Özlem Boybeyi

Common characteristics of jejunal heterotopic gastric tissue in children: a case report with review of the literature.

An 11-year-old boy underwent laparotomy for intermittent intussusceptions because of a polypoid lesion located in the proximal jejunum. The polypoid lesion was diagnosed as heterotopic gastric mucosa (HGM). Jejunal HGM is a very rare entity, and review of the literature revealed common characteristics in children with this pathologic condition. The usual presenting age is about 14 years, and the common clinical picture is intermittent intussusceptions. Jejunal HGM is usually polypoid and predominantly located within a few centimeters distal to ligament of Treitz. The aforementioned characteristics should suggest jejunal HGM be included in the differential diagnosis of jejunal polypoid lesions in children.

Cystic nephroma and localized renal cystic disease in children: diagnostic clues and management

BACKGROUND AND PURPOSE Cystic nephroma (CN) and localized renal cystic disease (LRCD) are rare benign renal entities that can occur in children. A retrospective evaluation of the experience with these conditions was performed to emphasize the importance of differential diagnosis, recognition, and selection of the proper mode of treatment. MATERIALS AND METHODS The medical records of patients treated for CN (n = 4) and LRCD (n = 1) between 1980 and 2007 were retrospectively evaluated. The age, sex, family history of renal cystic disease, presenting symptoms, physical examination findings, diagnostic radiologic imaging used, operative findings, details of the surgical procedure, histopathologic features, and outcomes were evaluated. RESULTS There were 4 cases of CN and 1 case of LRCD without a positive family history. The age and sex of patients with CN were two 1-year-old girls and two 1.5-year-old boys. The patient with LRCD was a 4.5-year-old girl. The presenting symptoms were palpable mass in patients with CN and urinary tract infection in the patient with LRCD without impairment of renal function. Intravenous pyelography (n = 2), abdominal ultrasonography (n = 4), and computed tomography (n = 4) were used. All patients underwent an operation. Nephrectomy was performed in all 4 CN cases, and total excision of the cyst was performed in the LRCD case. CONCLUSION Pediatric surgeons should be familiar with the clinical, radiologic, surgical, and histopathologic characteristics of CN and LRCD. The presumptive diagnosis of CN prompts surgical intervention. Enucleation or partial nephrectomy is the treatment of choice in suitable cases. Nephrectomy should be reserved for CN, which totally replaces the parenchyma. Conservative treatment with follow-up imaging may be considered in selected cases of LRCD that have characteristic computed tomographic criteria. Operative intervention is indicated in symptomatic cases and should be limited to simple excision of the cysts.

Hemihyperplasia-multiple lipomatosis syndrome: an underdiagnosed entity in children with asymmetric overgrowth

Hemihyperplasia can be found as an isolated abnormality, or as a predominant associated feature of an asymmetric overgrowth syndrome. This report describes 2 patients with hemihyperplasia-multiple lipomatosis syndrome. The finding of hemihyperplasia prompts careful examination for associated lipomatous lesions. Close follow-up is required because progressive growth of lipomatous lesions can be encountered as well as recurrence after excision.

Management of unusually extensive esophagogastric corrosive injuries: emergency measures and gastric reconstruction

Ingestion of a corrosive substance only rarely presents with life-threatening symptoms because of acute necrosis of the esophagus and/or stomach and necessitates emergency surgery. Once the patient is stabilized, a staged reconstruction of the alimentary tract is planned. The surgeon should be familiar with the various types of gastric reconstruction in conjunction with or without esophageal replacement. The authors report 2 illustrative cases, which presented severe symptoms after corrosive substance ingestion, to emphasize the important aspects of management of this condition. The reconstruction of the gastrointestinal tract in children is managed with a staged approach using various methods, including Hunt-Lawrence J pouch gastric substitution.

Multifocal leiomyosarcomatosis in a 6-year-old child with epidermodysplasia verruciformis and immune defect.

Leiomyosarcoma and epidermodysplasia verruciformis are rarely encountered in children. The association of either leiomyosarcoma or epidermodysplasia verruciformis with immune deficiency has previously been documented. A 6-year-old girl, who has had multifocal leiomyosarcomatosis after the previous diagnoses of epidermodysplasia verruciformis and immune defect, represents an interesting association of these features.

Caudate lobe of the liver as the only content of the umbilical cord hernia

Umbilical cord hernia (UCH) is the least common of abdominal wall defects, wherein there exists an amniotic sac only containing intestinal loops protruding through a defect with a diameter less than 4 cm (Klein 2012). A 2600 gram female newborn was referred. Her physical examination revealed an umbilical cord hernia with a diameter of 3.5 cm. There was no associated anomaly. Bowel ischemia was suspected because of purple color and urgent surgical repair was planned (Fig. 1a). When the sac was opened, there was no bowel in it. Instead, caudate lobe of the liver was the only content of the sac (Fig. 1b). The liver was dissected free from the sac and repositioned into the abdomen. She fed orally on postoperative second day, and was discharged on the sixth day uneventfully. The well-known facts about UCH are that it is the least common of abdominal wall defects, its diameter is less than 4 cm, a membrane covers it, the only content is midgut, and it is able to be repaired primarily (Haas et al. 2011; Klein 2012). When bowel ischemia is suspected because of dark purple color of the sac, the management should be done immediately. We did so in the present case; however, we were faced with an unusual content – “caudate lobe of the liver”. There are very few reports revealing liver content of UCH in the literature. It has been reported that the accessory lobe of the liver or gallbladder has been in the sac (Festen et al. 1988; Elmasalme et al. 1995). Nearly the whole liver appearing in the UCH has also been reported very rarely (Zameer et al. 2012). The present case is the first report of UCH including only the caudate lobe of the liver besides these cases. This unusual and rare content should be kept in mind in cases with umbilical cord hernia that is dark in color to avoid causing fatal liver damage. DISCLOSURE

Achalasia-like findings in a case with delayed diagnosis of H-type tracheoesophageal fistula

H-type tracheoesophageal fistula (TEF) may lead to chronic respiratory disease if the diagnosis is delayed. Long-standing fistula causes esophageal distention which is named as pneumoesophagus or megaesophagus and possibly affects the motility of the esophageal body which may also be encountered as a part of tracheoesophageal anomalies. Both dysmotility and megaesophagus may mimic achalasia radiologically and the patient may be advised an unnecessary esophagocardiomyotomy. The authors report a 15-year-old adolescent with H-type TEF who has been diagnosed during investigations for chronic respiratory disease due to presumptive diagnosis of achalasia. The authors emphasize that a complete anatomical and functional evaluation of the upper gastrointestinal tract should be done before recommending operation for achalasia in patients with chronic respiratory disease. H-type TEF should be investigated to avoid unnecessary cardiomyotomy.

A rare cause of acute abdominal pain in children: Isolated tubal torsion; a case series

Isolated tubal torsion -a rare cause of acute abdomen in children-is usually difficult to diagnose because of non-specific findings. Surgical salphingectomy is required in delayed diagnosis in most cases. Three sexual inactive adolescents diagnosed in isolated tubal torsion (ITT) were discussed for its diagnostic features and surgical management. Laboratory tests and radiological studies including ultrasonography (US), color doppler ultrasound were performed in all patients after evaluation for acute lower abdominal pain in emergency department and they underwent surgical intervention with laparotomy (n:2) and laparoscopy (n:1). One of the patients in this study had salpingectomy. Detorsion of the fallopian tube and cyst excision were performed in the remaining two patients who also had paratubal cysts. There was no recurrence in these patients during the follow-up for 3 and 2 years. The isolated tubal torsion should be kept in mind and early surgical management is essential in order to preserve fallopian tube because of its importance in fertility.

Evaluation of intraabdominal hypertension and genitofemoral nerve motor conduction.

BACKGROUND/AIM The aim of this study was to electrophysiologically evaluate the effect of increased intraabdominal pressure (IAP) on genitofemoral nerve (GFN) motor conduction. MATERIALS AND METHODS Seven Wistar albino rats were included. After anesthetization, latency and duration of GFN conduction was recorded with a needle-probe at rest. IAP was increased to 15 mmHg by insufflating atmospheric air with a percutaneous intraperitoneal needle. At 30 min of IAP, GFN motor conduction was recorded. Abdominal pressure was then increased to 20 mmHg. At 60 min, GFN motor conduction was recorded again. The consecutive recordings of latency and duration of GFN conduction (rest, 30 min, 60 min) were evaluated statistically. RESULTS There was a significant difference between latencies at rest (1.90 ± 0.22 ms), at 30 min (2.3 ± 0.36 ms), and at 60 min (2.74 ± 0.57 ms) (Friedman test, P = 0.001). The latency was significantly increased at 60 min compared to rest (post hoc Tukey test, P = 0.003). No similar difference was detected between the recordings at 30 and 60 min. The duration of GFN motor conduction showed no difference between consecutive recordings (P = 0.067). CONCLUSION Both increased and prolonged IAP causes prolonged latency of GFN conduction, probably due to a compression effect on GFN. Neuropraxial consequences of increased IAP are thought to be related to the compression effect of peripheral nerves.

Pulmonary Mucoepidermoid Carcinoma and Solid Pseudopapillary Neoplasm of the Pancreas: A Coincidence of 2 Rare Tumors in an 8-Year-Old Boy.

Primary pulmonary mucoepidermoid carcinoma (MEC) is extremely rare in children and is characterized by a mucus-secreting and squamous cell combination. Solid pseudopapillary neoplasm of the pancreas is a rare tumor of the pancreas, which is mostly seen in young women. We present the case of an 8-year-old boy previously diagnosed and treated by lung lobectomy for MEC. He was admitted to our hospital with an incidentally recognized mass at the pancreas during postoperative workout studies. Distal pancreatectomy was performed. Surgery was curative for both tumors. To the best of our knowledge, this is the first reported case of pseudopapillary neoplasm concomitant with MEC in the literature.