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Biomedical subjects

I Karnak

Publications and source records attributed to I Karnak.

At least 19 recordsLinked to original sources
PubMed2006

Bannayan-riley-ruvalcaba syndrome from the point of view of the pediatric surgeon.

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is an overgrowth syndrome and polyposis condition, which carries an increased risk for development of benign and malignant tumors of various tissues and organs. A 9-year-old boy with BRRS who had multiple subcutaneous mesenchymal tumors and follicular adenoma of the thyroid and a 14-year-old boy with multiple subcutaneous mesenchymal tumors and gastrointestinal polyposis are presented, with special emphasis on the surgical management of these patients.

Adolescent↗
PubMed2006

Sigmoid colon vaginoplasty in children.

BACKGROUND: Vaginal construction is necessary for the patients with aplasia of Mullerian ducts, testicular feminisation and androgen insensitivity syndromes. Many methods of vaginal construction have been described. We report here the outcomes of six adolescent patients who underwent sigmoid colon vaginoplasty with special emphasis on the surgical technique and outcomes. PATIENTS AND METHODS: Between 1990 and 2003, six patients underwent sigmoid vaginoplasty after a diagnosis of 5alpha-reductase deficiency (n = 3), testicular feminisation (n = 2) or vaginal atresia (n = 1). The mean age was 16 years (13 to 18). Wide spectrum antibiotics and whole-gut preparation were used in all cases. A 15-20 cm segment of sigmoid colon was pulled through the retrovesical tunnel. The proximal end was closed in two layers in patients with 5alpha-reductase deficiency and with testicular feminisation. A distal anastomosis was carried out to the opening made on the vaginal plate (5alpha-reductase deficiency) or on the tip of the shallow rudimentary vagina (testicular feminisation). The sigmoid segment was interposed between the blind end of the atretic vagina and the perineum in the patient with vaginal atresia. Patients were instructed to perform daily vaginal irrigation. The neovagina was examined and calibrated under anaesthesia. No routine vaginal dilatation was recommended. RESULTS: All but one patient had an uneventful postoperative period and were discharged within 7-8 days. All patients had an excellent cosmetic result with an appropriate vaginal length. One of the patients experienced late stenosis of the introitus which responded to dilatations. Mucus discharge was not a significant problem. The patient with vaginal atresia (Bardet-Biedl syndrome) experienced deep vein thrombosis, renal failure and sepsis, resulting in death. CONCLUSION: Sigmoid colon vaginoplasty is a special procedure which appears appropriate for the construction of a new vagina in children. A sigmoid colon neovagina meets all necessary criteria after a vaginoplasty. It provides an adequate diameter and length, and produces less scar tissue in the perineum. It is self-moistening, easily adaptable to the uterus, cervix and rudimentary atretic vaginal segments and does not require routine dilatation. Mild stenosis of the introitus can be treated by dilatations and revision can be easily performed in severely stenotic cases. On the other hand, the patient may face morbidity after laparotomy and other serious complications may occur due to accompanying diseases.

Adolescent↗
PubMed2004

The effects of orchidopexy and orchidectomy on testes of rats subjected to ipsilateral abdominal testis with vas deferens obstruction.

An experimental study was planned to evaluate and compare the effects of orchidopexy and orchidectomy on the testes of rats subjected to unilateral abdominal testis with vas deferens obstruction. Four groups were established. Rats in the control group underwent a sham procedure. While the testis was maintained in the abdomen with the vas deferens ligated for 8 weeks in group 2, rats in groups 3 and 4 underwent orchidopexy or orchidectomy after 4 weeks. Remaining testes were harvested at the end of the 8-week period. Testis and body weights were obtained during harvest. Samples were evaluated through DNA flow cytometry, and percentages of haploid cells were determined. Groups were compared through unpaired t-test, and p-values less than 0.05 were considered significant. All three treatments had decreased testis weight over body weight values of ipsilateral testes. Ipsilateral orchidectomy increased the value among contralateral testes. However, none of the groups had a contralateral testicular value less than the sham-operated group. All three treatments decreased the percentage of haploid cells among ipsilateral testes, but only an abdominal testis was associated with a decrease in the percentage of haploid cells among contralateral testes. Maintaining a testis with an obstructed vas deferens in the abdomen for 8 weeks damages both ipsilateral and contralateral testes. Orchidopexy, while showing amelioration of the ipsilateral testis, spares the contralateral testis as well as orchidectomy. Orchidopexy for an undescended testis with vas deferens obstruction is a rational approach.

Animals↗
PubMed2004

Tracheoesophageal fistula due to disc-battery ingestion.

Ingestion of a disc battery can lead to hazardous complications including tracheoesophageal fistula (TEF), especially when the battery is impacted in the esophagus. Urgent esophagoscopic removal of the battery is essential in all cases. Persisting respiratory symptoms and/or feeding difficulty after removal should alert the surgeon to evaluate for TEF. Esophagography and/or esophagoscopy should be performed. Once the TEF is identified, conservative management is the initial treatment of choice. Delayed primary repair can be tried if spontaneous closure does not occur. The authors present a case of TEF secondary to disc-battery ingestion with review of the current literature and discuss the management of this rare but potentially life-threatening complication.

Esophagoscopy↗
PubMed2004

Thymic teratoma or thymic remnant attached to mediastinal teratoma? The cellular origin of mediastinal teratomas revisited.

Thymic tissue can be encountered in about 20 % of mediastinal teratomas. It is found located at the periphery of the teratoma mass. Although rare, mediastinal teratoma arising within the thymus can be seen. It has not been defined yet whether the thymus is an organ simply attached to the teratoma or whether the teratoma arises from the thymus. The authors present here two illustrative cases of mediastinal teratoma, one raised within the thymus and the other closely attached to the thymic remnant, to discuss the cellular origin of mediastinal teratomas.

Adolescent↗
PubMed2003

Sclerotherapy for lymphangioma in children.

Although surgical excision has been considered to be the treatment of choice by most of the surgeons, sclerotherapy of lymphangioma has gained popularity during recent years. A prospective clinical trial was conducted to evaluate the efficacy of bleomycin and OK-432 sclerotherapies for treating lymphangioma in children. Fifteen patients were enrolled in the study conducted between 1998 and 2002. All patients were hospitalized. Bleomycin or OK-432 was injected into the lesion. Patients were observed in the hospital for 1 day. The response was considered as excellent in cases with total disappearance of the lesion. If the lesion was regressed more than 50% of the original size, the response was considered as good. Little or no change in the size of lesion was considered to be a poor response. Ten girls and five boys with ages ranging from 4 days to 12 years were treated. Five patients had been operated previously for lymphangioma. Most of the lesions were located only in the cervical region (n=13). Other cases revealed extensions into the mediastinum (n=1) or axilla (n=1). Bleomycin (n=8, 2.87+/-2.03 inj.), OK-432 (n=5, 2+/-1 inj.) or both in order (n=2, 6 and 16 injs.) were injected. No allergic reaction, scar formation or pulmonary complication was encountered. Fever (11%), local reactions (4%) and vomiting (2%) were encountered following a total of 55 injections. After a follow-up period of 6-36 months, the responses were excellent in 53.4%, good in 26.7% and poor in 6.6%. Macrocysts disappeared in the remaining 13.3% of patients who had mixed cervicofascial lymphangioma. Lower success rates were encountered among patients who had undergone prior surgery. Sclerotherapy with bleomycin and OK-432 is effective in the treatment of lymphangioma in children. Adverse effects are minor and rarely encountered. However, the surgeon should be alert for the possible serious complications. Sclerotherapy can be also used as an adjunctive therapy in the treatment plan of widespread or mixed forms of lymphangiomas. In the light of our results, sclerotherapy should be preferred as the primary mode of treatment in childhood lymphangiomas.

Antibiotics, Antineoplastic↗
PubMed2003

Inflammatory myofibroblastic tumour of the extrahepatic bile ducts: an unusual cause of obstructive jaundice in children.

Inflammatory myofibroblastic tumour is a rare entity in children with few reported series. The extrahepatic bile duct is an unusual location for this tumour. The authors report here the case of an 8-year-old girl presenting with obstructive jaundice due to inflammatory myofibroblastic tumour of the extrahepatic bile ducts with differential diagnosis of obstructive jaundice in children.

Bile Duct Neoplasms↗
PubMed2002

Xanthogranulomatous pyelonephritis in children: diagnostic and therapeutic aspects.

BACKGROUND: Xanthogranulomatous pyelonephritis (XGP) is an uncommon chronic, inflammatory disease of the kidney. Etio-pathogenesis, diagnosis and management of pediatric XGP is still obscure due to the limited number of cases. Therefore, a retrospective clinical study was carried out to present an updated picture of the entire spectrum of pediatric XGP based on our 30 years' experience covering one of the largest non-collected series treated in a single medical center. METHODS: Records of children who were treated for XGP in our unit from 1970 to 1999, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, past medical history, clinical characteristics, diagnostic procedures,treatment methods, histopathologic findings and outcome. RESULTS: 17 children with a mean age of 6.6 +/- 0.8 years, consisting of 15 males and 2 females, were treated for XGP. Eight patients had a urological disease history and 4 of them underwent surgery for urinary calculi and exstrophia vesica repair. The most common presenting symptoms were abdominal pain, fever,weight loss and anorexia. Palpable flank mass was the most common physical examination finding. Left and right kidneys were involved in 10 and 7 patients, respectively. Diagnostic procedures were intravenous pyelography, retrograde pyelography, ultrasound scan and computerized tomography (CT). Renal calculi and/or calcifications and non-functioning kidney were the most frequent findings in radiologic investigations. By combining all these investigations, the preoperative diagnoses were XGP (n = 2), renal and/or perirenal abscess (n = 5), psoas abscess(n = 2), non-functioning kidney due to recurrent pyelonephritis(n = 4), Wilms' tumor and/or renal clear-cell carcinoma (n = 4). XGP was correctly diagnosed in only two patients based on cr findings. Drainage of the renal or psoas abscess was performed in 8 patients as an initial procedure (surgical drainage in 6 and ultrasound-guided percutaneous drainage in 2 patients). Surgical procedures included nephrectomy (n= 13), partial nephrectomy (n=2), nephrectomy and diversion of reno-colic fistula(n= 1), and renal biopsy and nephrostomy (n= 1). Operative and postoperative complications were colonic perforation (n= 3) and wound infection (n = 3). Complications were noted only in patients who underwent nephrectomy without initial drainage procedures. Histopathologic examinations showed diffuse and focal XGP in 14 and 3 patients, respectively. CONCLUSION: XGP should be included in the differential diagnosis of all children presenting with perirenal or psoas abscess, renal mass and/or non-functioning kidney associated with/or without urolithiasis. Clinical awareness and a high index of suspicion is required to achieve the correct preoperative diagnosis and appropriate management. CT seems to be the most valuable imaging method for the diagnosis. We strongly recommend percutaneous drainage of the abscess and adjunctive antibiotic therapy prior to nephrectomy to avoid complications. Complete nephrectomy is the proper treatment for the diffuse form whereas frozen section biopsies followed by partial nephrectomy are mandatory for the proper treatment of focal disease.

Adolescent↗
PubMed2002

Pulmonary metastases in children: an analysis of surgical spectrum.

Pulmonary surgery is frequently used for the treatment of metastasis or nodules in children with various types of malignancies. However, the indications and effectiveness of pulmonary metastatectomy have not been evaluated recently. Therefore, a retrospective study was conducted to analyse the results of pulmonary metastatectomy in children. Children who underwent pulmonary metastatectomy at our department between 1990 and 2000 were reviewed. Eighteen children consisting of 11 boys and 7 girls (age range, 3 to 18 years) underwent thoracotomy for pulmonary metastasis excision. The primaries were osteosarcoma (n = 2), synovial sarcoma (n = 1), fibrosarcoma (n = 1), Ewing's sarcoma (n = 2), mesenchymal chondrosarcoma (n = 1), Wilms' tumour (n = 4), clear-cell sarcoma (n = 1), Hodgkin lymphoma (n = 3), hepatoblastoma (n = 1), hepatocellular carcinoma (n = 1) and haemangioendotheliosarcoma (n = 1). Pulmonary metastases were encountered either at the time of initial diagnosis (22 %) or occurred within 6 months to 5 years. They were frequently nodular (94 %), unilateral (94 %) and located in the right lung (70 %). The number of metastases were frequently one (56 %) or two (28 %). Excision was done by means of wedge resection (88 %), segmentectomy (6 %), and lobectomy + wedge resection (6 %). The nodules contained tumour cells in most cases (n = 14) (78 %), mature nephrogenic elements (6 %) and no tumour tissue (16 %) in the remaining cases. Histology was similar to that of the original tumour in 12 cases. However, synovial sarcoma was encountered in metastasis in one case with fibrosarcoma primary. Re-thoracotomy was performed in 22 % of cases for the recurrent lesion, which in only one case was a true local recurrence. Overall disease-free survival rate was 56 % during the follow-up period (mean, 36.4 +/- 31.8 months). Pulmonary metastatectomy may increase survival in carefully selected children, though it is unlikely to cure the patient. Therefore combined therapies such as chemotherapy and/or radiotherapy should be continued in the postoperative period.

Adolescent↗
PubMed2002

Ectopic thymic tissue as a rare and confusing entity.

A 16-year-old girl with intrathyroidal ectopic thymic tissue, which was diagnosed incidentally after surgery for thyroid nodule, is reported to emphasise the possible clinical and surgical presentations of this rare entity.

Adolescent↗
PubMed2001

Multilocular cervical thymic cyst: an unusual neck mass in children.

Thymic cysts are usually considered uncommon lesions in the differential diagnosis of neck masses. Approximately 100 cases have been reported to date and most of these cases have occurred asymptomatically. Herein, the authors report a 4-year-old boy with cervical thymic cyst, which was misdiagnosed preoperatively as conglomerated lymph nodes. The correct diagnosis was made after surgical excision and through determination of specific histopathological findings of the thymic cyst. The etiologic theories, differential diagnosis and the treatment of thymic cysts are discussed.

Child, Preschool↗
PubMed2001

Late presentation of Bochdalek hernia: clinical and radiological aspects.

Three infants with late presentation of Bochdalek hernia are presented. The presenting symptoms were cough, intermittent vomiting, dyspnea, and cyanosis. Initial diagnoses of isolated paravertebral mass and foreign material aspiration were made in two infants, based on plain chest x-ray findings and history of the patients. Further radiological investigations, such as contrast upper gastrointestinal series or enema, computerized tomography, and magnetic resonance imaging of the chest, suggested the diagnosis of Bochdalek hernia. The hernia was found on the left side in two patients and on the right side in one. At operation, the stomach, small intestine, and spleen were found as herniated organs in one patient, ascending colon in one, and all of the small intestine together with ascending colon in the other. A congenital diaphragmatic defect should be suspected in every child presenting with unusual respiratory or gastrointestinal symptoms and with abnormal chest x-ray findings. The radiological findings vary greatly from one case to another, and even in the same case at different times because of differences in herniated organs and intermittent spontaneous reduction. The possibility of congenital diaphragmatic hernia should be kept in mind to avoid a wrong diagnosis, undue delay in diagnosis, and inappropriate treatment.

Age of Onset↗
PubMed2001

Colonic atresia: surgical management and outcome.

Colonic atresia (CA) is a very rare cause of intestinal obstruction, and little information has been available about the management and predictors of outcome. A retrospective clinical trial was performed to delineate the clinical characteristics of CA with special emphasis on surgical treatment and factors affecting outcome. Children with CA who were treated in our department between 1977 and 1998 were reviewed: 14 boys and 4 girls aged 1 day to 5 months. All but 2 referred patients and 1 with prenatal diagnosis presented with intestinal obstruction. Plain abdominal X-ray films showed findings of intestinal obstruction in 14 cases; a barium enema demonstrated a distal atretic segment and microcolon in 4. The types of atresia were IIIa (n=9), I (n=6), and II (n=3). Type IIIa atresias were located proximal to the splenic flexure (n=8) and in the sigmoid colon (n=1), type I atresias were encountered throughout the colon; and all type II atresias were proximal to the hepatic flexure. Associated anomalies were multiple small-intestinal atresias (MSIA) (n=4), gastroschisis (GS) (n=2), pyloric atresia (n=1), Hirschsprung's disease (n=1), and complex urologic abnormalities (n=1). The initial management was an enterostomy in 15 patients (83%), including 2 referred and 2 with GS, and primary anastomosis in the remaining 3 (17%). Secondary procedures were the Santulli operation (n=2), colostomy closure and recolostomy followed by a Swenson operation (n=1), sacroabdominoperineal pull-through (n=1), and colostomy closure (n=1). Leakage was encountered in all primarily anastomosed patients. The overall mortality was 61%. Deaths occurred in patients with associated major anomalies (GS 2, MSIA 3, pyloric atresia 1) (55%) and in 3 patients who were initially managed by primary anastomosis (27%). Two additional patients died of sudden infant death syndrome (18%). Type I atresia was more common than in previously reported series and was associated with proximal multiple atresias. The initial management of CA should be prompt decompression of the intestine by an ostomy procedure, preferably end- or double-barrel. The type of surgery (primary anastomosis without prior colostomy) and associated abnormalities are the major determinants of poor outcome.

Anastomosis, Surgical↗
PubMed2001

Abnormal esophageal anatomy associated with a congenital diaphragmatic hernia: report of a case.

Gastroesophageal reflux associated with anatomic and functional abnormalities of the esophagus may be encountered following the repair of a congenital diaphragmatic hernia (CDH). We report herein the case of a newborn male infant with CDH found to have an air-filled paravertebral structure. Upper gastrointestinal series confirmed the presence of an ectatic esophagus with poor peristalsis and severe gastroesophageal reflux. The patient required jejunostomy after a safe period of total parenteral nutrition. He tolerated oral feeding following medical treatment without the need for antireflux surgery. The association of dysphagia, esophageal ectasia, and gastroesophageal reflux is rarely seen with CDH, and feeding intolerance is a self-limited disorder that does not usually necessitate antireflux surgery.

Deglutition Disorders↗
PubMed2001

Diaphragmatic injuries in childhood.

The early detection and surgical repair of diaphragmatic injury is vital for saving the life of symptomatic children suffering from trauma. Furthermore, an accurate diagnosis may be difficult, particularly in right-sided diaphragmatic injuries. Fifteen children with diaphragmatic injury treated at our department between 1977 and 1998 were evaluated retrospectively. They included 9 boys and 6 girls, and consisted of 8 left- and 6 right-sided injuries, and 1 midline retrosternal injury, due to a blunt (n = 13) or penetrating (n = 2) trauma. The most frequent symptoms were dyspnea (86.6%), and abdominal pain and vomiting (13.4%). The diagnosis was confirmed preoperatively in 13 patients based on chest X-ray (n = 7), gastrointestinal series (n = 3), barium enema (n = 1), and computed tomography and/or ultrasonography findings (n = 2). Among these, a diagnostic delay occurred in 3 patients with right-sided injuries. A primary repair was performed through a laparotomy (n = 14) or thoracotomy (n = 1). Postoperative intussusception was the most frequent complication (n = 2). Diaphragmatic injury must be considered in any child who has sustained a thoracoabdominal trauma. Serial chest X-rays should be taken especially in right-sided injuries in which a considerable diagnostic delay may occur. Further radiological methods may be necessary to confirm the diagnosis. In addition, postoperative intussusception may be encountered following diaphragmatic repair.

Abdominal Pain↗
PubMed2001

The effects of flow rate, length and external pressure upon the pressure required for fluid to flow through a ureter.

OBJECTIVE: To determine in vitro the effects of increments of external pressure on the pressure required to conduct fluid through ureters of various lengths at different flow rates, as the flow of a fluid through a collapsible tube is influenced by various factors (e.g. external pressure, the pressure gradient between the ends, the length and diameter of the tube, and the viscosity of fluid). MATERIALS AND METHODS: Two in vitro systems were designed, composed of three parts, i.e. a perfusion line, an exit line and a container of two different widths in which short or long ureteric segments, obtained from cattle, could be placed; the ureter was connected to the perfusion and exit lines. Physiological saline was added to the container until the desired external pressure was applied to the ureter. The flow pressure (height of the perfusion line) was recorded when producing flows through ureters of varying length at 1.5 and 6 mL/min, and determined under various external pressures. The intra-ureteric pressure during flow was also monitored by a pressure transducer. The four combinations of long and short ureters with high and low flow rates were compared using analysis of variance, with the Pearson correlation coefficient used to evaluate the relationships between the various pressures. RESULTS: There were close relationships between flow pressure and external pressure (r = 0.727), intra-ureteric and external pressure (r = 0.766), and the flow pressure and intra-ureteric pressure (r = 0.940, all P < 0.001). Increments in external pressure resulted in greater flow and intra-ureteric pressure (P < 0.05). Increases in flow pressure were more pronounced than increases in intra-ureteric pressure at the same external pressure (P < 0.05) at high flow rates. A longer ureter and higher flow rates caused greater intra-ureteric pressure (P < 0.05). CONCLUSION: External pressure increases the pressure required to conduct fluid through a ureter and the effect is more pronounced at high flow rates. The length of the ureter also affects the flow pressure at high flow rates. Therefore, flow through the ureter follows the Poiseuille equation only at high flow rates. Thus, increases in intra-abdominal pressure may cause greater intrapelvic pressure and induce ureteric obstruction, contributing to the pathogenesis of hydronephrosis.

Animals↗
PubMed2001

Renal pelvic pressure responds with augmented increases to increments in intraabdominal pressure.

BACKGROUND/PURPOSE: Flow of a fluid through a collapsible tube is under the influence of various factors including the external compressing pressure. Because the intraabdominal pressure may compress the ureter, an experimental study has been planned to determine and compare the normal intraabdominal and renal pelvic pressures and the alterations in renal pelvic pressure in response to the increments in intraabdominal pressure in the rabbits. METHODS: Eight adult rabbits were used for the experiment. Under general anesthesia, an urethral catheter, a nasogastric tube, and an intraperitoneal catheter were placed to measure intravesical (IVP), intragastric (IGP), and intraabdominal pressures (IAP), respectively. Intracranial pressure monitorization catheter was placed into the renal pelvis to monitor intrapelvic pressure (IPP). Basal pressure measurements have been recorded. The pressures have been recorded in every 5 minutes, and IAP has been increased gradually about 3 to 4 cm of water pressure in every step for 30-minute periods. RESULTS: Increases in the intrapelvic pressure values have been significantly higher than the increases in the IAP (P < .001). A significant correlation has been found between IPP and IAP (P = .000, r = 0.866). By using linear regression analysis the relationship has been found to be IPP = 7.303 + 1.985 (IAP). Intragastric pressure values have been higher compared with IAP values (P < .001), whereas intravesical pressures have not differed from IAP (P > .05). CONCLUSIONS: Elevations in IAP results in augmented increases in the IPP. Poiseuille and Laplace Laws suggest this augmented increase to resemble proximal ureteric obstruction. Increases in IAP may simulate proximal ureteric obstruction and may take part in the pathogenesis of hydronephrosis. J Pediatr Surg 36:901-904.

Animals↗