Cengiz Güney

Amyand's hernia in the children: a single center experience.

BACKGROUND The presence of a vermiform appendix in an inguinal hernial sac is termed Amyands hernia. It may present as a tender inguinal or inguinoscrotal swelling, and it is often misdiagnosed as an incarcerated or strangulated hernia. METHODS Between 1998 and 2006, we have managed 564 patients with acute appendicitis, 1,090 patients with inguinal hernia, 33 patients with incarcerated inguinal hernia, and 12 patients with Amyands hernia on our pediatric surgery service. A retrospective analysis of clinical data of these patients with Amyands hernia was performed. RESULTS All patients with Amyands hernia were boys with a median age of 40 days (range, 15 days-14 months). One patients condition was diagnosed pre-operatively. All of them, therefore, underwent emergency operation with a presumptive diagnosis of either incarcerated or strangulated inguinal hernia. Operative findings included 2 normal appendices, 6 inflamed appendices, and 4 appendices with external signs of serosal inflamation of uncertain significaince in the inguinal hernial sac. Two patients with a normal appendix had hernia repair without an appendectomy. The other 10 patients with an abnormal appendix underwent an emergency open appendectomy with repair of the inguinal hernia. None of the patients developed recurrent hernia. The median postoperative follow-up period was 2.5 years. CONCLUSION In pediatric patients with Amyands hernia, the inflammatory status of the appendix can be used to determine the type of hernia repair and the operative approach. Incidental appendectomy in the case of a normal appendix is not favored by us. Treatment includes appendectomy (via the hernia sac) and hernia repair in children with an inflamed appendix.

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome in Which a Different De Novo Actg2 Gene Mutation was Detected: A Case Report

ABSTRACT Introduction: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is characterized by bladder distension without urinary tract obstruction, decreased or absent intestinal peristalsis and microcolon. Although the definitive cause remains unknown, changes in the ACTG2 gene are thought to be responsible for the intestinal and bladder hypoperistalsis. Case report: This female newborn with MMIHS had a c.532C>A /p.Arg178Ser heterozygous de novo mutation detected in the ACTG2 gene. Normal immature ganglion cells, normal calretinin punctate positivity, maintence of smooth muscle actin immunoreactivity, and decreased numbers of interstitial cells of Cajal(ICCs) were detected. Conclusion: This previously unreported c.532C>A /p.Arg178Ser heterozygous de novo mutation in the ACTG2 gene may lead to a severe form of MMIHS.

On bir yaşındaki çocukta dev seröz kistadenoma bağlı over torsiyonu: Olgu sunumu

Adolasanlarda over torsiyonu siklikla overdeki benign ya da malign tumorlerin zemininde gelisir. Bu yazida, akut karin agrisi yakinmasiyla basvuran seroz kist adenoma bagli over torsiyonu tanisi konulan on bir yasinda kiz olgu sunulmustur. Adolasanlarda overin seroz kist adenomu ender olsa da over kitleleri ve torsiyonlarinda epitelyal tumorlerin ayirici tanida akilda tutulmasi onemlidir. Cocukluk cagi over torsiyonlarinda ameliyat sirasindaki makroskopik tani ameliyatta birakilacak over dokusunun planlamasi ve izlemde onemlidir.

An Adolescent Girl with an Abdominal Mass

Figure 2. Three-dimensional computed tomography image of AVM. A 14-year-old girl presented with a 6-month history of swelling in the left lower quadrant of her abdomen. The patient was asymptomatic and her only complaint was “cosmetic distresses” due to themass. Hermedical history was unremarkable. Physical examination was normal except for the pulsatile soft mass with an approximately 6-cm diameter in the left lower region of her abdomen; this was located under the skin, and a palpable thrill was present. Laboratory tests were normal (acute phase reactants, blood count, kidney and liver tests, and urine analysis). Contrast-enhanced abdominal computed tomography revealed an arteriovenous vascular malformation (AVM) in tortuous form (Figures 1 and 2). Transcatheter embolization was performed due to the cosmetic complaints and the location of the mass in a superficial area. Six feeder arteries of the lesion were monitored via angiography, and embolization was applied to ninth, tenth, and eleventh intercostal arteries from the thoracic aorta and to 3 inferior epigastric arteries separated from the left iliac artery. The mass was reduced and the lesion completely regressed. AVMs are generally sporadic and latent during infancy and childhood but expand with an underlying thrill or bruit noted during adolescence. The hormonal changes of puberty or local trauma may trigger expansion. In the treatment of an AVM, the main goal is to obtain complete eradication

An unusual cause of abdominal ascites and acute kidney injury: Questions

A 12 year-old boy was referred with the complaint of vomiting six times in a day. History was marked by premature birth at the 28th gestational week, birth asphyxia, cerebral palsy, motor mental retardation, and epilepsy. He had been treated chronically with baclofen, diazepam, and L-Dopa and had been hospitalized for chronic constipation and rectal bleeding twice. Evaluation for metabolic diseases was reported to be normal. His parents were not relatives, and he had two healthy siblings. Physical examination was characterized by normal vital signs, impaired anthropometric development including head circumference, severe mental–motor retardation, and spasticity in all extremities. Laboratory tests revealed low hemoglobin (10.8 g/dl), low mean corpuscular volume (MCV) (74 fl), and normal kidney and liver function tests and electrolytes. Urinalysis showed microscopic hematuria; urine culture was sterile. Persistent and bloody vomiting necessitated stopping enteral feeding and placement of a nasogastric tube. Abdominal ultrasonography could not be interpreted