Pseudocholangiocarcinoma sign associated with hepatoblastoma: a previously unreported entity in children.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to M E Senocak.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Primary ovarian malignant lymphomas are rarely encountered in children. We present herein the unusual case of an 11-month-old female infant with primary bilateral non-Hodgkin's lymphoma of the ovaries who was treated by bilateral salpingo-oophorectomy followed by chemotherapy. The clinicopathological features of this rare entity are discussed, focusing special emphasis on the diagnostic and therapeutic strategies.
An 11-month-old girl with a prolonged history of bloody, mucoid diarrhea is presented. Although the initial diagnosis given by the rectosigmoid biopsy obtained during laparotomy was neuronal intestinal dysplasia, accompanying findings including mixed inflammatory cell infiltration of the mucosa and submucosa with mucosal ulcerations suggested nonspecific colitis. The subsequent biopsy specimen that was obtained after performing colostomy and treating with broad-spectrum antibiotics and rectal irrigations showed improvement in the structure of ganglion cells and submucous and myenteric plexuses. Although the mucosal ulcerations and inflammatory reaction improved, the colonic stricture persisted, so the Duhamel procedure was performed, and the patient had an uneventful outcome. It is claimed that inflammatory disease of the rectosigmoid colon of unknown etiology and neuronal intestinal dysplasia have occurred together in the current case or that one disease might cause the other in time.
BACKGROUND/PURPOSE: The intraabdominal pressure (IAP) of children presenting with acute, perforated, or suspected appendicitis were determined and compared to define if the IAP has any diagnostic value or helps to predict a complicated course. METHODS: Eighty-four patients with a initial diagnosis of appendicitis were evaluated. In addition to preoperative measurements, IAP of each patient was determined repeatedly on the postoperative first, second, and third days. The patients were grouped according to the final diagnoses as acute, perforated, or suspected appendicitis or negative exploration. The preoperative and postoperative IAP of the patients were compared among the groups. Postoperative complications were recorded, and IAP of those patients were additionally compared with the others in the same group. RESULTS: Whereas a normal appendix was found in 4 of the operated patients, 27 and 38 patients had acute and perforated appendicitis, respectively. The mean preoperative values of IAP for acute, perforated, or suspected appendicitis and negative exploration were 6.2 +/- 0.4, 9 +/- 0.3, 0.3 +/- 0.4, and 3 +/- 0.4 cm H2O, respectively (P< .001). Postoperative first day and second day values of the IAP for acute appendicitis, perforated appendicitis, and negative laparotomy groups were 2 +/- 0.2 and 0.6 +/- 0.1,3 +/- 0.1 and 1.5 +/- 0.1,0.5 +/- 0.6 and -0.2 +/- 0.6 cm H2O, respectively. The difference between acute and perforated appendicitis groups was significant (P< .05). Wound infection was encountered in 7 among 38 patients with perforated appendicitis. The preoperative and first postoperative day IAP values of patients with perforated appendicitis who experienced a wound infection and who were without a wound infection have been 11.8 +/- 0.4 and 4.8 +/- 0.2, and 8.4 +/- 0.2 and 3.1 +/- 0.3 cm H2O (P< .001). Discriminant analysis has shown that 93.3%, 70.4%, and 73.3% of patients with suspected, acute, and perforated appendicitis have been within the expected groups. IAP less than 1.39 cm H2O has excluded appendicitis with a 95% confidence interval. Although the interval has been between 5.40 and 7.04 cm H2O for acute appendicitis, it has varied between 8.46 and 9.70 cm H2O for perforated appendicitis. CONCLUSIONS: Although the IAP does not increase in conditions mimicking appendicitis, it increases among children with appendicitis. A further increase is encountered among children with perforated appendicitis. Complicated course is encountered among children with highest IAP values. Therefore, IAP may be used both as a diagnostic parameter and a predictor of a complicated course associated with appendicitis in children.
PURPOSE: Although whole bowel irrigation (WBI) is a widely used method of bowel preparation in daily surgical practice, almost nothing is known about the histopathologic alterations caused by WBI and whether these differences have any detrimental effect on the outcome of gastrointestinal surgical procedures. Therefore, an experimental study has been conducted to evaluate and compare the effects of WBI with various solutions on the histology of gastrointestinal tract. METHODS: During the experimental procedures animals were divided into 4 groups consisting of 8 animals each as follows: group A, WBI performed by using isotonic saline solution; group B, WBI performed by using an isoosmolar solution containing polyethylene glycol (PEG); group C, WBI performed by using Lactated Ringer's solution; group D, Animals that were not irrigated but sham operations that were performed served as controls. Four hours after WBI the animals underwent laparotomy and a segment of transverse colon with intact vascular peduncle was prepared. After waiting for 30 minutes, specimens from duodenum, small intestine, large bowel, colonic segment, and liver were obtained from each animal. Histopathologically, all of the specimens were evaluated and graded by 3 parameters including congestion, edema, and inflammation. RESULTS: Although varying degrees of congestion, edema, and inflammation were encountered from all of the specimens of group A, B, and C, only slight congestion was noted in all specimens of group D. The difference between group D and other groups was statistically very significant (P < .001). When the sections from duodenums of groups were evaluated, the degree of congestion, edema, and inflammation were found to be moderate in group B, mild-moderate in group A, and mild in group C. Histopathologic examinations of specimens of the small, large bowel, and isolated colonic segment showed severe congestion, edema, and inflammation in group A, moderate-mild in group B, and mild in group C. The difference between A and B, A and C, and A and D was statistically significant (P < .01). Although severe congestion was encountered in liver specimens of group A, only mild congestion was encountered in groups B and C (P= .0001). The matched durations of irrigations and total volume of irrigation solutions were found to be not related with the difference in histopathologic findings. CONCLUSIONS: WBI has induced varying degrees of histopathologic alterations from mild to severe in the rat gastrointestinal tract. Lactated Ringer's solution and PEG solution have induced the least alterations. Therefore, WBI with Lactated Ringer's solution and PEG solution seem to be safe alternatives of mechanical bowel preparation before elective large bowel surgery. Because saline solution has caused detrimental alterations in distal gastrointestinal tract histology, WBI with saline solution seems to be unadvisable.
A 10-year-old girl has experienced 3 recurrences of hepatic artery thrombosis (HAT) after a liver transplantation. She responded to intraarterial administrations of urokinase after the first 2 attacks. However, the restoration of the arterial flow was not possible after the third attack. The child and her father were both heterozygous for factor V Leiden mutation. In addition to the technical factors, the factor V Leiden mutation should be considered as a factor that plays a role in HAT.
PURPOSE: The records of 4 patients who had necrotic bowel secondary to acute mesenteric vascular occlusion affecting various levels of mesenteric vasculature were reviewed to determine the clinical manifestations, diagnostic investigations, predisposing factors, complications, and outcome of mesenteric vascular thrombosis in children. METHODS: The medical records of the patients (3 boys, 1 girl) treated between 1981 and 1996, inclusive, for bowel infarction secondary to mesenteric vascular thrombosis, were reviewed with regard to signs and symptoms, laboratory tests, radiological investigations, surgical findings, histopathologic examinations, and outcome. RESULTS: The ages of the patients ranged between 1 and 14 years with a mean age of 8.2 years. Initial symptoms, present in all patients, were abdominal pain, abdominal distension, and tenderness. Laboratory and radiological findings including abdominal radiographs and abdominal ultrasonography were nondiagnostic. Selective superior mesenteric angiography showed complete obliteration of the superior mesenteric artery with absence of venous return in 1 case. Three patients with massive intestinal necrosis died of multiorgan failure or the complications of short bowel syndrome. Histological examination of the resected intestinal segments showed the typical findings of polyarteritis nodosa in 2 patients. One patient had a previous history of right femoral vein thrombosis, whereas 1 patient had no known underlying disorders predisposing vascular thrombosis. CONCLUSIONS: Mesenteric vascular occlusion is a rare but serious disease leading to death in children. The patients present with similar clinical signs, most frequent and important are acute abdominal pain, vomiting, and distension. Mesenteric vascular occlusion is a rare cause of acute abdomen in childhood, which requires urgent diagnosis and intervention. In suspected mesenteric vascular insufficiency, angiography should be performed followed by intraarterial thrombolytic infusion therapy in selected cases. When intestinal infarction is suspected, immediate surgical resection of compromised bowel is necessary with appropriate postoperative anticoagulation or treatment of any underlying disease.
Renal oncocytoma is one of the most unusual benign lesions, which presents as a complicated mass resulting in a diagnostic and therapeutic dilemma. A new case of renal oncocytoma in a 13-year-old boy is presented. The clinocopathologic features of this rare entity are discussed, with special emphasis on diagnosis and treatment. There are no specific presumptive clinical and laboratory findings, including tumor markers, ploidy analysis, and imaging techniques that distinguish oncocytoma from other renal masses. The most important diagnostic aid is to bear this entity in mind when a child presents with an unexplained renal mass. Frozen section biopsies followed by partial nephrectomy are mandatory for the appropriate treatment after excluding bilateral or multifocal occurrence.
INTRODUCTION: A retrospective clinical study was carried out to define the appropriate definitive operation for treating differentiated thyroid carcinoma (DTC) in children. METHODS: During a 24-year period between 1974 and 1997, 18 children younger than 16 years at presentation were histopathologically proven to have DTC in Hacettepe University Children's Hospital, Department of Pediatric Surgery. The clinical characteristics on admission, operative findings, procedures, operative complications, and follow-up results according to definitive operations were compared to find out the appropriate procedure. RESULTS: There were 15 female (83.3%) and 3 male (16.7%) patients yielding a female/male ratio of 5 and the average age being 11.6+/-2.1 years. The definitive operations were near total thyroidectomy, total thyroidectomy, near total thyroidectomy plus excision of the only palpable lymph nodes (EPLN), total thyroidectomy plus EPLN and total thyroidectomy plus modified radical neck dissection (MRND) in 3 (16.7%), 3 (16.7%), 1 (5.5%), 9 (50%), and 2 (11.1 %) patients respectively. Among the patients having undergone near total thyroidectomy, one had recurrences both in the thyroid bed and the cervical lymph nodes. In a patient undergoing almost total thyroidectomy and EPLN, recurrences involving thyroid bed, cervical lymph nodes and lungs were encountered. In 9 patients undergoing total thyroidectomy and EPLN 4 had cervical lymph node recurrences. Complications have included permanent hypoparathyroidism following total thyroidectomy and MRND. Additionally, operations for recurrences resulted in unilateral permanent recurrent laryngeal nerve palsies in two patients. CONCLUSIONS: Total thyroidectomy with evaluation of central compartment, entire jugular chain and region of lower spinal accessory nerve and removal of all identifiable lymph nodes seems to be the most appropriate surgical treatment for DTC encountered 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.
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.
Discordant Hirschsprung's disease in monozygotic twins is extremely rare. A pair of monozygotic twins with congenital deafness and discordant colonic aganglionosis is presented and genetic and prenatal environmental factors are discussed.
Explore the source record for details and available documents.
A retrospective clinical study was performed to determine the incidence, management, and outcome of gastric outlet obstruction (GOO) caused by caustic ingestion in children. Of 220 patients who sustained caustic substance ingestion and were treated at our unit between 1976 and 1996, 168 ingested alkaline substances; of these, 9 children (5.3%) developed GOO in addition to esophageal strictures. The remaining 52 patients ingested acid agents, and 2 of them (3.8%) presented with GOO without esophageal strictures. The overall incidence of corrosive GOO was 5% (n = 11). The mean age of the patients with GOO was 5.7 +/- 2.8 years (range 2-14) with a female:male ratio of 6:5. Sodium hydroxide (n = 6), potassium hydroxide (n = 3), and hydrochloric acid (n = 2) were the ingested caustic agents. The patients were subdivided into two groups according to serial endoscopic and radiologic findings: group I: moderate (dense superficial and spotty ulcerations with intact mucosa) mucosal injury with partial pyloric obstruction; and group II: severe (deep ulcerations, extreme hemorrhagic erosions, eschar formation with white plaques) mucosal injury with complete pyloric obstruction. Group I consisted of 5 patients who ingested alkali agents while group II included 6 who presented with ingestion of alkaline (n = 4) and acid (n = 2) agents. Surgical treatment included Billroth I (n = 6) operations performed in group II and Finney (n = 3) and Heineke-Mikulicz (n = 2) pyloroplasty procedures done in group I. All patients are alive without any complaints. Fiberoptic endoscopy should be the preferred method of evaluating a patient with ingestion of a corrosive agent. It determines the presence of injury and assesses the extent of damage, establishing the diagnosis and allowing therapy to be instituted immediately. Our experience revealed that substantial damage has occurred early after ingestion, and early surgical intervention has decreased the morbidity and mortality. The extent of the mucosal injury and status of the pylorus and antrum determined the type of surgical treatment. A Billroth I procedure recommended for severely injured mucosa with complete pyloric obstruction, and pyloroplasty for moderate mucosal injury associated with partially obstructed but still viable pylorus. In contrast to the current belief, alkali ingestion also has a high risk of corrosive gastric injury causing GOO, which should be considered during assessment of the injury. We emphasize that a detailed evaluation of radiologic and especially endoscopic findings is very important for determining the timing, necessity, and type of appropriate surgical treatment.
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.
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.
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.