Surgery in patients with congenital factor VII deficiency - a single center study.

INTRODUCTION: Congenital factor VII deficiency is a rare hemorrhagic disorder inherited in an autosomal recessive pattern. Surgical treatment with insufficient diathesis correction is burdened with high risk of bleeding complications. The aim of the study was evaluation of the surgical outcome in patients with congenital factor VII deficiency and assessment of the efficacy and safety of recombinant activated factor VII (rFVIIa) used for perioperative hemostatic coverage in our two schemas of substitutive therapy. MATERIAL AND METHODS: In the years 2002-2017 a total of 22 patients with congenital factor VII deficiency were subjected to surgery. Substitution therapy relied on rFVIIa used in two schemas. One involved 15 patients with factor VII activity of<10% of normal value who were injected rFVIIa at a dose of 30 µg/kg b.w. every12 hours on surgery day, 15 µg/kg b.w. every 12 hours on the first postoperative day and 15 µg/kg b.w. every 24 hours on the following days. The second schema involved 7 patients with factor VII activity of 10-25% of normal value who were given rFVIIa at a dose of 15 µg/kg b.w. every 12 hours on surgery day and the first postoperative day; then the same dose was administered every 24 hours on consecutive days. The treatment continued for 4-10 days. RESULTS: In the 22 patients a total of 26 surgeries were performed; 17 surgeries in 15 patients with factor VII<10% of normal and 9 in 7 patients with factor VII deficiency of 10-25% of normal. The surgeries included: 9 cholecystectomies (8 laparoscopic,1 open), 7 thyroidectomy procedures, 2 exploratory laparotomies, 1 left hemicolectomy, 1 total proctocolectomy, 3 inguinal hernia repairs and 3 excisions of varicose veins. One patient with factor VII activity of 9% required an additional dose of rFVIIa in the intraoperative period due to diathesis bleeding. Intraoperative hemostasis was normal for all other patients; no postoperative hemorrhagic complications were reported. In patients with FVII activity<10% average daily dose of rFVIIa was 31.3(range 20-56) µg/kg b.w., total daily dose 186(136-303) µg/kg b.w., total dose of rFVIIa-15.2(12-112) mg. In patients with FVII activity 10-25% the doses were 21.2(15-31), 117(46-271) µg/kg b.w. and 9.1(6-17) mg respectively. CONCLUSIONS: Surgery in patients with congenital factor VII deficiency can be safely and efficiently performed with rFVIIa as substitutive treatment securing perioperative hemostasis.

Sclerotherapy of esophageal varices in hemophilia patients with liver cirrhosis - a prospective, controlled clinical study.

INTRODUCTION: Bleeding from esophageal varices is a serious clinical condition in hemophilia patients due to congenital deficiency or lack of clotting factors VIII (in hemophilia A) and IX (in hemophilia B), decreased clotting factor II, VII, IX, X synthesis in the course of chronic liver disease and hipersplenic thrombocytopenia. The aim of this study was to assess the efficacy and safety of endoscopic sclerotherapy in acute esophageal variceal bleeding and in secondary prophylaxis of hemorrhage. The aim was also to investigate the optimal activity of deficiency factors VIII or IX and duration of replacement therapy required to ensure proper hemostasis after sclerotherapy procedures. MATERIAL AND METHODS: 22 hemophilia patients (A-19, B-4) with coexistent liver cirrhosis and active esophageal variceal bleeding treated with endoscopic sclerotherapy were subjected to prospective analysis. The patients who survived were qualified to repeated sclerotherapy procedures every 3 weeks within secondary prophylaxis of bleeding (investigated group). A 3-day substitution therapy enhanced the infusion of the deficient or lacking factor in doses allowing to reach 80-100% of normal value activity of factor VIII on the 1st day and 60-80% in the next two days. The desired activity of factor IX was 60- 80% and 40-60% respectively. The control group consisted of 20 non-hemophiliac patients with liver cirrhosis comparable in terms of age, sex, stage of advancement of liver cirrhosis, who underwent the same medical proceedings as the investigated group. RESULTS: Active esophageal bleeding was stopped in 21 of 22 (95%) hemophilia patients. Complications were observed in 3 patients; 2 patients died. The rate of hemostasis, complications and deaths in the control group were comparable and no statistical differences were found. In hemophilia patients subjected to secondary prophylaxis of hemorrhage, in 18 of 20 (80%), complete eradication of esophageal varices was achieved after 4 to 7 sclerotherapy procedures in 1 patient (average 5.4). Recurrent bleeding was observed in 15% of patients, complication in 20%; 1 patient died. Time lapse from bleeding to eradication was 12-21 weeks (average 15.2). In the control group the rate of variceal eradication, complication and deaths was comparable and no statistical differences were found. The usage of factor VIII concentrates was as follows: in hemophilia A, in a severe form - 80.9 U/kg b.w./day, in hemophilia A in a severe form with an inhibitor <5 BU - 95.2 U/kg b.w./day, in mild form - 64.2 U/kg b.w./day and in severe hemophilia B - 91.6 U/kg b.w./day. CONCLUSIONS: Sclerotherapy is an effective method in the management of esophageal variceal bleeding in hemophilia patients. It is also effective for total eradication of varices when applied as a secondary prophylaxis of hemorrhage. In our opinion, a 3-day replacement therapy at the applied doses is sufficient to ensure hemostasis and avoid bleeding complications.

Splenic abcesses as infectious complication following implantation of left ventricular asssist device - case report.

Left ventricular assist device (LVAD) is one of the modern management therapies in patients with advanced heart failure, and it serves as a bridge to heart transplantation or even as destination therapy. However, it is burdened with a high risk of thromboembolic, hemorrhagic, and infectious complications despite prophylactic management. Splenic abscesses, as septic complications following implantation of mechanical ventricular support, have not yet been described in the literature. We report of a patient with severe left ventricular insufficiency (NYHA II/III), pulmonary hypertension, and arrhythmia who underwent implantation of the Heart Ware® pump for left ventricular support with simultaneous tricuspidvalvoplasty, as a bridge therapy to heart transplantation. During two years after LVAD implantation, the patient had three MRSA skin infections, localized at the exit site of the drive-line connecting the artificial ventricle with external unit, that were complicated by sepsis and treated with broad-spectrum antibiotics. A few months later, abdominal CT revealed two abscesses in the spleen, and the patient was qualified for splenectomy. Open splenectomy was performed under full-dose anticoagulant therapy with continuous intravenous infusions of unfractionated heparin (UFH). The intra- and postoperative course was uneventful. UFH therapy was continued for 6 days, and oral anticoagulation was re-administered on day 4 after surgery. The patient was discharged on day 7 after surgery with primary healed wound. Open splenectomy, performed with full-dose anticoagulant therapy, proved to be an effective and definitive method of treatment without any complications.

[Acute mastoiditis with retroauricular abscess - a report of two cases]. / Ostre zapalenie wyrostka sutkowego z ropniem zamalzowinowym ­ opis dwóch przypadków.

Otogenic complications may occur as a result of both acute and chronic otitis media. The purpose of the diagnostic process and treatment of patients with otogenic complications is to identify and eliminate focal points of the infection. Due to general application of antibiotics, these complications are rarely observed. Retroauricular abscess may be the first symptom associated with the development of acute mastoiditis in patients with communication disturbances. Intravenous antibiotics and surgery have been successfully used for treatment in the two described cases. First case: 9 years old girl suffering from hydrocephalus, mental retardation. Retroauricular abscess occured as a result of the left ear acute otitis media. Second case: 17 years old girl with multiple congenital defects, mental retardation. Four surgery because of epidural abscess, meningitis, external auditory canal atresia, granulation and cholesteatoma during two years. Retroauricular abscess occured in the course of the right ear chronic otitis media.

[Granuloma on tympanic membrane - a case report]. / Ziarniniak blony bebenkowej ­ opis przypadku.

Granuloma on tympanic membrane is a rare complication after ventilation drainage of tympanic cavity. The paper presents a case of a child for chronic otitis media with effusion with ventilation drainage of the right tympanic cavity, retained for 24 months, and granuloma on tympanic membrane growing all over the ventilation drain, imitating acute inflammation, causing conductive hearing loss. The patient was treated with the vent tube removal together with granulation inflammatory. Spontaneous healing of the perforation of the tympanic membrane and improve hearing were obtained after a four-month follow-up.

Prevention of bleeding and hemorrhagic complications in surgical patients with inherited factor VII deficiency.

Inherited factor VII (FVII) deficiency is a rare autosomal recessive hemorrhagic disorder. The major clinical symptoms include: bleeding from the oral cavity, epistaxis, menorrhagia, spontaneous hemarthros, bleeding to the gastrointestinal tract and central nervous system, and perioperative bleeding. The aim of this study was to present our experience in preventing bleeding and hemorrhagic disorders in surgical patients with inherited FVII deficiency by using recombinant activated FVIIa (rFVIIa), and with prothrombin complex concentrates (PCCs). In 2002-2011, 17 patients with inherited FVII deficiency underwent surgery. Thirteen patients had isolated FVII deficiency below 10%, and four patients 10-25. To prevent bleeding and hemorrhagic complications, we administered small single doses of rFVIIa (Novo-Seven) at 12-h intervals to 15 patients on surgery day and on day 1 following surgery, then every 24 h; PCCs were administered (Prothromplex, Beriplex) to two patients. No symptoms of bleeding, hemorrhagic or thromboembolic complications were observed in the perioperative and 1-month observation period in surgical patients treated with rFVIIa. One patient treated with PCC (Prothromplex) developed distal deep vein thrombosis on postoperative day 7. The results suggest that small, single, every 12-h doses of rFVIIa (NovoSeven) and in next days after surgery one time every 24 h are well tolerated and effective for prevention of thromboembolic, bleeding and hemorrhagic complications in FVII-deficient patients. Antithrombotic prophylaxis with low-molecular-weight heparin should be applied in patients using PCCs.

Radiofrequency ablation in the management of Barrett's esophagus - preliminary own experience.

INTRODUCTION: Barrett's esophagus develops as a result of chronic injury of esophagus epithelium from gastroesophageal reflux disease. It is defined when metaplastic columnar epithelium replaces the stratified squamous epithelium which normally lies in the distal esophagus. The condition represents a risk factor for esophageal adenocarcinoma. The aim of the radiofrequency ablation (RFA) method is to destroy metaplastic epithelium with radiofrequency electric current and to stimulate reappearance of the flat multilayer epithelium in the distal esophagus. AIM: To evaluate the efficiency and safety of the RFA technique, newly introduced in Poland, in the management of Barrett's esophagus. MATERIAL AND METHODS: Twelve patients were treated with the RFA method. Patients with Barrett's esophagus confirmed in the histopathological report were qualified for treatment. Two RFA techniques were applied using a BARRX(®) device: circular based on the balloon HALO(360) system or focal based on the HALO(90) system mounted to the endoscopic ending. The procedures were performed at 2-month intervals. The macroscopic and microscopic effects of RFA therapy, the patients' treatment tolerance as well as potential complications were evaluated. RESULTS: In the group of 12 patients subjected to RFA therapy, 10 completed the therapeutic cycle. A total of 37 procedures were performed: 5 HALO(360) and 32 HALO(90). In all patients eradication of the abnormal metaplastic esophageal epithelium was achieved, as confirmed in both endoscopic and histopathological evaluation. In 2 patients with ongoing therapy progressive eradication of metaplastic epithelium was observed. No significant RFA-related complications were reported. CONCLUSIONS: Based on our preliminary results we consider this method to be promising, free of significant complications and well tolerated by patients. In most patients it results in successful eradication of metaplastic epithelium in the distal esophagus.

[Excision of the rectum due to carcinoma in patient with hereditary hemorrhagic teleangiectasia]. / Wyciecie odbytnicy z powodu raka u chorej z wrodzona naczyniakowatoscia krwotoczna.

UNLABELLED: Hereditary hemorrhagic telangiectasia (HHT) also known as Rendu-Osler-Weber disease, is a rare, autosomal dominant disorder of the fibrovascular tissue. Clinically, it is characterized by the triad of symptoms of mucocutaneous telangiectasias and arteriovenous malformations of visceral organs, recurrent hemorrhages from vascular changes at different localization, and familial occurrence. A coexistence of HHT syndrome and malignant neoplasms in various sites, including large bowel, is suggested. The authors describe the case of 67-year old woman with HHT with massive bleeding from digestive tract in history and persistent chronic sideropenic anemia. In upper gastrointestinal endoscopy performed in regional hospital multiple telangiectasias in the stomach were found. These were diagnosed as the possible bleeding source therefore colonoscopy was not performed. After twelve months in diagnostic colonoscopy due to chronic anemia carried out in our Institute, rectal carcinoma was found. The patient were subjected to surgery--lower anterior excision of the rectum. Intra operatively and in the postoperative period complications were not observed. CONCLUSION: In every case of bleeding from digestive tract and also in chronic anemia in patients suffering from Rendu-Osler-Weber disease, beside the endoscopy of esophagus, stomach and duodenum, full visualization of the large bowel is necessary to exclude malignant neoplasm. In the presented case no colonoscopy performed in the regional hospital delayed the carcinoma diagnosis by twelve months.

[Nonoperative management of spontaneous splenic rupture in infectious mononucleosis]. / Leczenie nieoperacyjne samoistnego pekniecia sledziony w przebiegu mononukleozy zakaznej.

UNLABELLED: Spontaneous splenic rupture is a rare complication of infectious mononucleosis observed in 0.1-0.5% of patients with this condition. Mandatory mode of management in hemodynamically stable patients is nonoperative treatment. We report the case of a 19-year old man with splenic rupture, during the course of serological and hematological confirmed infectious mononucleosis, with no history of trauma. Parenchymal and subcapsular splenic hematomas and presence of blood in vesico-rectal recess was demonstrated. Circulatory and respiratory findings and blood cell count were stable. Nonoperative management was instituted which comprised monitoring of valid vital signs, serial USG and tomography scans and vital activity limitation. Imaging radiological investigations demonstrated disappearance of observed abnormalities on post admission day 20. The patient was discharged from the hospital in good general condition. CONCLUSION: Nonoperative management can be a safe alternative to splenectomy in hemodynamically stable patient with spontaneous rupture of the spleen.

[Self-fixating Progrip implant used in the laparoscopic totally extraperitoneal technique for inguinal hernia repair]. / Zastosowanie samomocujacego sie implantu Progrip w technice calkowicie pozaotrzewnowej w operacji naprawczej przepukliny pachwinowej.

UNLABELLED: Modern tension-free techniques have become standard for inguinal hernia repair. The most highly regarded minimally invasive technique is laparoscopic totally extraperitoneal hernioplasty (TEP) which results in shorter recovery, earlier return to normal activities and better quality of life as compared to open repair techniques. The aim of the study was to evaluate the effects of inguinal hernia management in a von Willebrand (type 2a) patient. For the first time in Poland a partially absorbant mesh with a self-fixating system was implanted in a completely extraperitoneal approach using laparoscopy (TEP technique). MATERIAL AND METHODS: The procedure used was typical for the method; two 5 mm ports and a 10 mm port for the camera were applied below the umbilical line. A 15 x 9 cm synthetic mesh was inserted through the 10 mm port and positioned preperitonealy to close the hernia ring. Substitution therapy was administered for 7 days to control factor VIII activity in plasma at a level of approximately 100% for the first 3 days and 70% until healing of the postoperative wound. RESULTS: Surgery was uneventful with no postoperative complications. The patient was discharged from hospital within 7 days of suture removal. No hernia recurrence nor persisting pain were reported at follow-up examination 6 months after the procedure. CONCLUSIONS: Synthetic mesh with a self-fixating system used in the TEP technique for inguinal hernia repair has been proved safe and fully effective.

Randomized clinical trial comparing inguinal hernia repair with Lichtenstein technique using non-absorbable or partially absorbable mesh. Preliminary report.

INTRODUCTION: The Lichtenstein technique is currently considered the "gold standard" of open, anterior inguinal hernia repair. It is not free, however, of adverse effects, which may be caused by the implemented synthetic material. AIM: Determination the influence of the mesh employed on treatment results including immediate complications, return to everyday activities, chronic pain occurrence and hernia recurrence. MATERIAL AND METHODS: Tension-free hernia repair using the Lichtenstein technique was performed in all the 59 patients randomized to trial groups. Group P with heavyweight polypropylene mesh contained 34 patients; group V with lightweight, partially absorbable mesh (polypropylene/polyglactin 910) consisted of 25 people. Controlled, scheduled follow-up appointments took place after the 7(th) day and the 3(rd) and 6(th) month. Patients were clinically assessed and pain intensity was determined on an analogue-visual scale. RESULTS: No statistically significant influence of the type of mesh on the risk of early complications, severe pain intensity, the length of hospital stay, time of recovery, or patients' satisfaction with treatment was observed. After 6 months also no statistically significant differences were observed between groups with regard to recurrence rate (P 3.4% vs. V 4.0%), chronic pain (P 5.9% vs. V 4.0%) and ailments such as "foreign body presence" (V vs. P, OR = 0.30, 95% CI 0.077-1.219, p = 0.093) incidence, although their probability was 70% lower for V mesh. CONCLUSIONS: The preliminary results confirm the effectiveness of the Lichtenstein technique for hernia repair with both types of meshes. It appears that use of a partially absorbable mesh is connected with lower risk of postoperative complications, but the final results require multicentre trials in a larger series of patients.

[Laparoscopic cholecystectomy with single incision laparoscopic surgery]. / Cholecystektomia laparoskopowa wykonywana metoda jednego ciecia.

UNLABELLED: For the last seventeen years laparoscopic cholecystectomy has remained the gold standard procedure for gallbladder surgery The single incision laparoscopic surgery (SILS) approach is a step toward less invasive surgery and the intraumbilical access improves the cosmetic effect (no scar surgery). The aim of the study was to present our preliminary experience in laparoscopic cholecystectomy through single intraumbilical incision. MATERIAL AND METHODS: Three patients with gallbladder symptoms (2 female, 1 male) underwent surgery through single intraumbilical incision. In two cases the procedure was performed with three 5 mm Dexide port approach and in one, with a flexible rubber/gel SILSTM-Port (Covidien, USA) access. A simple 5 mm optics was employed. In two procedures the following disposable instruments were used: roticulator Endo dissect, roticulator Endo grasp, mini Sears scissors as well as standard hook and clips while for the third procedure, a conventional multi-use grasp and roticular Endo dissect. The gallbladder was elevated to the integuments by two sutures to expose Calot's triangle and bladder site. Following exposure, the bile duct and the vesical artery were clipsed and cut routinely to remove the gallbladder in a plastic bag through the bell-button. After procedures no drains were left in the peritoneal cavities. RESULTS: In all three cases the laparoscopic procedure was performed. The median operative time was 88 min. In one female patient, bleeding into the peritoneal cavity was reported and called for laparotomy within a few hours of laparoscopic procedure. Intraoperatively, the branch of vesical artery was sutured to arrest bleeding. The median hospital stay following procedure was 3, 8 and 5 days respectively. After the procedure, the last female patient was observed to have pharyngitis and enlarged jugular lymph nodes that abated after antibiotic therapy. CONCLUSIONS: Single-incision laparoscopic cholecystectomy should be performed by experienced laparoscopic surgeons. For adequate evaluation of the efficacy and safety of this procedure it is necessary to perform further clinical studies on a larger number of patients.

[Emergency sclerotherapy in esophageal varices bleeding: prospective study in unselected patients with portal hypertension]. / Dorazna skleroterapia w leczeniu krwotoków z iylaków przetyku: badanie prospektywne w niewyselekcjonowanej grupie chorych z nadcisnieniem wrotnym.

UNLABELLED: Bleeding from esophageal varices is a serious life-threatening complication of portal hypertension. The aim of the study was to evaluate the efficacy and safety of endoscopic sclerotherapy in the emergency management of esophageal variceal bleeding and analysis of recurrent bleedings, complications and mortality within 6-week observation period. MATERIAL AND METHODS: Prospective study involved 128 unselected patients with portal hypertension, including 116 with liver cirrhosis and 12 with portal vein thrombosis, with active bleeding from esophageal varices, in age 20 to 83, mean 50.9. Twenty one patients were classified as Child-Pugh grade A, 43 as grade B and 64 as grade C. After confirmation of diagnosis by endoscopy, emergency scierotherapy procedures were performed according to strictly defined protocol. RESULTS: Active esophageal variceal bleeding was ceased in 123 out of 128 (96.1%) patients. Two patients with persisting bleeding were subjected to gastro-esophageal devascularisation and splenectomy. Three patients died of bleeding and liver insufficiency. Recurrent bleedings in 19 (14.8%) patients were successfully treated with repeated sclerotherapy procedures. Complications were found in 17 (13.3%) patients. During a 6-week observation period, 14 patients died, including 12 in Child-Pugh grade C. CONCLUSIONS: Endoscopic sclerotherapy was proved to be an effective method of esophageal variceal bleeding management which allows to ceases hemorrhage in over 95% patients and reduced mortality rate to 11%. An important factor for achieving good results is experience of treating center.

Angiodysplasia as a cause of recurrent bleeding from the small bowel in patients with von Willebrand disease. Report of 4 patients.

Angiodysplasia, characterized by the presence of malformed vessels in the submucosa of the gastrointestinal tract, may be a cause of recurrent bleeding. Bleeding angiodysplasia can be associated with von Willebrand disease (vWD) and this coincidence is probably the consequence of the lack of high molecular weight molecules of von Willebrand factor in the plasma. We report four patients with unexplained repeated massive intestinal bleeding, recurrent melena and iron deficiency anemia, which required numerous blood transfusions. All patients were adults (average age 68 years). Three patients have congenital von Willebrand disease (type 1, 2A and 3) and one idiopathic acquired von Willenbrand syndrome. Correct diagnosis was made 2-5 years after the onset of the symptoms and was confirmed by histopathological examination of surgically resected small bowel, where vascular lesions were located. Elderly patients with recurrent gastrointestinal bleeding and unexplained iron deficiency anemia should be diagnosed for angiodysplasia and vWD.