A rare presentation of adrenal adenoma in infancy: isolated Cushing`s syndrome.

Adrenocortical tumors are rare in children. Most of these tumors present with endocrinological manifestations, majority of which with virilizing features alone or in combination with over production of other adrenal hormones. However, it is uncommon of an adrenocortical tumor to present solely with Cushing`s syndrome. In this paper we discuss the clinical presentation and management of a 5-month-old infant who had presented with Cushing`s syndrome due to a functioning adrenocortical adenoma without androgen and mineralocorticoid excess, and made a brief review on the clinical and histopathological characteristics of adrenocortical tumors.

Spleen Salvaging Treatment Approaches in Non-parasitic Splenic Cysts in Childhood.

The aim of this study was to evaluate our experience with primary non-parasitic splenic cysts (NPSC) which are relatively rare in children and consist almost exclusively of single case reports or small case series in the literature. The medical records of all patients who presented to our clinic with NPSC between 2005 and 2015 were evaluated retrospectively. There were 22 children whose ages ranged from 2 months to 14 years (mean 9.2 ± 4.7 years). The size of the cysts was in the range of 5 to 200 mm (mean 55.4 ± 48.2 mm). Ten patients underwent surgery for splenic cysts. Partial splenectomy (n = 2), total cyst excision (either open n = 4 or laparoscopically n = 1), and total splenectomy (n = 3) were performed. The non-operated patients were asymptomatic and followed with ultrasound (US). The follow-up period in non-operated patients ranged from 6 months to 5 years (mean 2.27 ± 1.29 years). Complete regression was observed in four (33 %) non-operated patients. The regressed cyst measurements were 10, 16, 30, and 40 mm, respectively. Approximately half of the NPSC is diagnosed incidentally. Small (<5 cm) asymptomatic cysts should be under regular follow-up with US/physical examination for regression. If surgery is required, we prefer open cyst excision as it gives excellent results and preserves splenic immune function.

Intestinal malrotation needs immediate consideration and investigation.

BACKGROUND: The aim of this study was to evaluate clinical presentation, diagnostic studies, and volvulus rate and to describe the unusual clinical clues of intestinal malrotation. METHODS: A retrospective descriptive review was carried out of all patients diagnosed with intestinal malrotation between 2002 and 2014. Patients were divided into two groups: infants (≤1 year, n = 16; group 1); and children (>1 year, n = 12; group 2). Patient demographics, clinical history, symptoms, physical examination, diagnostic work-up, operative findings and early outcome were evaluated. RESULTS: Bilious vomiting was the cardinal complaint in both groups. Unusual symptoms such as respiratory insufficiency, dehydration, afebrile convulsion, and lethargy were prominent symptoms in six patients in group 1, whereas history of frequent hospitalization due to recurrent abdominal pain and feeding intolerance were prominent in six patients in group 2. Midgut volvulus was identified in 15 patients, four of whom were in group 2. Standard Ladd's procedure was done in addition to correction of volvulus. CONCLUSION: Malrotation with or without midgut volvulus is not a rare condition and should be kept in mind for any age group. Specific signs of diagnosis are not easily identified. In the case of unusual clinical presentation, diagnosis may be delayed and can result in catastrophic consequences if intestinal perfusion occurs. Although midgut volvulus is seen most frequently in infants, risk and complication rate are high beyond 1 year of age as well, and can manifest as failure to thrive, food intolerance, and abdominal pain needing recurrent hospitalization. Diagnostic suspicion and interdisciplinary coordination are essential for timely diagnosis and surgical treatment.

Huge Neck Masses Causing Respiratory Distress in Neonates: Two Cases of Congenital Cervical Teratoma.

Congenital cervical teratomas are rare and usually large enough to cause respiratory distress in the neonatal period. We present two cases of congenital huge cystic neck masses in which distinguishing cervical cystic hygroma and congenital cystic teratoma was not possible through radiologic imaging techniques. Experience with the first case, which was initially diagnosed and treated as cystic hygroma by injection sclerotherapy, led to early suspicion and surgery in the second case. The masses were excised completely and histopathologic diagnoses were congenital teratoma in both patients. Our aim is to review congenital huge neck masses causing respiratory distress in early neonatal life to highlight this dilemma briefly with these interesting cases.

Choledochal cysts in children: Intrahepatic ductal dilatation does not indicate true intrahepatic biliary duct disease.

BACKGROUND/AIMS: Choledochal cysts (CCs) are rare abnormalities of the biliary tract. Presenting our clinical experience with CCs herein, we aimed to identify if intrahepatic ductal dilatation indicates true intrahepatic biliary duct disease. MATERIALS AND METHODS: We retrospectively reviewed all cases of CCs in children diagnosed at a single center (Baskent University Fac-ulty of Medicine, Department of Pediatric Surgery) institution from 2005 to 2015. RESULTS: Of 18 patients with CCs, 7 were males (39%). The age range was 3 months to 17 years (mean age, 6.2±3.8 years). Intrahepatic bile duct dilatation was detected in 13 (72%) patients by preoperative scanning. Type I, II, III, and IVA cysts were diagnosed in 13, 1, 1, and 3 patients, respectively. In all patients, total cyst excision and Roux-en-Y hepaticoenterostomy were performed. CONCLUSION: In this study, most intrahepatic ductal dilatations seen on preoperative imaging were thought to be caused by a distal obstruction, not true intrahepatic biliary duct disease. This study supports the hypothesis that preoperatively distinguishing between type I and type IVA CCs is not necessary; it does not affect the initial treatment. We consider that complete cyst excision with Roux-en-Y hepaticojejunostomy is safe and should be performed soon after diagnosis, irrespective of symptom severity to avoid future complications.

Aspiration of an interesting foreign body: Myiasis.

Myiasis is a rare condition caused by the invasion of tissues by the larvae of flies. Many cases of myiasis involving various human organs have been reported. Tracheopulmonary or intratracheal myiasis is a very unusual and aberrant form of the disease in humans. We present a case of respiratory myiasis after aspiration of larvae by a healthy 8-month-old girl, which cannot be found in the English literature.

Variations in apparent diffusion coefficient values following chemotherapy in pediatric neuroblastoma.

PURPOSE: In children the assessment of solid tumors' response to chemotherapy is based primarily on size reduction, which can be unreliable and a late marker, in the presence of necrosis. We aimed to establish whether apparent diffusion coefficient (ADC) values of childhood neuroblastomas show proportional changes in relation to chemotherapy response. METHODS: We evaluated 15 pediatric patients with abdominopelvic neuroblastomas, who had undergone MRI before and after chemotherapy. Two radiologists retrospectively analyzed all images by drawing a round uniform region-of-interest in the solid/contrast-enhancing portion of the lesions in consensus. The ADC values from pre- and postchemotherapy images were compared. RESULTS: Postchemotherapy ADC values were significantly higher than those obtained before treatment (P < 0.05, for minimum, maximum, and median ADC values). CONCLUSION: Our results support diffusion-weighted MRI as a promising noninvasive biomarker of therapeutic responses. To the best of our knowledge, this is the first report to compare diffusion- weighted imaging findings before and after chemotherapy in childhood neuroblastic tumors.

Solid variant of aneurysmal bone cyst of the rib presenting as a left intrathoracic mass without radiological bone destruction.

An aneurysmal bone cyst (ABC) is a benign but often rapidly expanding osteolytic multi-cystic osseous lesion that occurs as a primary, secondary, intra-osseous, extra-osseous, solid, or conventional lesion. A 15-year-old boy presented with a left-sided intrathoracic mass displacing the lung without bone destruction. The mass was totally resected without rib resection, and the pathological diagnosis was ABC. The clinical manifestations, etiology, management, and pathology are discussed, with a brief discussion regarding the difficulty in the preoperative differential diagnosis.

Complications of total implantable access ports and efficacy of Taurolidine-citrate lock solution against catheter-related infections.

BACKGROUND: Totally, implantable access ports (TIAPs) are used for long standing venous catheterization. This study was designed to present our experiences of the TIAPs applications and efficacy of Taurolidine-citrate lock solution (TCLS) against catheter-related infections. MATERIALS AND METHODS: We evaluated records of the 108 patients implanted with 112 TIAPs, which had been performed using heparin solution or TCLS between 2005 and 2013. RESULTS: Duration of exposure to TIAPs was 17-2051 days (median: 411 days). The primary diagnoses were solid tumours (n = 57), lymphoma (n = 23), haematologic diseases (n = 23), nephrotic syndrome (n = 4), Hirschsprung disease (n = 1). The right external jugular vein was most frequently used vascular access route (72.3%). Mechanical complications were observed in four cases. TIAPs were removed due to remission in 19 cases and infection in 19 cases. Median time from implantation and to the development of infection was 60 days. Heparin solution had been used for care in 33 ports, whereas heparin and TCLS had been used in 79 ports. Based on statistical comparison, use of TCLS was considered to be an important factor for preventing infection (P = 0.03). CONCLUSION: We consider that TCLS reduces infection prevalence so TIAPs would be used more extensively and effectively to prevent infections.

Comparative effects of different sclerosing agents used to treat rectal prolapse: an experimental study in rats.

BACKGROUND/PURPOSE: Sclerosing agents injected into the rectal submucosal area produce an inflammatory response and scar that prevent rectal prolapse. This study aimed to investigate the histopathological changes following submucosal injection of different sclerosing agents in rats. METHODS: Rats (n=35) were divided into control, sham, and five experimental groups, each treated with a different sclerosing agent: cow's milk, 30% saline solution, 30% dextrose solution, 70% ethyl alcohol, and 5% phenol in almond oil (PAO). All agents were injected into the submucosal area. After 4 weeks, all animals were sacrificed. Histopathological evaluation was performed according to a semi-quantitative fibrosis scoring system (grades 0 to 3), by using Masson trichrome and hematoxylin and eosin staining. RESULTS: Histopathological changes in the 5% phenol in almond oil group were significantly different from other groups (p=0.0001). Prominent submucosal fibrosis (grade 3), lymphatic vascular dilation, foreign body reaction, and lipogranuloma were observed in the 5% PAO group (p=0.007). No significant histopathological differences were seen between the 30% saline, 30% dextrose, and 70% ethyl alcohol groups. Significantly increased mucosal fibroblast proliferation (grade 2) was seen in 60% rats of the 30% dextrose group (p=0.026). The cow's milk and ethyl alcohol groups had mucosal erosions and congestion (grade 1) which were significantly different from the control group (p=0.024). No statically significant difference was observed between the 30% saline group and the control group. CONCLUSIONS: In this study we showed that 5% PAO can induce some histopathological changes in the submucosal area that increase the mucosal tightness of the mucosa, which are necessary for the treatment of rectal prolapse.

Different clinical presentations, diagnostic difficulties, and management of cecal duplication.

BACKGROUND: Cecal duplications (CDs) are very rare, representing 0.4% of all gastrointestinal duplications. This study evaluates the variable clinical presentations, imaging workup, and surgical management of CDs. METHODS: The records of 7 patients who underwent surgery between April 2001 and December 2011 for CD were retrospectively reviewed. Sex, age, duration of complaints, diagnostic studies, surgical intervention, and pathologic findings were recorded. RESULTS: The median age was 8 months, and mean age was 1.65 ± 2.88 years. Complaints were abdominal pain, rectal bleeding, vomiting, cough and dyspnea, and a prenatally detected cystic mass. The patients were referred with the diagnosis of appendicular abscess, necrotizing enterocolitis, gastrointestinal lymphoma, and intraabdominal cystic mass. Abdominal distention, signs of peritonitis, substernal retraction, fullness at right lower quadrant, and normal findings were detected on physical examination. Diagnostic imaging included plain abdominal radiography (7), ultrasonography (7), computed tomography (3), and magnetic resonance imaging (2). A cystic mass was reported on radiologic studies in 6 patients and appendicular abscess in 1. Cyst and cecum were resected, ileocolostomy was performed in 6 patients, and cyst excision was performed in 1. The diagnosis of duplication cyst was made by pathologic examination in all patients. CONCLUSIONS: Cecal duplications may be detected incidentally; however, they may mimic appendicular abscess, a tumor mass, or necrotizing enterocolitis. Whether cystic lesions are symptomatic, they should be excised when detected because of possible complications.

Congenital leiomyoma of the distal ileum associated with ileal atresia: a case report.

Association of distal ileal atresia with leiomyoma is a quite rare condition. Herein, we describe a newborn who underwent surgery for ileal atresia and was found to have leiomyoma on pathological examination in the atretic segment. The presence of leiomyoma may indicate a malformation during recanalization and could play a role in the etiology of intestinal atresia. Thus, some cases of atresia may be due to tumor (leiomyoma) in the bowel wall.

Xanthogranulomatous orchiepididymitis: a very uncommon cause of scrotal mass in childhood.

Xanthogranulomatous orchitis (XGO) is an extremely rare inflammatory destructive lesion of testis. We report a case of a 14-year-old boy who presented with painful right testicular mass and discharging scrotal fistulas. Serologic tumor markers were normal. Tissue destruction was profound in the initial biopsy. The antibiotic treatment did not change the course of the disease. The definitive cure was achieved by orchiectomy and excision of the fistulous extension to the scrotum. Histopathological investigation was consistent with xanthogranulomatous process. Although XGO is very rare, the basic interest in the present report is considering this entity in the broad differential diagnosis spectrum of scrotal masses in childhood.

Surgical treatment of the solid breast masses in female adolescents.

STUDY OBJECTIVE: To evaluate the histopathologic results of the excised solid breast masses in our clinic and to draw attention to breast masses in adolescents. DESIGN: A retrospective chart review study and review of the literature. SETTING: This study was conducted in Baskent University Adana Research Center between March 2003 and May 2011. PARTICIPANTS: Patients included 10 adolescent girls admitted to pediatric surgery clinic with the complaint of palpable breast mass who underwent surgical excision of the breast mass. MAIN OUTCOME MEASURES: The necessity of surgery in breast masses of female adolescents. RESULTS: 10 female patients with an average age of 14 years were operated on for breast masses. A palpable mass in the breast and social anxiety were the cardinal complaints. Two patients had a family history of breast carcinoma. One patient had a history of neuroblastoma in remission. Histopathologic examination revealed 3 juvenile fibroadenomas, 1 conventional fibroadenoma, 3 tubular adenomas, and 3 phyllodes tumors. CONCLUSIONS: Palpable breast masses in adolescents are uncommon and are usually benign. The low malignancy risk and rarity of breast masses in childhood create a controversy as how to manage breast masses. Ultrasonography is the initial imaging modality to define the characteristics of the breast mass. Excisional biopsy and histopathologic examination is essential to rule out rare, but probable malignant, lesions in adolescents in selected patients such as maternal history of breast carcinoma, previous malignancy history in the patient, big size of the mass, and no regression on follow-up.

Predictability of outcome of caustic ingestion by esophagogastroduodenoscopy in children.

AIM: To assess the necessity of esophagogastroduodenoscopy (EGD) to predict the outcome of caustic ingestion in children. METHODS: The study included 206 children who underwent EGD because of ingestion of caustic substances between January 2005 and August 2010. Retrospective analysis of data of the patients was performed. RESULTS: The male/female ratio was 1.6 and mean age was 38.1 ± 28.8 mo. The caustic substances were acidic in 72 (34.9%) cases, alkaline in 56 (27.2%), liquid household bleach in 62 (30.1%), and unknown in 16 (7.8%). Fifty-seven (27.7%) patients were symptom-free. Significant clinical findings were observed in 149 (72.3%) patients. Upper gastrointestinal endoscopy findings of esophageal injury were grade 0 in 86 (41.7%) patients, grade 1 in 49 (23.8%), grade 2a in 42 (20.4%), grade 2b in 28 (13.6%), and grade 3a in 1 (0.5%) patient. 35 patients with grade 2a, 2b, and 3a injuries underwent esophageal dilation at second week of ingestion. Esophageal stricture, which necessitated a regular dilation program developed in 13 of the aforementioned 35 patients. There is no statistically significant difference in the rate of development of esophageal stricture between the patients who ingested acidic (15.3%) and alkaline (8.9%) substances (P = 0.32). Severe gastric injury was detected in 38 (18.5%) patients. The rate of development of gastric injury was significantly higher in the acidic group (14%) than in the alkaline group (2.9%) (P = 0.001). Out of 149 patients with clinical findings, 49 (32.9%) patients had no esophageal injury and 117 (78.5%) patients had no gastric lesion. Esophageal and severe gastric injuries were detected in 20 (35.1%) and 8 (14%) of patients with no clinical findings respectively. Pyloric stenosis developed in 6 patients. Pyloric obstruction improved with balloon dilation in 2 patients. Mean hospitalization time were 1.2 ± 0.5 d for grade 0 and 2.3 ± 5 d for grade 1 and 6.3 ± 6.2 d for grade 2a and 15.8 ± 18.6 d for grade 2b. It was significantly longer for patients with grade 2a and 2b injuries (P = 0.000). CONCLUSION: Endoscopy is an effective technique for determining the presence of esophageal and gastric damage and to avoid unnecessary treatment in patients with no or mild injury.

Management of pyloric stricture in children: endoscopic balloon dilatation and surgery.

BACKGROUND: Surgical correction is the most preferred treatment modality in pyloric stricture (PS). Recently a few studies reported the experience of balloon dilation in children with PS. This study was designed to present our experiences of the management of the patients with PS with balloon dilation and corrective surgery. METHODS: The records of 14 patients who were treated with the diagnosis of PS between August 2003 and August 2011 were reviewed retrospectively. RESULTS: There were nine boys and five girls (mean age, 3.4 ± 1.7 years). The history of caustic ingestion was detected in eight patients; six of them were admitted on the day of ingestion. Two patients were admitted with nonbilious vomiting more than 2 weeks after ingestion. Four patients did not have a remarkable medical history, including caustic ingestion. They admitted with the complaint of nonbilious vomiting. PS was detected during endoscopy in two patients who had a diagnosis of peptic ulcer disease. PS was shown by barium meal study in all patients. Endoscopy was performed in all patients. Endoscopic balloon dilation was tried in 12 patients. Overall eight patients required surgical procedures for PS. The complaints were resolved by endoscopic balloon dilation of pylorus in the remaining six patients. CONCLUSIONS: Although endoscopic balloon dilatation for benign PS in adults is a generally accepted method of treatment, there is less experience with endoscopic balloon dilatation for PS in children. PS due to benign disorders can be effectively and successfully treated through endoscopic balloon dilatation in suitable patients. In patients with successful pyloric balloon dilatation, surgery can be avoided.

Foreign body aspiration in children: analysis of diagnostic criteria and accurate time for bronchoscopy.

OBJECTIVES: The aims of this study were to evaluate the predictive diagnostic value of history, clinical signs and symptoms, and radiological finding and to evaluate whether bronchoscopy is a safe procedure and whether it should be performed in urgent conditions. METHODS: The medical records of 191 children who underwent bronchoscopy for suspected foreign body aspiration (FBA) between 2001 and 2009 were reviewed for demographic data, radiological studies, and bronchoscopic findings retrospectively. RESULTS: There were 117 male and 74 female patients. Their ages ranged from 2 months to 14 years. The major complaints were paroxysmal cough and respiratory insufficiency. Foreign body aspiration was confirmed in 123 patients (65%). Of 106 patients who were admitted in the first 24 hours, FBA was confirmed in 75 patients. Of 116 patients with a definite history of witnessed FBA, 87 patients (75%) were found to have positive bronchoscopic findings. Of 46 patients who had prolonged history of recurrent pulmonary infections, allergic asthma, or bronchiolitis, 31 (67.4%) were found to have FBs. Foreign bodies were frequently organic. Nineteen patients required urgent bronchoscopic evaluation. Sensitivity and specificity for each diagnostic criterion were as follows: clinical history (63% and 32%); symptoms (68% and 53%); physical examination findings (70.5% and 63%); radiological findings (73% and 68%); and the triad of cough, wheezing, and diminished breath sound (88% and 51%), respectively. There was a positive correlation between the presence of wheezing and FB-positive patients. CONCLUSIONS: Although there are no specific symptoms and signs to make a clear-cut diagnosis, history of witnessed FBA, admittance within 24 hours from the beginning of respiratory symptoms, and wheezing are proper indications for bronchoscopy. Bronchoscopy is a safe procedure with few and confined complications.

Diagnostic difficulties in esophageal atresia with proximal and distal tracheoesophageal fistula: a case report.

The authors report two patients with esophageal atresia and double fistula, one of whom was diagnosed intraoperatively and the other preoperatively through upper pouch contrast study. Patients with proximal and distal fistula do not present with excessive oral secretion and drooling because they can swallow the saliva/meals that reach the trachea from the proximal esophagus via the proximal fistula and then from the trachea to the distal esophagus via the distal fistula. Thus, since this clinical entity can be easily overlooked, some radiological and clinical signs that are helpful for preoperative and intraoperative diagnosis of a proximal fistula are discussed.