Saniye Ekinci

Partial external biliary diversion for the treatment of intractable pruritus in children with progressive familial intrahepatic cholestasis: Report of two cases

Progressive familial intrahepatic cholestasis (PFIC) is a cholestatic liver disease of childhood. Pruritus secondary to increased bile salts in the serum may not respond to medical treatment. Partial external biliary diversion (PEBD), which reduces the serum bile salt level in the enterohepatic cycle, is used in the treatment of this symptom. In this study, our experience in performing this technique and the early promising results of PEBD in two children with PFIC are reported along with a review of the current literature. Partial external biliary diversion was performed by interposing a 15-cm jejunum between the gallbladder and abdominal wall. Biliary drainage through a stoma began in the fi rst postoperative day and reached 120–200 ml/day. Pruritus improved and then stopped on the 15th postoperative day, while the serum bile acid concentration also decreased. Partial external biliary diversion by jejunal interposition provides an excellent control of pruritus in children with PFIC with no adverse effects. A cholecystectomy should therefore be avoided in patients with PFIC.

Cardiac rhabdomyoma as an unusual mediastinal mass in a newborn

A newborn case with cardiac rhabdomyoma is presented to discuss the differential diagnosis of mediastinal tumors around the heart in children. The diagnosis of rhabdomyoma can be suggested by modern visualization methods such as computerized tomography, magnetic resonance imaging and echocardiography. However, surgery is clearly indicated in cases with obstruction of flow tracts, valvular dysfunction and dysrhythmia, and in cases with indefinite diagnosis, or in the absence of association with tuberous sclerosis. Cardiac rhabdomyoma should be included in the differential diagnosis of masses located adjacent to the heart in infants.

Ureteropelvic junction obstruction causes histologic alterations in contralateral kidney.

BACKGROUND/PURPOSE Ureteropelvic junction (UPJ) obstruction causes histologic alterations both in ipsilateral and contralateral kidney. Because these alterations directly affect the fate of renal damage, definition of these alterations is of utmost importance from the clinical point of view. Thus, an experimental study is designed to determine the alterations of renal histology in response to partial and complete UPJ obstruction. METHODS Fifteen adult female New Zealand rabbits were assigned randomly into 3 groups (each containing 5 rabbits) according to the degree of unilateral UPJ obstruction as group I, sham operation was performed and served as the control group; group II, partial UPJ obstruction was made; group III, complete UPJ obstruction was made. The animals in group I and II were killed after 3 weeks, and animals in group III were killed after 2 weeks. Tissue samples were prepared and processed according to routine light microscopic tissue processing. RESULTS UPJ obstruction led to glomerulosclerosis, dilatation of proximal and distal tubules of loops of Henle, and dilatation of collecting tubes consistent with necrotic and apoptotic changes in ipsilateral kidneys. Severity of these degenerative changes depended on degree of obstruction. UPJ obstruction also led to histologic alterations on the contralateral kidneys such as glomerular edema, congested blood vessels, dilated tubuli, and necrotic and apoptotic changes in epithelia, which were more prominent in group III than group II. CONCLUSIONS It is well known that compensating changes including increased blood flow and parenchymal hypertrophy occurs in contralateral kidney as a response to unilateral UPJ obstruction. However histologic findings of this study confirmed progression of parenchymal damage and presence of apoptosis in contralateral kidney for the first time.

Imaging Findings of Fetal-Neonatal Ovarian Cysts Complicated With Ovarian Torsion and Autoamputation

OBJECTIVE Large nonresolving neonatal ovarian cysts may be a risk factor for complications such as torsion, mass effect, rupture, intracystic hemorrhage, and autoamputation. Torsed cysts and autoamputated cysts can cause a diagnostic dilemma. The objective of our study was to correlate the imaging findings of intrauterine ovarian torsion and autoamputated ovaries with their pathologic findings. MATERIALS AND METHODS We retrospectively analyzed the pre- and postnatal medical records, sonographic findings, operation notes, and pathologic reports of 15 patients with ovarian torsion. All patients had complex cysts noted on postnatal sonographic examination. A complex heterogeneous ovarian cyst was defined by the presence of a fluid-debris level indicating hemorrhage within the cyst, a retracting clot, septations with or without internal echoes, calcification, and a solid component. RESULTS On ultrasound examination, four cysts had solid components, and 11 were heterogeneous and had a fluid-debris level. Calcifications were seen in two patients. The mean patient age at the time of surgery was 3.9 months. Exploratory laparotomy was performed on all patients. Torsed ovaries were identified in five patients. Ten patients had ovaries that were floating free in the peritoneal cavity at the time of surgery. Histopathologic evaluation revealed that 11 of the cysts consisted of extensive hemorrhagic, necrotic autolytic tissue with dystrophic calcification. None of the cysts contained any ovarian tissue. CONCLUSION A complex heterogeneous ovarian cyst with a fluid-debris level indicating hemorrhage is a significant sonographic hallmark for the diagnosis of ovarian torsion. A calcified abdominal mass, with or without wandering, can be an autoamputated ovary.

Childhood pneumonectomies: two decades' experience of a referral center.

PURPOSE We aimed to review the pre- and postoperative characteristics of children undergoing pneumonectomy, with special emphasis on the management of surgical complications, in comparison with the current literature findings. PATIENTS AND METHODS A total of 20 patients who underwent pneumonectomy for various etiologies from 1988 to 2011 were investigated retrospectively with respect to the presenting findings, preoperative evaluation, surgical information, postoperative follow-up, and outcome. RESULTS A total of 11 girls and 9 boys with a median age of 8 years (0.5-17 years) presented with symptoms of productive cough (n = 9), recurrent chest infections (n = 9), nonproductive cough (n = 1), respiratory distress (n = 1) with a median duration of 2 years (0.16 to 12 years). Medical histories revealed chest infection (n = 11), foreign body aspiration (n = 3), aspiration pneumonia (n = 2), tuberculosis (n = 2), caustic aspiration (n = 1), and congenital lung pathology (n = 1). Clinical evaluation provided diagnoses of bronchiectasis (n = 16), total atelectasis (n = 2), bronchopleural fistula (BPF) (n = 1), and cystic lung disease (n = 1). Pneumonectomy was performed on the right in 6 and left in 14 of the cases. Pericardial (n = 1) and esophageal (n = 1) laceration were the perioperative (11.1%), massive hemorrhage (n = 1) and chylothorax (n = 1) the postoperative (11.1%), and scoliosis (n = 1) and BPF (n = 1) the long-term (11.1%) complications. Pathological evaluation revealed bronchiectasis (n = 14), consisting of three cases with additional sign of foreign body, chronic inflammation (n = 4), atelectasis with fibrous pleural thickening (n = 1), and congenital pulmonary lymphangiectasia (n = 1). A total of 14 cases were free of symptoms and 5 had significant improvement in general condition during the follow-up, for a median duration of 2 years (1 to 10). The one with the diagnosis of Becker muscular dystrophy died 2 years after pneumonectomy because of respiratory failure. CONCLUSIONS Correct selection of indications, careful preoperative preparation with eradication of infection, meticulous performance of surgical and anesthetic techniques, early detection and management of complications, and long-term follow-up including pulmonary rehabilitation are essential parameters to reduce morbidity and mortality rates in childhood pneumonectomy. Better compensatory lung growth and improvement in development after the operation will facilitate better health and improved life quality in children.

Multiple jejunoileal perforations because of intestinal involvement of miliary tuberculosis in an infant

Infants are more susceptible to tuberculous bacilli and may develop severe extrapulmonary and miliary forms of the disease. However, miliary tuberculosis (MT) presenting with multiple intestinal perforations as an initial manifestation of the disease is extremely rare in children. The authors describe an infant who underwent emergency laparotomy because of jejunoileal perforations secondary to intestinal involvement of MT to emphasize the characteristics of the abdominal disease and the importance of controlling systemic disease to achieve a favorable outcome.

Increased maternal intraabdominal pressure alters the contractile properties of fetal rabbit bladder

BACKGROUND/PURPOSE Increased intraabdominal pressure (IAP) causes impairment of urine flow by compressing the urine-transporting structures and leads to development of various types of anatomical and functional abnormalities in the urinary system. An intrauterine experiment was conducted to determine the relationship between IAP and intraamniotic pressure (IAMNP) and the effects of increased IAMNP on the contractile properties of fetal bladder in the rabbit model. MATERIALS AND METHODS Fourteen time-mated pregnant rabbits were used. A preliminary study (n = 5, 20-day gestation) was performed to determine the relation between IAP and IAMNP. Intraabdominal pressure and IAMNP were recorded through an intraperitoneal catheter and 2 intraamniotic transducers, respectively. Basal IAP and IAMNP were recorded. Then, IAP was increased for 4 cm H(2)O in each subsequent 30-minute period until reaching 20 cm H(2)O. Control (n = 5) and experiment (n = 4) group animals underwent intraperitoneal catheter placement in the 15th day of gestation. Intraabdominal pressure was increased by intraperitoneal air insufflations from 20th day to term in the experiment group. At term, the fetal bladders were excised and the contractile activity was then recorded isometrically. Electrical field stimulation was applied, and contractile responses to carbachol and high potassium (20 mmol/L KCl) were also evaluated. RESULTS A strong relationship was found between IAMNP and IAP and defined as IAMNP = IAP x 0.8 + 2.0 (R(2) = 0.816, P = .000). Increased IAP did not change basal rhythmic activity but resulted in frequency-related electrical field stimulation responses being higher contractility responses for frequencies below 10 Hz (P < .05) and similar responses at and above 10 Hz. Bladders imposed to elevated IAP displayed greater sensitivity to carbachol with a shift to the left in the concentration-response curve. High potassium-induced contractions had a shorter rise time (P < .05) but similar contraction amplitudes and half decay times in bladders imposed to increased IAP. CONCLUSION Intraamniotic pressure is affected by IAP in pregnant rabbits in accordance with an equation (IAMNP = IAP x 0.8 + 2.0). Increased IAMNP changes contractile properties of the fetal rabbit bladder without affecting spontaneous activity and shortens the rise time of high potassium-induced contractions. Increased IAMNP also results in cholinergic hypersensitivity in fetal bladders. These results may explain the mechanism of dysfunctional voiding and abnormal bladder function observed in conditions in which IAP and/or IAMNP are elevated.

Chemical sympathectomy by 6-OH dopamine during fetal life results in inguinal testis through altering cremasteric contractility in rats

BACKGROUND/PURPOSE Androgens are proposed to influence testicular descent through modulating sympathetic tone. An experimental study was undertaken to evaluate the effects of prenatal chemical sympathectomy on testicular location associated with the alterations in contractile properties of cremaster muscles in rats. METHODS Time-mated pregnancies were started in 10 rats. Two groups, each receiving saline or 6-hydroxydopamine from day 15 to day 19 of intrauterine life were established. At 2 months of age, localization of testes were evaluated, cremaster muscles were removed, and contractile properties were studied. Twitch and tetanic contractions were recorded isometrically at 37 degrees C. Effects of verapamil, isoprenaline, and L-NNA were investigated. Results were compared through analysis of variance (ANOVA), and P values less than.05 were considered to be significant. RESULTS Both testes of all male offspring in the control group (n = 19) were in the scrotum. Six offspring among 17 subjected to 6-hydroxydopamine had undescended testes. Treatment with 6-hydroxydopamine had no effect on force-frequency relationship of cremaster muscle strips. Cremaster muscles of rats exposed to 6-hydroxydopamine had lower sensitivity to voltage-sensitive Ca++ channel blockade by verapamil (3 x 10(4) mol/L; P <.05). These muscles displayed greater contractile response to isoprenaline (10(-5) mol/L; P <.05) but not to nitric oxide synthase inhibition by N(omega)-nitro-L-arginine. Alterations in contractile properties of the muscles did not differ according to localization of testes among rats subjected to 6-hydroxydopamine. CONCLUSIONS Administration of 6-hydroxydopamine resulted in suprascrotally located testes. This localization has been associated with less exposure at sympathetic tonus. These findings support that sympathetic activity plays an important role in localization of testis.

Inguinal hernia as a rare manifestation of meconium peritonitis: report of a case.

Abstract.The usual manifestations of meconium peritonitis confined to the inguinoscrotal region are soft hydroceles, hard nodules in the scrotum, and, occasionally, calcified nodules in the wall of a hernia sac, with or without calcification on abdominal X-ray. Inguinal hernia is an extremely rare manifestation of healed meconium peritonitis. An unusual presentation of meconium peritonitis encountered during hernia repair is described herein to alert the pediatric surgeon of this possibility to avoid unnecessary abdominal exploration.

Surgical Treatment of Childhood Inflammatory Myofibroblastic Tumors.

Aim A retrospective study was performed to evaluate the clinical features, diagnostic methods, and treatment alternatives of childhood inflammatory myofibroblastic tumors (IMTs). Patients and Methods Patients who underwent surgical treatment for IMT between 2000 and 2015 were evaluated for age, sex, presenting symptoms, physical examination findings, diagnostic methods, treatment modalities, histopathologic findings, and results of surgical treatment during long‐term follow‐up. Results Eleven patients who underwent surgical treatment were included in the study. Male:female ratio was 7:4 and the mean age of the patients was 6.09 years (1‐10 years). Presenting symptoms were respiratory difficulty, cough (n = 7, 63.3%), abdominal pain, vomiting (n = 2, 18.8%), loss of body weight (n = 1, 9.09%), palpable mass (n = 1, 9.09%), and rectal bleeding (n = 1, 9.09%). Ultrasonography (n = 4, 36.3%) and computed tomography (n = 9, 81.1%) were used for diagnosis. Localizations of tumors were lungs (n = 5, 45.4%), mediastinum (n = 2, 18.1%), spleen (n = 1, 9.09%), neck (n = 1, 9.09%), colon (n = 1, 9.09%), and rectum (n = 1, 9.09%). The mean size of mass was 6.6 cm (2‐12 cm) and six patients were diagnosed with preoperative biopsy. Lung lobectomy (right lower lobe; n = 3, right middle and lower lobe; n = 2), total resection of mass with adjacent bowel (n = 2), partial splenectomy (n = 1), total resection of neck mass (n = 1), and incomplete resection (n = 2) were the choice of surgical treatment. Incomplete resection was performed in masses closely adjacent to atrium and mediastinal structures. In histopathologic evaluation, surgical margins were free of tumor in four cases, positive in six cases, and were not reported in one case. Anaplastic lymphoma kinase (ALK) positivity was detected in six cases, negative in two cases, and was not evaluated in three cases. Two cases who had residual mass with positive ALK received chemotherapy. Mean follow‐up time was 68.2 months (5 months to 12 years). During follow‐up, there was no recurrence or distant metastasis. Ten patients survived and one patient was lost to follow‐up. Conclusion IMT is a rare tumor of childhood with a spectrum of clinical findings because of variable localization. Surgical treatment is the first choice of treatment. Patients with residual mass and ALK positivity may require medical treatment. In our series, long‐term survival of patients was favorable in patients with total resection.