The Korean Association of Pediatric Surgeons (KAPS) performed a nationwide survey on sacrococcygeal teratoma in 2018.
The authors reviewed and analyzed the clinical data of patients who had been treated for sacrococcygeal teratoma by KAPS members from 2008 to 2017.
A total of 189 patients from 18 institutes were registered for the study, which was the first national survey of this disease dealing with a large number of patients in Korea. The results were discussed at the 34th annual meeting of KAPS, which was held in Jeonju on June 21–22, 2018.
We believe that this study could be utilized as a guideline for the treatment of sacrococcygeal teratoma to diminish pediatric surgeons' difficulties in treating this disease and thus lead to better outcomes.
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Hirschsprung’s disease (HD) is a congenital intestinal disorder with absence of ganglion cells in the intestinal muscle and submucosa. Diagnosis is based on histopathological study such as H&E, and acetylcholinesterase (AchE) immunohistochemistry. Calretinin immunohistochemistry was introduced as a new diagnostic method against limitations of other staining. The aim of this study is to investigate the usefulness of calretinin immunohistochemistry for the diagnosis of HD compared to H&E and AchE.
Ten patients with HD and 22 non-HD patients were included in the study. H&E staining, AchE and calretinin immunohistochemistry were performed in all 32 patients. All slides were evaluated by same single pathologist and the diagnostic value was calculated for each H&E stain, AchE immunohistochemical staining, and calretinin immunohistochemical staining.
Calretinin method had sensitivity of 100% and specificity of 100% for diagnosis of HD. Its diagnostic accuracy was 100%. AchE staining showed 100% of specificity and 80% of sensitivity. Diagnostic accuracy of H&E staining was 56.3%.
We concluded that calretinin immunohistochemistry is a very useful and valuable method to diagnosis HD patient.
Tuberculous Iliopsoas muscle abscess is a rare manifestation in patient with extrapulmonary tuberculosis and hardly observed in developed country. Paradoxical response to anti-tuberculous medication could make difficult therapeutic decision to clinicians. The authors report a case of tuberculous iliopsoas muscle abscess with multiple intraabdominal and thoracic abscesses in 9 year-old-boy who presented paradoxical response to anti-tuberculous treatment.
The purpose of this study is to analyze the early experience of the laparoscopic adhesiolysis for the intestinal obstruction due to postoperative adhesion. Seven patients were included in this study. The median age of those patients was 13, and there were 3 males and 4 females. Previous diagnosis and surgical procedure were various in seven cases, including small bowel resection with tapering enteroplasty, Boix-Ochoa fundopl ication, Ladd's procedure with appendectomy, mesenteric tumor resection with small bowel anastomosis, ileocecal resection and anastomosis, primary gastric repair, and both high ligation. A successful laparoscopic adhesiolysis was performed in one who had high ligation for inguinal hernia and had a single band adhesion. Six out of 7 (86%) cases needed to convert open surgery due to multiple and dense type of adhesion. In conclusion, laparoscopic approach with postoperative small bowel adhesion seems safe. However, it might be prudently considered because of high rates of conversion in children.
Delayed gastric emptying (DGE)commonly occurs after Nissen fundoplication in patients with gastroesophageal reflux disease. Since the understanding of its pathogenesis is insufficient, an effective method of management has not yet been suggested. The authors report a case of a 16-year-old girl with DGE after laparoscopic Nissen fundoplication and treated with intravenous injection of low dose erythromycin.
The purpose of this study is to analyse clinical impact of specific MRI findings in liver in patients of long-term survivors after Kasai portoenterostomy (KPE). Twenty-eight patients who were underwent KPE were followed up more than 5 years. Macro-regenerative nodule (MRN) and beaded-duct dilatation (BDD) were considered as important findings in liver MRI. The association between these findings in MRI and clinical indicator, serum bilirubin level and history of cholangitis were evaluated. Sixteen patients (57.1%) were shown MRN in liver MRI. There were 14 patients(50%) whose MRI showed BDD. Serum total and direct bilirubin were 3.6mg/dL and 1.8mg/dL respectively in positive MRN group whereas 1.4mg/dL and 0.7mg/dL in negative MRN group (
The onset of hypertrophic pyloric stenosis in the postoperative course of esophageal atresia with tracheoesophageal fistula is rarely reported. The diagnosis could be delayed due to its mimicking symptoms of other postoperative complications including gastroesophageal reflux or anastomotic stricture. We present an infant who had surgery for esophageal atresia with tracheoesophageal fistula. He had never fed since birth. The infant presented with an increased amount of orogastric tube drainage and consistently distended gastric air on simple abdominal X-ray. Abdominal ultrasonography showed hypertrophic thick pyloric muscle. The diagnosis of pyloric stenosis was confirmed d is rarely reported. The diagnosis could be delayed due to its mimicking symptoms of other postoperative complications including gastroesophageal reflux or anastomotic stricture. We present an infant who had surgery for esophageal atresia with tracheoesophageal fistula. He had never fed. The infant presented with uring surgery. After pyloromyotomy, the patient's condition improved.
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This study was aimed to evaluate associated congenital anomalies in the patients with esophageal atresia with tracheoesophageal fistula (EA/TEF). Forty-two neonates with the diagnosis of EA/TEF treated over a 10 year period in a single institution were included in this study. The demography of EA/TEF was analyzed. Major associated anomalies including vertebral, anal, cardiac, renal, limb, neurologic and chromosome were reviewed and categorized. Males were slightly more dominant than females (1.47:1) and all patients had Gross type C EA/TEF. Only 19% of the patients had solitary EA/TEF without associated anomalies. Cardiac anomalies were the most common associated congenital anomaly in patients with EA/TEF (73.8%). But 47.6% were cured spontaneously or did not affect patients' life. Atrial septal defect (ASD) was the most common cardiac anomaly followed by patent ductus arteriosus (PDA) and ventricular septal defect (VSD). Among gastrointestinal anomalies (23.8%), anorectal malformations were the most frequent, 70% Vertebral and limb abnormalities accounted for 11.9% and urogenital malformations 9.5% of the anomalies in patients with EA/TEF. VACTERL associated anomalies were 23.8% and 4.8% had full VACTERL. Almost 12% of EA/TEF had neurologic anomalies. Patients with EA/TEF require preoperative evaluation including neurologic evaluation to detect anomalies not related to VACTERL. Though associated cardiac anomaly occurred in 73.8% of patients in our study, only 21.42% needed surgical correction. The authors suggesrs further studies with large numbers of patients with EA/TEF.
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Tracheal injury is a rare complication of endo-tracheal intubation. However in neonates, the rates of morbidity and mortality are high. Recommendations for treatment are based on the several reports of this injury and are individualized. Conservative management can be effective in some cases. We describe the case of a neonate who presented with subcutaneous emphysema after intubation in a neonatal intensive care unit. This patient suffered full VACTERL syndrome and had 1.7mm diameter subglottic stenosis. Conservative management resulted in no further increase in subcutaneous emphysema and after 10 days the patient was stable.
Extraosseous Ewing's sarcoma is a rare primary malignant soft tissue tumor which is histologically identical to Ewing's sarcoma. This tumor tends to involve the soft tissue of the lower extremity and paravertebral region of adolescents and young adults but particularly rare in infants. We recently experienced a case of extraosseous Ewing's sarcoma which presented in the left arm of 4 months infant.
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