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Biomedical subjects

I Karnak

Publications and source records attributed to I Karnak.

At least 37 records · Page 2Linked to original sources

Inflammatory myofibroblastic tumor in children: diagnosis and treatment.

BACKGROUND/PURPOSE: Inflammatory myofibroblastic tumor (IMT) is a rare benign neoplasm. Although it is commonly seen in children, the number of childhood cases in the current literature is limited. Furthermore, malignant degeneration or transformation to lymphoma in the recurrent or residual IMT have directed attention to this interesting entity. Herein, the authors present their experience with IMT with special emphasis on diagnosis and treatment. METHODS: All records of children treated with diagnosis of IMT between 1977 and 1999 inclusive were evaluated retrospectively. RESULTS: Seven children were treated for IMT with the mean age of 9.14 +/- 2 years (range, 6 to 12 years). Male to female ratio was 5:2. Respiratory symptoms and clubbing were present in a patient with pulmonary IMT (n = 1). Abdominal pain (n = 3), fever (n = 2), and weight loss (n = 4) were encountered in intraabdominal IMTs. The most frequent physical finding was palpable intraabdominal mass (n = 4). Plain films showed nonspecific findings such as radiodense area in the hemithorax (n = 1), displacement of bowel segments (n = 2), air-fluid levels (n = 1), and amorphous calcification (n = 4). Ultrasonography and CT showed calcified masses in 4 cases. Except the case with intrathoracic IMT, all the tumors were located in the abdomen at various sites such as cardioesophageal junction (n = 1), left hepatic lobe (n = 1), mesentery of the small bowel (n = 2), and antimesenteric wall of the descending colon (n = 1), gastrosplenic region and porta hepatis (n = 1). Tumor sizes ranged from 3 x 2 x 2 cm to 15 x 15 x 13 cm. The masses were excised totally in all but one case. Infiltrated organs (esophagogastric junction, a segment of jejunum, and spleen, stomach wall, and renal capsule) were resected in 3 cases. Total surgical excision of IMT was considered adequate for treatment in 6 cases. One patient with aggressive IMT required further treatments such as immunomodulation and chemotherapy and died of neutropenic sepsis. CONCLUSIONS: IMT is a benign neoplasm rarely presented with malignant features such as local invasiveness, recurrence, distant metastasis, or malignant transformation. IMT can be suspected preoperatively through some hematologic abnormalities and radiologic findings, but precise diagnosis should be made on the basis of histologic findings. Complete surgical resection and close follow-up are all necessary for appropriate treatment to avoid recurrences as well as unnecessary and potentially harmful therapy. The optimal management of locally aggressive and recurrent forms should be decided individually for each patient. J Pediatr Surg 36:908-912.

Abdominal Neoplasms↗

Achalasia in childhood: surgical treatment and outcome.

Achalasia is a rare disorder of the esophagus in children. From 1971 to 1999, 20 children with achalasia of the esophagus have been treated at our institution including two patients who were referred to us after esophagomyotomy. There were 13 boys and 7 girls (average age, 8.2 years; range 2 to 15 years). Presenting symptoms were vomiting (n = 18), dysphagia (n = 11), loss of weight (n = 5), recurrent respiratory infections (n = 3), cough (n = 2) and noisy respiration (n = 1). Barium swallow established diagnosis in all patients. Esophagoscopy was used as a supportive investigation in some patients (n = 10). Nineteen patients underwent Heller-Zaiger operation (modified Heller esophagomyotomy) either by transabdominal (n = 16) or transthoracic approach (n = 3) with (n = 6) or without concomitant antireflux procedure. The postoperative period was uneventful in all patients. Follow-up ranged from 2 months to 16 years. Decreased or absent peristalsis persisted in initial control esophagograms in all patients. Gastroesophageal reflux was encountered in only one patient. Complete relief of symptoms was noted in 14 patients. Mild to moderate dysphagia was encountered in 5 patients and all of them were evaluated by endoscopy and upper gastrointestinal series. Dysphagia resolved spontaneously in one child and following two dilations in another child. One child has moderate dysphagia after a short follow-up period. Esophageal stenosis was seen in the remaining two and subsequently treated by esophagocardioplasty (Heyrowsky and Wendel operations). Achalasia should be considered in the differential diagnosis in any children with persistent dysphagia, recurrent respiratory tract infections and vomiting, including children treated for clinically suspected gastroesophageal reflux. The obvious mode of treatment is surgical myotomy in children. Modified Heller esophagomyotomy is the procedure of choice, which can be performed either by an abdominal or a thoracic approach. The need to carry out a concomitant antireflux procedure remains controversial. The most frequent postoperative problem is persistent dysphagia. It may be self-limited in some cases and disappear during follow-up. Resistant stenosis following esophagocardiomyotomy can be treated by esophagocardioplasty procedures such as Heyrowsky and Wendel operations.

Adolescent↗

Surgical injury of the biliary tract in children.

A retrospective clinical study was performed to evaluate the etiology, incidence, diagnosis, management and outcome of patients presenting with surgical injury to the biliary tract. 4 boys were treated for operative biliary tract injuries between 1970 and 1997. This number represents less than 0.03% of all patients who underwent laparotomy in our unit during the same period. The mean age of the patients at presentation was 7.5 +/- 3 (range, 4 to 10 years). Accidental ligation of choledochus (n = 2), vascular insult of the biliary tract (n = 1) and formalin toxicity (n = 1) were the causes of injuries. The latter presented with caustic sclerosing cholangitis and biliocutaneous fistula while obstructive cholangitis (n = 2) and jaundice (n = 1) were noted in the remaining patients. The duration between surgical injury and presentation ranged from 6 to 125 days. All patients presented with elevated levels of transaminases, alkaline phosphatase and bilirubin. Ultrasonography, percutaneous transhepatic cholangiography and biliary drainage catheter placement were performed in all patients to visualize the extent of injury and to provide better patient status for operation. Biliary stent application provided temporary relief of obstruction in one patient, but all patients required surgical treatment subsequently. Roux-en-Y hepaticojejunostomy (n = 3), and choledochoduodenostomy (n = 1) were the operative procedures. No complications were encountered in the short and long-term follow-up. Our experience revealed that surgical biliary tract injuries have special features that warrant consideration with respect to prevention and management in children. They may be caused by partial or complete transection, suture ligation, clip application or vascular insult and can be avoided by adequate exposure, accurate gentle dissection, use of hemostatic clips rather than clamps and ties, and the liberal use of operative cholangiography. The presenting clinical picture depends on the cause, extent and duration of the injuries. Preoperative detailed evaluation of the hepatobiliary system by radiological and endoscopic means is mandatory for successful treatment. Percutaneous and/or endoscopic techniques can be employed in selected cases, but if these fail or can not be done, open surgical techniques should be performed without hesitation as delayed treatment results in biliary cirrhosis and hepatic failure. Excision of excessive scar tissue at the biliary tract and portal hilus, constructing the widest possible stoma, obtaining mucosa to mucosa approximation around 360 degrees, enduring a good blood supply to the anastomotic line and avoiding tension on the anastomosis are mainstays of successful surgery. Thus, reconstructive biliary tract surgery should be considered as a specialized procedure and should be performed by skillful and experienced hands.

Adolescent↗

Endobronchial leiomyoma in children.

Pulmonary leiomyomas are among the most rarely encountered benign tumors of the lung in all age groups. The endobronchial origin and occurrence in children is extremely rare for this tumor. The cases described reflect the efficacy of bronchoscopy in the diagnosis and treatment of endobronchial leiomyoma encountered in children.

Bronchial Neoplasms↗

Pulmonary blastoma: diagnostic and therapeutic aspects.

Pulmonary blastoma (PB) is an extremely uncommon intrathoracic neoplasm in children. It accounts for 0.5% of all primary malignant tumors of the lung, and 20%-25% of the cases present in childhood. A new case of PB in a 3-year-old girl is discussed with special emphasis on diagnosis and treatment. The authors point out that the possibility of a primary lung tumor should be considered for any childhood cases with intractable signs and symptoms of a space-occupying lesion. A primary lung malignancy should not be excluded only on the basis of the patient's age, and childhood lung disorders should receive the same detailed and vigorous diagnostic evaluation and appropriate treatment given to adults.

Abdominal Pain↗

Organ growth and lung maturation in rabbit fetuses.

The rabbit fetus is one of the most commonly used animal models in experimental studies investigating fetal organ development. However, there is no detailed information about normal growth of organs of rabbit fetuses in English language literature. Fetal rabbits were studied in the second half of gestation between 18th and 30th days. Amniotic fluid volume, body mass (BM), lung, heart and liver masses (LM, HM, LiM), lung and thorax volumes (LV, TV) were determined and LM/BM, HM/BM, LiM/BM, TV/BM and LV/TV ratios were calculated. Additionally fetal lungs were evaluated histologically, BM, LM, HM, LiM and LV were increased until 27th gestational day and then remained unchanged. TV was always increased between 18th gestational day and term. The lung maturation was almost completed in the 27th-28th gestational days. Therefore, BM, LM, HM, LiM and LV are the parameters that can be used to evaluate normal fetal growth between 18th and 27th gestational days. TV seems to be the predictive parameter for evaluation of normal fetal growth during the second half of gestation in rabbit fetuses; 20th and 27th days of gestation are more appropriate for experiments to evaluate lung maturation.

Amniotic Fluid↗

A previously unmentioned surgical observation in the treatment of intussusception.

Intussusception is one of the most common causes of bowel obstruction in infancy which may require surgical treatment. We have recently recognized a pathologically weakened linear area located on the wall of the reduced colon at operation. Thus, a retrospective study was conducted to evaluate the operative and pathological findings of the resected bowel segments for the treatment of intussusception. A pathologically weakened longitudinal linear area was encountered in five patients. This line was strikingly antimesenteric and under the taenia libera. The bowel wall was very thin and effaced on palpation along this whitish line and showed mucosal necrosis, disruption of the muscularis mucosa, and loss of some of the muscular tissue on microscopic examination. The line may result from compression of the inner layers of the bowel wall between the intussusceptum and the noncompliant taenia. Location on the antimesenteric border and under the taenia libera can be explained by local vascular compromise due to the distribution of the terminal arteries of the colon. Thus, the antimesenteric border as well as the mesenteric side should be checked carefully for a longitudinal weakened pressure line. The recognition of such a potentially dangerous weak line on the bowel wall indicates resection.

Child, Preschool↗

Congenital lobar emphysema: diagnostic and therapeutic considerations.

BACKGROUND/PURPOSE: Congenital lobar emphysema (CLE) is characterized by overinflation of a pulmonary lobe and may present as a diagnostic and therapeutic dilemma. The authors have reviewed their experience to clarify the controversies about the diagnosis and management of CLE in children. METHODS: Children who had CLE and were treated at our department between 1991 and 1998, inclusive, were reviewed. RESULTS: Fourteen children consisting of 8 boys and 6 girls (age range, 25 days to 2.5 years) had CLE. Eight children (57%) were symptomatic at birth. Major presenting symptoms were dyspnea (n = 8, 57%), cyanosis (n = 6, 43%) and recurrent respiratory tract infection (n = 4, 28.5%). Chest x-rays and computerized tomography scans showed hyperaeration of the affected lobes in 14 and 12 patients, respectively. Pulmonary perfusion scan was performed in 8 patients and showed loss of perfusion in the affected lobe in 7 cases. The affected sites were left upper lobe (n = 8, 57%), right middle lobe (n = 4, 28.5%) and right upper lobe (n = 2, 14.5%). Congenital cardiac anomalies were encountered in 2 patients (14.5%). Ten children underwent lobectomy, and postoperative course was uneventful. Nonoperative management was performed in 4 cases (28.5%) presenting at older age with milder symptoms. In the latter, although symptoms subsided, radiological abnormalities persisted during the follow-up period of 3 months to 4 years. No mortality was encountered in the series. CONCLUSIONS: The diagnosis of CLE is established by combined radiological and scintigraphic imaging methods. Surgical excision of the affected lobe is the appropriate treatment in all infants under 2 months of age and in infants older than 2 months presenting with severe respiratory symptoms. Infants older than 2 months presenting with mild to moderate respiratory symptoms associated with normal bronchoscopic findings can be treated conservatively. In conservative management, the children should be under close follow-up, and the family should be alarmed about the disease. Further studies are required to determine the long-term outcome of conservative management.

Algorithms↗

Spectrum of complicated intestinal amebiasis through resected specimens: incidence and outcome.

BACKGROUND/PURPOSE: Entamoeba hystolytica (EH) is an enteric protozoan that may cause serious complications that require operative management in children. A retrospective clinical trial was performed to find out the incidence and outcome of complicated intestinal amebiasis (IA) by means of surgically resected specimens in children. METHODS: The histopathologic evaluation of 554 intestinal specimens (including appendix and polyp) of 482 patients between 1980 and 1997, inclusive, were reviewed. Intestinal biopsy specimens taken from successfully medically treated patients with uncomplicated IA were excluded. RESULTS: Complicated IA was noted in 18 children (3.7%) consisting of 7 girls and 11 boys with a mean age of 9.2+/-2.3 years. EH was present in the (1) appendices of 4 patients with perforated appendicitis and 3 patients with normal appendix who underwent negative exploration; (2) juvenile polyps of 4 patients presenting with hematochesia; (3) colonic wall of 1 patient presenting with intussusception and treated by resection and anastomosis; (4) cecum of 1 patient presenting with right lower quadrant mass and underwent exploration with the presumptive diagnosis of lymphoma; (5) colonic wall of 5 patients who underwent ileostomy followed by subtotal colectomy and endorectal pull-through with the presumptive diagnosis of chronic inflammatory bowel disease (n = 3), toxic megacolon + peritonitis (n = 1), and total colonic polyposis (n = 1). All above-mentioned patients are alive and free of symptoms. CONCLUSIONS: The diagnosis of IA should be considered in a very wide spectrum of clinical appearances. IA may be associated with colonic polyps and perforated appendicitis, may act as a leading point for intussusception, and may mimic the clinical picture of appendicitis and lymphoma resulting in negative explorations. The diagnosis and treatment of complicated IA in patients who received a misdiagnosis of inflammatory bowel disease is a great challenge and requires major and emergency surgery.

Appendicitis↗

Colorectal carcinoma in children.

BACKGROUND/PURPOSE: Colorecta I carcinoma is extremely rare in children and presents with a poor prognosis. Surgical management and long-term follow-up of this entity are still obscure because of lack of data. Therefore, a retrospective clinical trial was performed to evaluate the clinical characteristics of childhood colorectal carcinoma and to determine the predictors of poor outcome. METHODS: Records of children who had colorectal carcinoma and were treated at our unit between 1972 and 1997, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, clinical characteristics, diagnostic procedures, extent of disease, treatment methods, histological types, and outcome. A modified Dukes staging scheme was used. RESULTS: There were 12 boys and 8 girls who were treated for colorectal carcinoma (range, 7 to 16 years). Predisposing conditions were encountered in 2 patients (10%), one with Bloom's syndrome and another with familial occurrence of colonic carcinomas. Predominant symptoms were abdominal pain, vomiting, and rectal bleeding. Barium enema, ultrasonography, computerized tomography, and endoscopic procedures were used for the diagnosis. Rectosigmoid region was the most common site for the primary tumor (65%). All patients presented with advanced stages of disease (stage C, 7; stage D, 13). Surgical procedures were incisional biopsy (n = 4), palliative permanent colostomy (n = 4), segmental resection (n = 5), complete resection (n = 6), and rectal biopsy (n = 1). Peritoneum was the most common site of extensive intraabdominal disease followed by omentum majus and liver. The lung also was involved in 2 patients. Predominant histological type was mucinous adenocarcinoma (80%). All patients but one received adjuvant chemotherapy, and 2 received palliative radiotherapy. Thirteen patients died of disease in a period ranging from 1 day to 1 year after initial surgery. The fate of 4 patients who were discharged in a very ill status was unknown. Three patients were alive for 2 years to 4 years postoperatively, and one of them presented with end stage disease. CONCLUSIONS: Delayed diagnosis, advanced stages of disease at presentation, and, most importantly, mucinous type of histology are the major determinants of poor outcome in childhood colorectal carcinoma. We emphasize that possibility of a malignant colorectal tumor should be considered for any childhood case with signs and symptoms of intestinal obstruction, intractable abdominal pain, alteration in bowel habits and gastrointestinal bleeding. Colorectal malignancy should not be excluded only on the basis of the patient's age. Because of the steadily increasing incidence of precancerous bowel diseases and poor prognosis of colorectal carcinoma, childhood cases of bowel disorders should receive the same detailed and vigorous diagnostic evaluation and appropriate treatment as given to adult cases. Contrast studies, ultrasonography, computed tomography, and endoscopy are essential procedures for both confirming the diagnosis and detecting the extent of the disease.

Adenocarcinoma, Mucinous↗

Granulomatous osteomyelitis of the sternum presenting with a parasternal mass: a possible relation to the bacillus Calmette-Guérin vaccine.

Because they are frequently malignant, swellings of the chest wall during infancy are alarming lesions. Sternal osteomyelitis is a rare condition that may present with a chest wall mass locating in the parasternal region. A parasternal mass of granulomatous osteomyelitic origin is an extremely rare cause with only few case being reported to date. Herein, the authors report on 2 infants presenting with parasternal masses of sternal granulomatous osteomyelitic origin and discuss the possible etiologic role of the bacillus Calmette-Guérin vaccine.

Adjuvants, Immunologic↗

Adrenal calcification in children: a case report.

Unilateral adrenal calcification was encountered incidentally in a 9-year-old boy during evaluation of enuresis. He was a large baby at birth, and perinatal history was suggestive of difficult labor. The case has been discussed through the possible causes of adrenal calcification and the management of these children. Once the diagnosis of idiopathic adrenal calcification is made in light of detailed perinatal history, biochemical investigations, and roentgenologic examinations, these children can be observed by control evaluations with regular intervals.

Adrenal Gland Diseases↗

Pulmonary lymphomatoid granulomatosis in a 4 year old.

The authors report a pulmonary lymphomatoid granulomatosis (LG) in a 4-year-old girl. The clinicopathologic and radiological features of this rare entity are discussed with special emphasis on differential diagnosis, including a brief literature review. LG is an aggressive multiorgan disease that primarily affects the adult lung. There are no specific presumptive clinical and laboratory findings, including tumor markers and imaging techniques, that distinguish LG from other pulmonary nodular lesions. The most important diagnostic aid is to bear this entity in mind when a child presents with pulmonary nodule associated with intractable long-lasting symptoms. Open lung biopsy and total excision is mandatory for the appropriate diagnosis and treatment.

Child, Preschool↗

Epigastric heteropagus: a case report with review of the literature.

Incomplete conjoined twinning or heteropagus attached at the autosite's epigastrium is an extremely rare form of conjoined twinnings. We report a case of epigastric parasitic twinning in which the parasite has a well developed lower trunk and pelvis with rudimentary lower limbs, and well developed upper extremities without shoulder girdles and thoracic cage. The clinical features of this rare entity are discussed with a literature review. We emphasize that in spite of monstrous appearance, autosite component of epigastric heteropagus can be treated successfully with minor surgery. This fact should be kept in mind during the intrauterine evaluation of these type of anomalies in order to avoid needless terminations.

Abnormalities, Multiple↗

In-utero defecation: fact or fiction?

An experimental study was performed to investigate the gastrointestinal motility and in-utero defecation by radionuclide techniques. Forty-eight New-Zealand white rabbit fetuses at 25 days' gestation (fullterm, 31 to 32 days) were divided into two groups as A (n: 24) and B (n: 24). 0.1 ml of Technetium-99m (99mTC)-HIDA (a derivative of iminodiacetic acid) containing 1 mCi of radioactivity was injected into the gluteus muscle of each fetus, which had been exposed through the uterus. This procedure was used for all fetuses and additionally surgical closure of anus by a purse string suture was performed in Group B fetuses. After replacing the fetus and uterus into the abdomen, and beginning 1 hour after injection, a live fetus was killed each hour for 24 hours. Tissue samples from the reference organs (lung, heart, stomach, kidney, bladder), liver, meconium in the proximal, mid and distal bowel, and amniotic fluid were taken. The radioactivity of each sample was determined by a gamma counter and the percentage injected dose (uptake) per gram of tissue is calculated. 99mTc-HIDA was predominantly trapped by the liver via systemic circulation and excreted into the gastrointestinal tract in both groups through which it passed into the amniotic fluid only in Group A. The very low radioactivity levels detected in the amniotic fluid of Group B originated from urinary tract and indicated the in-vivo stability of 99mTc-HIDA, thus reliability of the experimental model. Intestinal transport of a radiopharmaceutical agent in both groups and demonstrated passage into the amniotic fluid only in Group A strongly suggest that fetal defecation is a physiologic event.

Amniotic Fluid↗