Three cases of triple A syndrome (Allgrove syndrome) in pediatric surgeons' view.

Triple A syndrome, also known as Allgrove syndrome, is a rare disease, and presents mainly in children. Its cardinal symptoms are achalasia, alacrima, and adrenocorticotropic hormone (ACTH) insensitivity. We report three cases of Triple A syndrome. Our aim is to inform pediatric surgeons about the existence of this rare syndrome and to highlight the need for suspicion of alacrima and ACTH insensitivity in cases of pediatric achalasia. Triple A syndrome should be considered in patients presenting with achalasia. Alacrima should be investigated by a Schirmer test, and adrenal dysfunction should be tested in cases of suspected triple A.

Effect of probiotic supplementation on bacterial translocation in common bile duct obstruction.

OBJECTIVE: To investigate the effects of probiotics on bacterial translocation in the obstructive common bile duct with comparison to an enteral product containing arginine and glutamine. MATERIAL AND METHOD: In our study, 40 Sprague-Dawley rats each weighing 250-300 g were used. Animals in Group 1 (sham) were laparatomized and fed standard chow supplemented with physiologic saline at daily doses of 2 ml through orogastric tube for 7 days. Common bile ducts of the animals in the other groups were ligated with 3/0 silk sutures. Group 2 (control group) was fed standard chow supplemented with daily doses of 2 ml physiologic saline. Group 3 (probiotic group) was fed standard chow supplemented with a probiotic solution (Acidophilus plus) containing strains of Lactobacillus acidophilus, Bifidobacterium bifidum and Lactobacillus bulgaricus at a daily doses of 2 × 10(9) colony forming units (CFU). Group 4 (formula group) was fed only an enteral solution (Stresson Multi Fiber) containing glutamine, arginine and a medium-chain fatty acid at daily doses of 2 g/kg. At the end of the 7th day, all animals were relaparatomized, and to determine bacterial translocation, aerobic, and anaerobic cultures were obtained from the specimens of mesenteric lymph nodes, intestinal mucosa, and blood samples. Smear cultures prepared from caecum were examined to determine the number of CFU. Finally, for histological examination specimens were excised from terminal ileum, and oxidative damage was assessed in liver tissues. Afterwards all animals were killed. RESULTS: Moderately lesser degrees of bacterial translocation, and mucosal damage were seen in Groups 3, and 4 relative to Group 2 (p < 0.05). In Group 4, any difference was not seen in the number of cecal bacteria relative to baseline values, while in Group 3, significant decrease in cecal colonization was seen. Among all groups, a significant difference between levels of malondialdehyde, and glutathione was not observed. CONCLUSION: At the end of our study, we have concluded that both probiotics, and enteral diets which contain immunomodulators such as glutamine, and arginine alleviate bacterial translocation, and impairment of intestinal mucosa.

Bilateral Multifocal Hamartoma of the Chest Wall in an Infant.

BACKGROUND: Hamartoma of the thoracic wall is a rare benign tumor that occurs in infancy and can be mistaken for a malignancy due to its clinical and imaging features. Hamartomas are extrapleural soft tissue lesions that cause rib expansion and destruction and appear on imaging as cystic areas with fluid levels and calcification. They can cause scoliosis, pressure on the neighboring lung parenchyma and mediastinal displacement. While conservative treatment is recommended in asymptomatic cases, growing lesions require surgical excision. CASE REPORT: In this report, we present the imaging findings in a 3-month-old infant that presented with a firm swelling in the chest wall and was histopathologically confirmed to have a bilateral multifocal hamartoma. CONCLUSIONS: Radiological imaging methods are important for accurate diagnosis of this very rare condition that can be confused with a malignancy.

Effects of tissue plasminogen activator in experimentally induced peritonitis.

BACKGROUND: We aimed to evaluate the microbiological and immunological effects of tissue plasminogen activator (tPA) in a rat model of peritonitis. METHODS: Twenty-four male Wistar albino rats were divided equally into three groups. Peritonitis and thereafter laparotomy and partial omentectomy were performed in all rats. The control group (C) had no further treatment. The antibiotics group (A) received metronidazole and ceftriaxone. The antibiotic and tPA group (A+T) received the same antibiotics as well as tPA. For microbiological and immunological analysis, blood samples were obtained at the 24th hour, and peritoneal fluid samples were obtained at the 24th and 72nd hours. On the fifth day after surgery, all rats were sacrificed, and the macroscopic findings of the peritoneal cavity were recorded. RESULTS: The mean number of intraperitoneal abscesses was significantly higher in the control group and the lowest in the two treatment group (A+T). The levels of cytokines were not significantly different between groups. Giving tPA reduced the number and sizes of the abscesses with no significant difference in inflammatory response. CONCLUSION: In this experimental peritonitis model, it can be postulated that tPA decreased abscess formation without exaggerating the inflammatory response.

Prenatal sonographic diagnosis of multicystic congenital mesoblastic nephroma.

The authors report an unusual presentation of congenital mesoblastic nephroma as a multilocular cystic renal lesion. Prenatal sonography revealed a unilateral, encapsulated, multilocular cystic mass with solid components measuring 5.7 × 5.4 × 4.3 cm in the left renal fossa. There was no increase in vascularity and no signs of hydrops fetalis. On the forth postnatal day left-sided radical nephrectomy was performed and histopathological examination revealed cellular type congenital mesoblastic nephroma. A multicystic appearance is rare as the vast majority of prenatally diagnosed congenital mesoblastic nephroma cases presented in the literature are of the classic type with solid homogenous or heterogenous appearence.

Intrapericardial diaphragmatic hernia: report of 2 cases with prenatal diagnosis.

An intrapericardial diaphragmatic hernia is a very rare congenital abnormality, with only a few cases reported in the English literature. The triad includes a massive pericardial effusion, bilateral pulmonary compression with or without pulmonary hypoplasia, and herniation of part of the liver into the pericardium. We report 2 cases of intrapericardial diaphragmatic hernias diagnosed prenatally in our obstetrics and gynecology unit.

Mediastinal masses in children: experience with 120 cases.

Primary mediastinal malignancies are rare tumors and can originate from any mediastinal organ or tissue such as thymic, neurogenic, lymphatic, germinal, or mesenchymal. The authors reviewed all cases of primary pediatric mediastinal masses diagnosed over a 25-year period to determine the pattern of presentation, the histology, and the outcome of the surgical treatment. In this study, 120 primary pediatric mediastinal mass cases diagnosed between 1985 and 2011 are retrospectively evaluated according to their age, sex, symptoms, anatomical location, surgical treatment, and histopathological evaluation. The median age of the patients was 5.8 years. There were 34 benign and 86 malign tumors. Thirty patients were asymptomatic. Common symptoms in the patients were cough, dyspnea, fatigue, fever, abdomen pain, back pain, and neurological symptoms. According to their origins, they were presented as neurogenic tumors (38.3%), lymphomas (18.3%), undifferentiated sarcomas (15%), germ cell tumors (7.5%), and the other tumors (22%) thymic pathologies, lymphangiomas, rhabdomyosarcomas, lipomas, hemangiomas, and Wilms' tumor. Complete resection of the tumor was performed in 86 patients, partial resection of the tumor was the intervention in 11 patients. In 23 patients, biopsy was undertaken. Because of the high incidence of asymptomatic or nonspecific presentation such as the upper airway disease, the presentation of a mediastinal mass in children may be challenging. Neurogenic tumors or lymphomas are indicating surgery, if possible complete resection, for both benign and malignant conditions. Although surgery is the mainstay of therapy for most mediastinal tumors, an experienced multidisciplinary approach is necessary.

Rupture of a congenital diaphragmatic eventration in a child: report of a case.

Diaphragmatic rupture may be traumatic or may occur spontaneously. Spontaneous diaphragmatic rupture is a rare entity seen almost entirely in adults. We report here a case with spontaneous rupture of a congenital diaphragmatic evantration in a child.

Gunshot wound of the fetus.

We report here a fetus, who was delivered via cesarean section in 32th gestational week from a 37-year-old mother and sustained multiple gunshots. Post-natal evaluation revealed eight entry-exit holes and the baby was transferred to our clinic.Upon admission, peripheral pulses were nonpalpable, appropriate fluid administration and blood transfusion were conducted and further examinations revealed bladder perforation. Bladder was repaired over a suprapubic catheter and bullet holes were primarily sutured. Left foot drop was observed on follow up and the patient was discharged with no further complication. Although the maternal and the fetal morbidity and mortality rates are high in intrauterine gunshot wounds, appropriate management may provide survival as seen in our case.

A rare neonatal testicular tumor: juvenile granulosa cell tumor of infant testis: a report of 3 cases.

SUMMARY: Juvenile granulosa, the cell tumor of testis are very rarely seen in children. Radical orchiectomy is sufficient for treatment. In this study, we presented 3 infants with juvenile granulosa cell tumor in testis.

Cervical mediastinoscopy for paratracheal masses in pediatric patients.

A cytological diagnosis is essential for a definitive diagnosis in children who have paratracheal lesions. Thirteen pediatric patients were biopsied using cervical mediastinoscopy. Age, gender, preoperative diagnosis, and postoperative biopsy results and complications were reviewed. Five patients had mediastinal lesions residuing or recurring after chemotherapy for either Hodgkin disease or non-Hodgkin lymphoma. In 2 of these patients, the diagnosis was recurrent disease. Among the 8 patients presenting with a paratracheal mass or enlarged lymph nodes, histopathologic diagnosis showed tuberculosis in 3 children, Hodgkin disease in 2 children, and histiocytosis X and non-Hodgkin lymphoma 1 patient in each. This study shows that cervical mediastinoscopy gave 100% correct diagnosis for mediastinal residual malignancies or uncommon forms of mycobacterium tuberculosis with paratracheal masses.

A congenital proximal tracheoesophageal fistula 14 years after surgical repair of esophageal atresia with distal tracheoesophageal fistula.

Congenital esophageal atresia with proximal and distal tracheoesophageal fistula occurs very rarely. This report describes late diagnosis of a congenital proximal fistula in a 14-year-old girl who underwent surgical repair of a congenital esophageal atresia with distal tracheoesophageal fistula on her first day of life and suffered recurrent bouts of lower airway infections and chronic cough, with the diagnosis of asthma in later childhood.

Osteosarcoma of the rib: A rare presentation.

In children and adolescents with chest pain and dyspnea, pneumonia, pleural effusion, and empyema are the frequent causes in the differential diagnosis. Malignant tumors of the chest wall are rare and most originate from the ribs. In children, the most frequent malignant tumor of the rib is Ewing's sarcoma. Osteosarcomas of the rib are very rare. Osteosarcoma has a predilection for rapidly growing long bones including the femur, tibia and humerus in adolescents. In this paper, we present an adolescent girl who presented with chest pain and dyspnea with osteosarcoma that originated from the rib and extended to the right hemithorax.

[Fournier's gangrene after anorectal surgery in infant: Two case reports]. / Süt çocugunda anorektal cerrahi sonrasi gelisen Fournier gangreni: Iki olgu sunumu.

Necrotizing fasciitis is a life-threatening soft tissue infection characterized by progressive necrosis of the skin, subcutaneous tissues and fascia. Fournier's gangrene (FG) is a serious and aggressive form of infective necrotizing fasciitis involving perineal region and genitalia. Presently described are 2 pediatric cases of FG with widespread necrosis of surrounding tissue following anorectal surgery, causing severe septic shock. Case 1: Six-month-old female patient with anal stenosis and duplication presented at emergency clinic with fever, somnolence, irritability, and feeding difficulty. Physical examination upon admission to Intensive Care Unit (ICU) with septic shock determined she had ecchymosis in anal region. At 12fth hour after admission, lesion had become necrotic and patient was started on broad-spectrum antibiotics after surgical debridement. Cultures were negative and patient had complete recovery 2 months after admission. Case 2: Nine-month-old male patient was admitted to ICU for convulsions and sepsis eight hours after fistulectomy. Scrotal, gluteal, and perianal edema and ecchymosis were observed on physical examination. Perianal debridement and colostomy were performed, and patient was given broad-spectrum antibiotics after basic life support strategies for septic shock. Complete recovery was achieved after hyperbaric oxygen treatment for perianal lesion and patient was discharged from the hospital in third month after admission. After anorectal surgery, every patient should be observed carefully for FG. Early debridement, proper antibiotics, and hyperbaric oxygen treatment can be life-saving.

Tuberculous liver abscess in an immunocompetent child with pulmonary tuberculosis as a cause of fever of unknown origin.

Infectious diseases are the leading cause of FUO. A case of prolonged fever with hepatic and pulmonary tuberculosis as a final diagnosis is herein presented. A 4-year-old, otherwise healthy boy presented with an axillary temperature of up to 39.5ºC for the previous 3 weeks. His medical history revealed an occasional increase in body temperature up to 38.5ºC for the last 6 months. Physical examination revealed coarse breath sounds on the basal lung area. Chest X-ray showed mediastinal lymphadenomegaly and computed tomography revealed paratracheal conglomerated lymph nodes and a groundglass appearance on the right lung. There were multiple contrast-enhanced, hypoechoic nodules with central necrosis in the liver parenchyma on abdominal magnetic resonance imaging. Open liver biopsy yielded chronic granulomatous inflammation compatible with pathological findings of tuberculosis infection. The culture specimen was positive for Mycobacterium tuberculosis. The patient improved rapidly after antituberculous therapy was initiated. Tuberculosis, especially in its disseminated form, poses a distinct diagnostic challenge in cases of prolonged fever with unproven etiology, and thus persistence should be exercised in disclosing the cause of such fevers.

Laparoscopic repair of a morgagni hernia in a child: a case report.

Morgagni hernias are anomalies of the sternal insertions of the diaphragmatic bundles and represent 1% to 4% of all surgically treated diaphragmatic hernias. We present a case of a laparoscopic repair of Morgagni hernia incidentally found in a 4-year-old boy. Primary laparoscopic closure of the defect with interrupted silk sutures was performed. The patient had uneventful recovery and is asymptomatic at 2 months of follow-up. We propose that the laparoscopic approach is feasible and effective treatment of this kind of hernia.

Multiple magnet ingestion resulting in small bowel perforation: a case report.

Foreign body ingestion is a common clinical situation that is primarily diagnosed by emergency clinicians. Most foreign bodies can be evacuated without difficulty. Although rare, magnets that reach the lower intestinal tract may cause complications such as intestinal fistula formation, perforation, volvulus or appendicitis. We report herein a two-year-old girl who was admitted to our department 3 days ago with abdominal pain and non-bilious vomiting. Upon admission direct abdominal roentgenogram revealed a foreign body consisting of multiple spheric parts bound together forming a circle in the lower quadrants of the abdomen. Her family, unaware of this ingestion, stated that a magnetic toy matching the object present on the plain radiograph was lost several days ago. Surgical intervention showed a magnetic toy in the proximal part of the ileum causing multiple perforations in the intestinal wall and the neighboring mesentery. The ileal portion containing the magnet toy was seen folded over itself forming a blind loop. The patient was discharged uneventfully in the 7th postoperative day. Our case highlights a well known fact that foreign body ingestion in children may not have eye witnesses and should be taken into consideration when evaluating children with abdominal pain.

Effect of probiotic supplementation on bacterial translocation in thermal injury.

PURPOSE: To examine the effects of probiotic supplementation and enteral solutions containing glutamine and arginine on bacterial translocation (BT) and intestinal villous atrophy in thermal injury. METHODS: Forty male Sprague-Dawley rats weighing 200-250 g were divided into four groups of ten. Group 1 served as control group without thermal injury and was fed standard chow. Thermal injury was inflicted as a 30% scald burn in the other three groups. Group 2 was fed standard chow and group 3 was fed standard chow supplemented with a probiotic (Acidophilus plus) containing Bifidobacterium bifidum, Lactobacillus acidophilus, and Lactobacillus bulgaricus (2 x 10(9) CFU/day) via an orogastric tube. Group 4 was fed only an enteral diet (Stresson multifiber) containing glutamine, arginine, and medium chain triglyceride, at 1 g/kg per day amino acid and 230 kcal/kg, for 7 days before thermal injury. All the animals were killed 24 h after thermal injury, and ileal segments were resected and examined histopathologically. To evaluate BT, samples from blood, mesenteric lymph nodes, and cecal content were cultured under aerobic and anaerobic conditions. Terminal ileum specimens were histologically examined to evaluate mucosal integrity. RESULTS: Significantly less BT was seen in groups 3 and 4 than in group 2 (P < 0.001). No significant difference was found between groups 3 and 4. Histological evaluation showed significant reduction in villous atrophy in groups 3 and 4. CONCLUSION: Probiotic supplementation seems to reduce bacterial translocation and decrease intestinal mucosal atrophy in rats with thermal injury, as do enteral solutions with arginine and glutamine.

Complete bronchial rupture in a child: report of a case.

Tracheobronchial rupture due to blunt chest trauma is a rare and serious injury in children. The diagnosis is usually difficult and may be overlooked because of the variability of symptoms and findings. Fiberendoscopy is useful in children with stable tracheal or bronchial ruptures. However, in the emergency situation, fiberendoscopy may not be appropriate, and thoracotomy and primary anastomosis may be the best option.