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J Siman

Publications and source records attributed to J Siman.

At least 19 recordsLinked to original sources

Separation of Siamese twins in Bratislava.

At the pediatric surgical clinic in Bratislava, in the year 2000, we successfully carried out the separation of Siamese twins, which we classified as "ischiopagus tetrapus"+. The pelvic ring, the gastrointestinal tract and the urogenital system were all malformed. There was a common terminal ileum and only a short segment of the large intestine with a blind end. This was a case of non-developed anal segment and rectum. Each of the twins had two kidneys and two commonly shared urinary bladders. There were two uteruses, which were both bicornuate in nature with a fallopian tube arising from each of the horns as well as an ovary. The orifices of both urinary bladders and those of the two uteruses joined into a common urogenital sinus. The external ostia of this sinus represented a bizarre fissure with a single skin fold similar to the labium majus, located dorsally at the point of fusion of the pelvic structures. The act of separation was performed in two stages. Separation of the gastrointestinal tract was urgent due to the severe ileus caused by aplasia of the anus and the rectum. This first stage procedure was performed on the third day of admission (24.2.2000) after the babies had spent a month in other health institutions. The definitive separation was carried out a month after the first procedure (28.3.2000) following the optimal stabilization of the functions of the gastrointestinal tract. The uropoietic and genital systems were separated. A new pelvic ring and a pelvic floor were formed. The anterior abdominal wall was reconstructed and plastic correction of the skin in the gluteal and perineal regions was performed. The colostomies are functioning well and the twins pass well-formed stools. Lucia has long dry intervals between mictions. Andrea has shorter dry intervals. The girls have recently celebrated their third birthday. Their mental development is excellent; they speak Slovak and Hungarian languages. They have a well-developed locomotive apparatus, without any movement impairments. (Tab. 1, Fig. 16, Ref. 10.)

Female↗

[Exchange amnioinfusion in conceptus with laparoschisis (first experience)].

OBJECTIVE: During amnioinfusion exchange (AE) a certain amount of amniotic fluid is repeatedly extracted and the same amount of physiological solution is consequently instilled into the amniotic fetal cavity. The aim of this procedure is to dilute the amniotic fluid that surrounds the eviscerated organs of fetuses with laparoschisis so as to avoid the genesis of fibrous coating on these organs. DESIGN: Prospective study. SETTING: Gynekologicko-pôrodnícka klinika SZU, FNsP akad. L. Dérera, Bratislava, Slovakia. METHODS: We have executed AE in five fetuses with laparoschisis since June 2002. Two patients underwent the treatment 2 times during the 32nd and 36th weeks of gestation. Two other patients were treated once during the 32nd week and one patient once during the 36th week. Under ultrasound control we used a spinal needle to extract 120-180 ml of dense, cloudy amniotic fluid. Consequently, we instilled the same amount of physiological solution warmed up to the temperature of 37 degrees C into the amniotic cavity through antibacterial filter. The fetuses were monitored cardiotocographically and with the help of ultrasound flowmetry in umbilical vessels, before and after the treatment. RESULTS: The AE were successful and without complications in all five cases. All patients delivered via elective caesarean section during the 36th - 37th gestation week. One patient delivered 24 hours after second AE due to the danger of intrauterine fetal hypoxia that was verified cardiotocographically. The other patients delivered 1-4 weeks after AE. CONCLUSION: The significance of AE lies primarily in the reduction of the occurrence of fibrous coating on eviscerated organs. It enables postnatal primary surgical closure of the defect in the front abdominal wall, an earlier onset of intestine peristalsis, transition from parenteral to peroral nutrition and shorter hospitalization.

Amniotic Fluid↗

Improved patient survival following surgery for coarctation of the aorta.

BACKGROUND: We conducted a retrospective review of children undergoing surgery for coarctation of the aorta in our institution over the last ten years with the aim of evaluating overall patient survival as well as detecting factors affecting it. We tried to identify the risk factors for mortality. METHODS AND DATA: Between January 1992 and December 2001, 201 patients with aortic coarctation were operated on at the Department of Cardiac Surgery of the Children's University Hospital, Bratislava. The three classes of aortic coarctation were represented: isolated coarctation, coarctation with ventricular septal defect (VSD) and coarctation with complex cardiac anomalies. Patients' preoperative, operative and immediate postoperative medical records were carefully studied with special attention paid to the type of lesion, patients' preoperative state, type of surgical technique employed, as well as the period of operation. For comparison, two equal time periods of follow-up were reviewed--1992 to 1996 and 1997 to 2001. The overall postoperative conditions of patients were also regularly monitored. Patient data were statistically analyzed using the JMP program version 4.04. RESULTS: An overall survival of 90% was recorded over the period of follow-up, ranging between one and ten years. A further break down showed a statistically significant difference between the various types of aortic coarctation, p=0.0001. Patients with simple or isolated coarctation had a survival rate of 100%, those with ventricular septal defect (VSD) in addition to coarctation had a survival rate of 80% while patients with associated complex cardiac anomalies had a survival rate of 65%. An improvement on overall patient survival was recorded in the period between 1997 and 2001--96% as against 86% for the period between 1992 and 1996. On univariate statistical analysis, the following variables were identified as significant risk factors for death: 1) Complex cardiac anomalies (p<0.0001), 2) Age at operation less than one month (p<0.0001) and 3) Treatment prior to the year 1997 (p=0.02). CONCLUSION: A considerable improvement on patient survival following surgery for coarctation of the aorta was recorded over the last five years. This could be attributed to new measures in preoperative, operative and postoperative care for patients with aortic coarctation. (Tab. 4, Fig. 5, Ref. 8.).

Adolescent↗

Recoarctation and patients' freedom from re-intervention--a study of patients undergoing surgery for coarctation of the aorta at the Department of Cardiac Surgery of the Children's University Hospital, Bratislava.

BACKGROUND: Many children and young adults have undergone surgery for coarctation of the aorta. Individual surgical techniques employed in the treatment of patients and their effects on incidence of recoarctation were reviewed. METHODS AND DATA: Over the last ten years, a total of 201 cases of aortic coarctation were surgically treated at our department. The three forms of coarctation of the aorta recognized by the Society for Cardiothoracic Surgeons namely: isolated coarctation, coarctation with ventricular septal defects and coarctation with complex cardiac anomalies, were represented. RESULTS: 19 cases of recoarctation were recorded over the period of follow-up, representing 10% of all operated patients. On univariate analysis, the risk of recoarctation was closely linked with the following variables: use of resection and end-to-end anastomosis (p=0.01), age at operation less than one month (p=0.0002) and weight at operation less than 3 kg (p=0.01). The risk of recoarctation was found to be highest when resection and end-to-end anastomosis was employed in neonates (p<0.0001). Most cases of recoarctation as shown by the Kaplan-Meier plot occurred within the first year after surgery. CONCLUSION: The use of simple resection and end-to-end anastomosis in neonates is associated with a high risk of recoarctation. Hence, our preferred surgical technique in neonates is the extended resection and end-to-end anastomosis, which is associated with a considerably lower risk of recoarctation in this age group. (Tab. 4, Fig. 5, Re. 7)

Aortic Coarctation↗

Functional outcome of surgery for coarctation of the aorta.

AIM OF STUDY: Coarctation of the aorta (CoA) accounts for about 8% of all congenital heart diseases. This represents about 30 new cases of coarctation every year in Slovakia, of which more than half will require surgical treatment. Over the past years, many children with this diagnosis have been successfully operated on at the Department of Cardiac Surgery of the Children's University Hospital, Bratislava. Thus, the need for a comprehensive follow-up and analysis of the postoperative well being of these young patients arises. Our study is therefore aimed at: 1) identifying factors affecting the incidence and persistence of postoperative systemic hypertension, as well as the need for heart failure and hypertension treatment, 2) assessing patients' psychomotor development following surgery for coarctation of the aorta. METHODS AND DATA: Between January 1992 and December 2001, a total of 201 patients with aortic co-arctation were operated on at our institution. The three classes of aortic coarctation namely: isolated coarctation, coarctation with ventricular septal defect and coarctation with complex cardiac anomalies were represented. Patients' medical records were retrospectively reviewed, with attention paid to such variables as the type of lesion, gradient across the site of coarctation, type of surgical technique employed and surgery-related complications. Subsequently, these patients were followed for a time period ranging between six months and ten years during which their psychomotor development and overall clinical state were evaluated. RESULTS: Of the 201 operated patients, 64 (33%) had early postoperative hypertension, so-called paradoxical hypertension. There was a significant correlation between the incidence of early postoperative hypertension and patients' age at operation (p < 0.0001). Age at operation was also a significant risk factor for late hypertension (p = 0.005). In both cases we noticed a higher incidence of high blood pressure in patients operated on after the age of six years. The need for antihypertensive treatment of patients with early postoperative hypertension decreases with a younger age at operation. At five years of follow-up, the need for antihypertensive treatment was 15%. Clinical psychological evaluation of 64 patients showed a normal distribution of patients' intelligence quotients. No surgery-related variable correlated with the incidence of delayed mental development. There was, however, a certain correlation between the presence of complex anomalies and low verbal IQ in examined patients (p = 0.04) CONCLUSIONS: Early surgical treatment of aortic coarctation reduces the likelihood of early, as well as late postoperative hypertension. The preferred protocol in our institution is early surgical treatment of patients at about the age of two years. The need for antihypertensive treatment of patients at five years of follow-up is 15%. Patients' psychomotor development following surgery for aortic coarctation is not affected by type of surgical procedure. On the whole, we can conclude that patients' psychomo-whole, we can conclude that patients' psychomotor development does not differ from the rest of population. There is however, a certain correlation between complex cardiac anomalies and a tal, Bratislava delay in some components of patients' psychomotor development. (Tab. 3, Fig. 4, Ref. 17.)

Adolescent↗

[Surgical treatment of children with necrotizing enterocolitis].

Necrotizing enterocolitis is an acute neonatal disease. It affects in particular premature neonates with a birth weight lower than 1500 g. Despite extensive research the etiology of the disease remains obscure. The majority of authors assume multifactorial causes. Research workers try to detect various laboratory and clinical factors which could serve as criteria for surgical intervention. The sensitivity and specificity of these tests and laboratory examinations detect the disease only in an advanced stage and in the stage of perforation of the gastrointestinal tract (GIT). The main surgical approach to the treatment of this disease remains laparotomy and resection of the necrotic portion of the gut with enterostomy. In a limited number of children resection of a portion of the necrotic gut with primary anastomosis is possible. In some very serious conditions and in infants with a birth weight below 1000 g peritoneal drainage and subsequent "second look" surgery should be sufficient. It is a disease with calls for a maximum individual approach and there are no accurate defined instructions for surgical treatment. The surgeon's experience and the standard of preoperative and postoperative care are decisive.

Enterocolitis, Necrotizing↗

[Maffucci's syndrome--case report of a 5-year-old female patient].

Maffucci's syndrome is a congenital non-hereditary syndrome characterized by a combination of dyschondroplasia (enchondromatosis) and haemangiomatosis. It is a rare disease. During the last 140 years only 200 cases were described [5]. 109 cases are described in the literature by other authors [21]. This disease is associated with a high risk of development of neoplastic processes, in particular sarcomatous transformation of enchondromatoses as well as other mesodermal and non-mesodermal malignities [13]. Based on the incidence of spinocellular haemangioendothelioma with other congenital syndromes, the presence at a young age of patients and the multicentric incidence support the assumption that spinocellular haemangioendothelioma may be the manifestation of genetically based mesodermal disease which may be associated with the picture of Maffucci's syndrome [6]. In their case-history the authors present a 5-year-old patient. They describe the clinical course and findings taking into account possible manifestations and risks associated with the disease. With regard to the low incidence of the disease and its interdisciplinary character the authors pen the question of possible causal, symptomatic or palliative treatment.

Child, Preschool↗

[Diastematomyelia and the tethered spinal cord syndrome. Case report].

The authors present a patient with diastematomyelia and associated congenital malformations of the spinal cord. They caused tethering of the spinal cord. Patient had cutaneous marks of malformation from birth. The congenital malformation was operated at the age 5 years. MRI and surgical findings confirm the existence of diastematomyelia together with thick tethered filum terminale and lipoma of filum terminale. On surgery the bony septum was removed, the filum terminale was resected and the lipoma was excised. Each of the anomalies caused the tethering of the spinal cord and restricted its physiological ascent. Only careful surgical revision with subsequent removal of all causes of the tethering of the spinal cord produced good result. Diastematomyelia is a congenital malformation with some morphological variations and coexistence of other malformations. The presented case is a typical example of malformations associated with diastematomyelia.

Cauda Equina↗

[Supracondylar fractures of the humerus and disorders of circulation].

Supracondylar fractures (SF) of the humerus are the third most common type of fractures in childhood. The highest rate of pulselessness fracture of the distal humerus is the extension-type. The aim of our treatment is to minimize potential complications. Our management of pulselessness supracondylar fracture is more aggressive compared with other dpts. 223 (100%) patients with SF were treated at the dpt. of Paediatric Surgery, University Children's Hospital in Bratislava, in the 5-year period of (1995-1999). 11 (4.93%) patients remained pulseless after reposition of bone fragments. These patients underwent surgical revision of the brachial artery. Spasms of the brachial artery (BA) occurred in six patients, a lesion of BA in three patients and thrombosis of BA in two patients. We performed revision of the brachial artery by a Fogarthy catheter in six patients--in four cases with thrombectomy. In two cases we released the vessel from bone fragments. In two patients was replaced the injured segment of the brachial artery by a venous allotransplant. Peroperative angiography was performed in six patients. We reoperated three patients with early postoperative complications. We did not record any case of Volkmann's contracture.

Brachial Artery↗

[Treatment of blunt injuries of the spleen in children].

The authors report study of patients with blunt splenic trauma treated at Paediatric Surgery Department in years 1991-1999. During 9 years period 50 patients (36 males, 14 females) with demonstrated rupture of the spleen were analyzed. 46 patients (92%) were treated conservatively (group A). Complications appeared in 7 patients (15.22%), rebleeding in two patients, arterio-venous fistula in early posttraumatical period in one patient and during control in one patient, fluidothorax in two patients and posttraumatic pseudocyst of the spleen in one patient. Four patients (8%) were operated (group B). Two of them submitted splenectomy, one patient was treated by splenorrhaphy and one patient by partial splenectomy. Period of the hospitalization in average lasted in group A 12,67 days, in group B 15 days. With regard to the immunologic functions of the spleen especially in childhood authors prefer conservative treatment, in case that laparotomy is inevitable authors try to preserve the spleen.

Child↗

Surgical treatment of transposition of the great arteries.

BACKGROUND: With regard to risk of the failure of systemic right ventricle after physiological correction of transposition of great arteries, anatomic repair is a current method of choice. OBJECTIVE OF STUDY: Analysis of results of surgical correction of transposition of great arteries performed between 1992 and October 1998. METHOD: A total of 111 patients were operated on for transposition of the great arteries. In the 1st group of patients (n = 21, mean age was 135 +/- 55 days), physiological correction according to Senning was performed. Patients of the 2nd group (n = 90, mean age was 15.4 +/- 21.6 days) underwent anatomic repair. RESULTS: Early mortality was 6% (7 patients). Mean follow-up is 2.95 years (1.9 SD) ranging from 0.2 years to 6.1 years. Actuarial 1-month survival in the whole cohort (n = 111) is 94%, and it remains unchanged at 1, 2, 3, 4, 5, and 6 years of follow-up. Patients, who underwent surgery after 1997, show significantly better survival compared to those operated before 1997 (p = 0.0997). Thus, a date of operation (before 1997) is the only significant risk factor for death. Survival in patients operated after 1997 (n = 40) is 98%. All patients belonging to the 2nd group are in functional group NYHA 1. CONCLUSION: Anatomic repair of transposition of the great arteries is a method of choice for treatment of this congenital heart defect. Left ventricle becomes systemic ventricle, which is essential in view of long-term performance. Psychomotor development of children, who underwent ASO, is comparable with that of healthy population. (Tab. 3, Fig. 3, Ref. 18.)

Follow-Up Studies↗

[Subacute appendicitis in children].

Over the period of 4 years from 1993 to 1996 the authors recorded a 6% incidence of patients with periappendicular mass (PM) in a group of 786 patients with acute appendicitis. The aim of the study is to compare the two methods in the management of patients (operation-A, conservative treatment-B) by applying the same criteria. In the subacute phase of the disease 22 patients (group A) were operated on, while 24 patients (group B) were successfully treated conservatively, with recommendation of elective appendectomy after 6 months. In group A indication for operation was PM in 7 patients, diagnostic problems (tumour, hydronephrosis) or complications (peritonitis, intestinal obstruction) in 15 patients. Antibiotic therapy was nearly the same in both group. Peritoneal drainage in postoperative care was performed in 10 patients of group A for 4.7 days on the average. In this group, secondary wound healing was recorded in 3 patients. In group B, elective appendectomy was performed only in 12 patients (50%). In 2 patients, however, a diagnostic error was revealed, i.e. Crohn's disease and an ovarian cyst had been suspected to be a periappendicular mass. Conservative treatment with subsequent elective appendectomy after 6 months seems to be an effective method in the treatment of patients with a typical clinical picture and well bordered periappendicular mass. Appendectomy in the subacute phase of the disease appears to be a safety technique of PM treatment in patients with complications or diagnostic problems.

Acute Disease↗

[Surgical treatment of total anomalous pulmonary venous return].

BACKGROUND: Total abnormal pulmonary venous return (TAPVR), mainly the obstructive type represents the most riskful critical congenital heart defect requiring urgent surgery immediately after birth. THE AIM OF THE STUDY: Analysis of surgical correction of TAPVR results performed from December 1992 to December 1998. METHODS: Twenty-seven patients underwent surgery for TAPVR. 13 of them (48%) presented with hemodynamically severe obstruction. Mean age in the group with obstruction was 3.6 +/- 3.2 days with mean weight of 3282 +/- 537 grams. RESULTS: From the 27 studied patients 5 (18.5%) died. Mean duration of the study in the whole group is 1.91 +/- 2.01 years. Actuarial survival in the first month is 85%, in the second month 81% and remains identical in the 1., 2., 3., 4., 5., 6. year of the study. Univariate analysis identified operation before the year 1996 (p = 0.0056) as a risk factor of immediate mortality. Introduction of ultrafiltration significantly eliminated mortality (p = 0.0101). Remaining variables (age, weight, sex, obstructive TAPVR, TAPVR, extracorporeal circulation duration, pulmonary hypertension) did not significantly influence the survival (p more than 0.05). Multivariate analysis defined operation before the year 1996 as the sole risk factor of mortality (p = 0.0033). In patients operated on in the year 1996 (n = 15) was the survival in the studied period 100%. CONCLUSION: Since the year 1996 the results of surgical treatment of TAPVR significantly improved. The key role in the improvement have better urgent diagnostic and surgery, improvement of surgical technique and myocardial protection, introduction of modified ultrafiltration and the quality of postoperative care. Psychomotor development of children after correction is comparable with healthy population, all patients are in NYHA I class. (Tab. 2, Fig. 4, Ref. 9.)

Heart Defects, Congenital↗

[Late postoperative complications after primary treatment of aortic coarctation].

During the period between 1963 and 1988, 343 patients were operated due to aortic coarctation. 14 patients (4%) were necessarily reoperated for late postoperative complications. In 3 cases (0.8%) reoperation was indicated due to the development of aneurysm in the region of primary operation of aortic coarctation. The development of aneurysm is prevalently predisposed by Vosschulte's indirect aortoplastic operation by the use of a synthetic patch. In the remaining 11 patients (3.2%) the reoperation was indicated owing to the development of recoarctation. Today recoarctation concerns children which were operated under the age of two. The most important moment favourable for the recoarctation development is the secondary constriction of the repaired region in consequence of the coarctation listel fibroid tissue and owing to the incapability of the anastomosis to grow. The perspective rests on using new suture material and combined techniques. Late postoperative complications which follow operations performed due to aorta coarctation remarkably augment the peri and postoperative mortality. Three cases out of the given group of 14 patients terminated by exitus (21%). Hence, it is necessary to remove all moments of predisposition creating a condition favourable for the development of recoarctation and aneurysm. (Tab 2, Fig. 1, Ref. 14.).

Adolescent↗