Pelin Oguzkurt

Increased Risk of Pyogenic Liver Abscess in Children With Papillon-Lefevre Syndrome

Among 16 with pyogenic liver abscess, two were found to have Papillon-Lefevre syndrome. The two cases are reported herein. Bacteremia from involved periodontal tissues and a possible impaired immune response could indicate an increased risk of pyogenic liver abscess among children with Papillon-Lefevre syndrome.

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.

Primary omental torsion in a 6-year-old girl

The case of a 6-year-old girl who was admitted with abdominal pain and diagnosed to have primary omental torsion through laparatomy is presented. The presence of serosangineous fluid mimicking acute appendicitis but clinically not as bad as expected is suggested to raise the suspicion of primary omental torsion.

Acute scrotum due to edidymo-orchitis associated with vasal anomalies in children with anorectal malformations

Epididymo-orchitis, an uncommon cause of acute scrotum in prepubertal boys, is infection or inflammation of epididymis and testis. Epididymo-orchitis may be associated with urinary tract infections or reflux of urine predisposed by an underlying vasal anomaly. Two infants with anorectal malformations who presented with acute scrotum are reported. The surgical exploration of the testes showed findings consistent with epididymo-orchitis. Further radiological investigations of urinary tract showed vasal anomalies in both patients. If a patient with anorectal malformation presents with acute scrotum, epididymo-orchitis should be suspected initially. Evaluations should be directed toward defining predisposing vasal anomaly, and appropriate therapeutic measures should be undertaken to prevent recurrences.

Vaginal atresia and Bardet-Biedl syndrome association: A component or a distinct entity?

Bardet-Biedl syndrome is an autosomal recessive disorder. It is characterized by cardinal anomalies including retinal dystrophy, digital malformations, mental retardation, obesity, and hypogonadism. Recently, renal anomalies also are mentioned among the cardinal signs. Although association of genital anomalies among affected boys are well known, the association of vaginal atresia and other structural genital anomalies are not mentioned among the less-common manifestations of Bardet-Biedl syndrome in girls. Two girls with Bardet-Biedl syndrome presented with hematometrocolpos in the preadolescent period and vaginal atresia was diagnosed. After surgical treatment and extended hospitalization, uncontrolled sepsis resulted in progressive renal failure and death of both patients. Vaginal atresia is often delayed or missed in the early childhood period. In girls with Bardet-Biedl syndrome, vaginal atresia or other structural genital anomalies should be evaluated more systematically during the initial diagnosis of the syndrome. In infancy, the evaluation of a child with vaginal atresia also should include the differential diagnosis of Bardet-Biedl syndrome. Vaginal atresia may either form a component of the syndrome, or girls who present with vaginal atresia in addition to other components of Bardet-Biedl syndrome might form a distinct entity.

Prenatal Diagnosis of a Mass in the Adrenal Region That Proved to be a Teratoma

A prenatally detected suprarenal cystic mass measuring 2 cm was found to have enlarged upon postnatal ultrasonography at 6 weeks of age. Magnetic resonance imaging showed a 57 x 50 mm mass in the left adrenal region displacing the kidney inferiorly. The infant underwent an adrenalectomy with total resection of the tumor, which proved on histologic examination to be a mature teratoma. Prenatally detected suprarenal masses are likely to be neuroblastoma or adrenal hemorrhage, but may be rare benign lesions such as extralobar pulmonary sequestration, bronchogenic cyst, or renal dysplasia. Although teratoma in the adrenal region is extremely rare, it should be included in the clinical and radiologic differential diagnosis of prenatally detected suprarenal masses. Total excision of the mass for histologic diagnosis is indicated.

An Uncommon Presenting Sign of Langerhans Cell Histiocytosis: Focal Perianal Lesions Without Systemic Involvement

A 3-year-old boy presented with constipation and perianal lesions resembling condyloma latum. The results of a biopsy of the perianal lesions confirmed the diagnosis of Langerhans cell histiocytosis (LCH). Although uncommon, LCH may involve the perianal region. In patients with functional constipation associated with perianal lesions that do not respond to conventional treatment, LCH should be part of the differential diagnosis. A simple biopsy of the external lesion and histologic examination of the mass are essential for diagnosis.

Multiple atresias with extensive intraluminal calcifications in a newborn with cystic fibrosis

Abstract A newborn patient with cystic fibrosis and multiple intestinal atresias demonstrated intraluminal calcifications on a plain abdominal radiograph. Cystic fibrosis may be an aetiological factor for intraluminal calcifications secondary to multiple intestinal atresias.

A Rare but Serious Complication of Ladd’s Procedure: Recurrent Midgut Volvulus

An eighteen-month-old boy who had undergone a Ladd’s procedure for malrotation in the newborn period presented with acute onset of nausea, vomiting, rectal bleeding, and confusion. Laparotomy revealed midgut volvulus, mesenteric lymphadenopathy and massive chylous ascites. Recurrent midgut volvulus following Ladd’s procedure is extremely rare but should be borne in mind in cases of persistent or recurrent gastrointestinal symptoms. Timely surgery is necessary to avoid intestinal gangrene and decrease morbidity and mortality related to consequences of midgut volvulus.

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 (Başkent 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.