of our hospital. Upper gastrointestinal endoscopy had shown esophageal varices of varying grade in all patients and band ligation or endoscopic sclerotherapy had been done for children with a history of bleeding. The indications for surgery were pain and discomfort caused by a large spleen (5-15 cm below the costal margin) and symptomatic hypersplenism with leucopenia, thrombocytopenia, and anemia. Partial resection of the spleen was performed, starting with ligation of the branches and tributaries of the caudal two-thirds. When an ischemic line demarcated the splenic parenchyma, it was transected using electrocautery or LigaSure, leaving 20-30 % of splenic tissue. After the spleen resection, a Warren shunt was performed. Platelet and white blood cell counts and liver function tests were performed before and after the operation. Growth was assessed using SD scores (z scores) for height, weight, and body mass index at the time of surgery and 1 year later. RESULTS: Postoperative recovery was uneventful and the leukocyte and platelet counts normalized. The shunt patency rate was 100 %. Two cases of shunt stenosis were treated successfully with percutaneous angioplasty. There was no postoperative mortality. During the follow-up period, from 1 to 7 years, all 16 children were asymptomatic, with improved quality of life, growth, and nutrition. No episodes of variceal bleeding, sepsis or encephalopathy occurred. CONCLUSION: Our results demonstrate that shunt surgery with partial resection of the spleen is effective and safe for pediatric patients with massive splenomegaly and severe hypersplenism secondary to EHPVO.

[Distal splenorenal shunt with partial spleen resection].

INTRODUCTION: Hypersplenism is a common complication of portal hypertension. Cytopenia in hypersplenism is predominantly caused by splenomegaly. Distal splenorenal shunt (Warren) with partial spleen resection is an original surgical technique that regulates cytopenia by reduction of the enlarged spleen. OBJECTIVE: The aim of our study was to present the advantages of distal splenorenal shunt (Warren) with partial spleen resection comparing morbidity and mortality in a group of patients treated by distal splenorenal shunt with partial spleen resection with a group of patients treated only by a distal splenorenal shunt. METHOD: From 1995 to 2003, 41 patients with portal hypertension were surgically treated due to hypersplenism and oesophageal varices. The first group consisted of 20 patients (11 male, mean age 42.3 years) who were treated by distal splenorenal shunt with partial spleen resection. The second group consisted of 21 patients (13 male, mean age 49.4 years) that were treated by distal splenorenal shunt only. All patients underwent endoscopy and assessment of oesophageal varices. The size of the spleen was evaluated by ultrasound, CT or by scintigraphy. Angiography was performed in all patients. The platelet and white blood cell count and haemoglobin level were registered. Postoperatively, we noted blood transfusion, complications and total hospital stay. Follow-up period was 12 months, with first checkup after one month. RESULTS: In the first group, only one patient had splenomegaly postoperatively (5%), while in the second group there were 13 patients with splenomegaly (68%). Before surgery, the mean platelet count in the first group was 51.6 +/- 18.3 x 10(9)/l, to 118.6 +/- 25.4 x 10(9)/l postoperatively. The mean platelet count in the second group was 67.6 +/- 22.8 x 10(9)/l, to 87.8 +/- 32.1 x 10(9)/l postoperatively. Concerning postoperative splenomegaly, statistically significant difference was noted between the first and the second group (p < 0.05). Comparing the postoperative platelet count between the first and second group, we found that there was a very significant statistical difference, too (p < 0.01). CONCLUSION: Distal splenorenal shunt (Warren) with partial spleen resection is a very reliable surgical technique in treatment of hypersplenism and decompression of oesophageal varices caused by portal hypertension and has advantage in treatment of hypersplenism over the distal splenorenal shunt method.

[Isolated aneurysm of the internal iliac artery and disorders of the pelvic organs functions].

BACKGROUND: Isolated aneurysm of internal iliac artery is very rare and often asymptomatic. Aneurysm itself can produce malfunction of the pelvic organs. Aneurysm rupture is followed by high mortality. CASE REPORT: A 76-year-old patient was admitted to the hospital with abdominal and left groin pain, disuric problems, obstipation and the signs of intestinal subocclusion. These problems persisted over the last few months. Digitorectal examination showed pulsatile tumor. Computed tomography and angiography revealed isolated aneurysm of the left internal iliac artery with a maximal diametar of 13.5 cm. The aneurysm was treated operatively using extraperitoneal approach in general anestesia. During the operation a Cell Saver was used. The left internal iliac artery was resected and ligated with end-aneurysmatic suture of its branches. CONCLUSION: Isolated aneurysm of the internal iliac artery should be considered by differential diagnosis in any case of the occurence of disorders of the pervic organs functions. Clinical findings, ultrasound examination, computed tomography and angiography are the diagnostic techniqnes of choice which can confirm the diagnosis. Surgical treatment with the use of retroperitoneal approach lead to complete recovery, so it could be considered the method of choice for patients with the condition permitting a radical surgical approach.

[Abdominal compartment syndrome caused by ruptured abdominal aortic aneurysm in vena cava].

BACKGROUND: Abdominal compartment syndrome (ACS) is a rapid increase in intra-abdominal pressure asssociated with multi-organs dysfunction. It is caused mostly by abdominal bleeding und massive volume compensation. CASE REPORT: We reported a 76-year-old patient admitted to the hospital with aortic abdominal aneurysm, 13.7 cm in diameter, ruptured in vena cava, which caused intraabdominal hypertension, the liver and kidney dysfunction, as well as circulation, respiration and metabolic disorders. Intraabdominal pressure was measured by bladder manometry. Central venous pressure and systemic arterial pressure were monitored continuously. Clinical signs were thrill and typical abdominal bruit. Aorto-caval fistula was diagnosed by the use of contrast computerized tomography. Caval endoaneurysmatic suture and aortobiiliac bypass with 18 x 9 mm Dacron prothesis were performed. Haemodynamic changes were mostly corrected during the surgery. The complete correction of haemodynamics, liver, kidney, respiration and metabolic changes was established in the next few weeks. CONCLUSION: The ACS was caused by rupture of abdominal aortic aneurysm in vena cava followed by edema of the abdominal organs, retroperitoneum, abdominal wall and ascites. Caval endoaneurysmatic suture and aortobiiliac bypass with 18 x 9 mm Dacron prothesis solved aortocaval fistula as well as all the organs and metabolic dysfunctions caused by ACS.

A well-differentiated liposarcoma co-existent with leiomyoma.

BACKGROUND: The coexistence of a well-differentiated liposarcoma (atypical lipomatous tumor) and benign smooth muscle component in a single soft tissue neoplasm is extremely rare. CASE REPORT: Histologic and immunohistochemical characteristics of tumor, which represented the recurrence of a retroperitoneal atypical lipoma in a 50-year-old female patient are presented in this paper. The tumor represented the recurrence of a retroperitoneal atypical lipoma. Lipomatous component consisted of a mixture of lipoma-like and sclerosing variant of well-differentiated liposarcoma. Characteristically, the heterologous smooth muscle differentiation was manifested as a macroscopically visible nodular growth in the form of a leiomyoma within the adipocytic component. It consisted of intersecting fascicles of spindle cells, which lacked mitoses and significant atypia, and were immunopositive for smooth muscle markers. CONCLUSION: This case of well-differentiated liposarcoma with benign smooth muscle is the first reported tumor of retroperitoneal localization. It is also the first one to exhibit the heterologous smooth muscle component as a distinct leiomyoma.

Single injection hepatic radionuclide angiography and hepatobiliary scintigraphy in the evaluation of liver transplant function.

BACKGROUND: The aim of the study was the evaluation of the perfusion, morphology and biliary three patency of liver transplants employing two radionuclide methods. MATERIAL AND METHODS: The study was performed on 10 controls and 10 patients after an orthotopic transplantation (up to two years). "First pass" dynamic acquisition was performed with a scintillation camera, after a bolus injection of 360 MBq 99mTc-diethyl- IDA, (60 frames/60s), continued by a 59 minute (1 frame/min) slower dynamic study. From the liver and kidney activity during the "first pass" study, the hepatic perfusion index (HPI) was calculated using slope-analysis. Hepatobiliary scintigrams obtained during second phase of the study were analyzed for morphology, and parenchymal and hepatobiliary TA curves were generated and analyzed according to the time to maximal activity (Tmax) and the time to half of maximum activity (T/2). RESULTS: In comparison to the controls (HPI, X = 0.64.5 +/- 0.05%) portal perfusion had slightly (X = 0.68 +/- 0.04%), but not significantly (p > 0.05) increased. In 3 patients, the biliary phase of hepatobiliary scintigraphy showed an increased accumulation of the radiopharmaceutical in the left (n = 1) or right (n = 2) hepatic duct. The uptake of the radiopharmaceutical (Tmax, X = 18.5 +/- 2.9 min) was slightly, but not significantly (p > 0.05) delayed in comparison to the controls (X = 14.2 +/- 3.4 min), while excretion was significantly (p < 0.05) prolonged (X = 59.5 +/- 12.1 min) (X = 34.2 +/- 4.1 min). Intrahepatic bile flow was insignificantly (p > 0.05) prolonged (X = 31.3 +/- 3.7 min) in comparison to the controls (X = 25.7 +/- 3.5 min) while extrahepatic bile flow was high, significantly (p < 0.01) prolonged (X = 89.0 +/- 14.3 min) than physiological one (X = 45.0 +/- 7.2 min). CONCLUSIONS: Radionuclide methods are noninvasive, sensitive and valuable in monitoring liver transplants.

Popliteal traumatic arteriovenous fistulas.

BACKGROUND: The purpose of this report is to analyze the clinical presentation, diagnosis, and outcome of surgical treatment in patients with popliteal arteriovenous fistulas (AVFs) in order to make trauma surgeons aware of the various issues patients with popliteal AVFs might present. METHODS: From 1991 to 2000, 49 patients were treated for traumatic AVF. Among these patients, seven suffered from popliteal AVF of various durations. The patients were men and ranged in age from 17 to 27 years, with a mean age of 22.4 years. The time from injury to admission to our institutions varied from 5 days to 2 years. A diagnosis of popliteal AVF was made after clinical examinations revealed thrill and bruit over the injury sites. The diagnosis was confirmed in four of the patients after they underwent angiography. Patients with long-standing popliteal AVF underwent cardiology examinations to check for signs of heart failure. All patients with popliteal AVF received surgical treatment. Five patients had major blood vessels reconstructed, one patient had a minor blood vessel ligated, and another patient had a minor blood vessel reconstructed. RESULTS: Five of the seven patients experienced no postoperative difficulties. No serious heart failure occurred; however, there were signs of cardiac overload in three of the five patients. The two remaining patients of the seven underwent leg amputations. However, one of the two patients had a gangrenous foot at admission to our institution, and vascular reconstruction on the other patient was unsuccessful. For all seven patients, the average hospital stay in the vascular surgery department was 16.2 days and the follow-up ranged from 2 to 44 months, with a mean of 21.5 months. CONCLUSION: Trauma of the popliteal space requires special attention, since blood vessel injuries in that zone might result in serious complications. Popliteal traumatic AVFs result in a high rate of leg amputation and long-standing fistulas produce cardiac overload. The presence of thrill and bruit over the injury site should alert the examiner to consider the existence of AVF. Angiography is a reliable diagnostic tool, and should be used in all vitally stable patients. Surgical or nonsurgical closure of AVF will prevent local and systemic complications that might be irreversible in long-standing fistulas.

[The von Hippel-Lindau syndrome with pheochromocytoma]. / Sindrom fon Hipel-Lindau s feohromocitomom.

The members of four generations of a family with Von Hippel-Lindau syndrome (VHL) have been followed by one of us (I.P.) for 30 years. The disease was proved in four members of this family, in three of them associated with pheochromocytoma. The grandmother (I-1) died at the age of 16 years two months after her first birth. The cause of death was not established. Her daughter (II-1) had 9 births with 5 children alive. Paresthesia and difficulties in walking followed by paraparesis and paraplegia were the first signs of the disease at the age of 58 years. The surgical treatment was performed because of an expansive lesion at the level of Th 3-4. Pathohistological examination was not done. It seems that a haemangioblastoma might be the cause of her disease. Diagnosis of pheochromocytoma was documented in a female patient (III-2) in 1972. Two years later she was successfully operated on. Pathohistological examination proved clinical diagnosis. She had also diabetes mellitus, cholelithiasis and cardiomyopathy. She died at the age of 56 years. A right-sided pheochromocytoma was diagnosed in a next female patient (III-4) at the age of 22 years. Her surgical treatment was successful. Retinal haemangioblastomatosis was established 7 years later in this patient. She was blind at the end of her life. Haemangioblastomatosis cerebelli was diagnosed soon, and she died at the age of 51 years. A 12- year old boy (IV-3) presented severe hypertension (36/24 kPa). Left-sided pheochromocytoma was removed in this patient one year later. Right-sided pheochromocytoma was operated on in the same patient at the age of 24 years. An elevated level of urinary dopamine was documented four years after the second operation. A malignant right-sided pheochromocytoma was operated on in the same patient 15 years later. At the same time metastases were found in the lower part of the right lung lobe. A 131-I-MIBG therapy could not be realized. He died at the age of 41. Pathohistological examinations proved the clinical diagnosis in this patient after all of three surgical treatments. MEN 2 syndrome was excluded by proper genetical analyses on the RET-protooncogen. Genetical analyses are in the course to identify the possible mutations of VHL-tumour-suppressor gene through the living members of the family. Multidisciplinary approach is mandatory in diagnosis, follow up and treatment of this specific group of patients. A collaboration among specialists of different fields of medicine (internal medicine, ophthalmology, neurology, radiology, urology, neurosurgery, biochemistry, pathology and genetics) is suggested.