Laparoscopic Choledochal Cyst Excision and Hepaticojejunostomy: A Case Series

PURPOSE: Choledochal cysts are congenital dilatations of the biliary tract and are generally surgically excised. Laparoscopic total excision of choledochal cysts and hepaticojejunal biliary tract reconstruction has gained acceptance among pediatric surgeons. We report our early experience with this procedure. METHODS: From May 2013 to April 2016, 10 consecutive patients (7 females and 3 males) underwent laparoscopic choledochal cyst excision and hepaticojejunostomy at our center. We retrospectively reviewed their medical records for age, sex, clinical symptoms, Todani classification, anomalous pancreaticobiliary duct union, operative time, starting day for enteral feeding, complications, and hospital stay. RESULTS: The median patient age was 22 months. Four patients were aged less than 6months, 3 of whom received prenatal diagnosis using ultrasonography. Patients presented with abdominal pain, jaundice, vomiting and fever. No abdominal mass was palpated in any patient. One patient was classified as Todani type Ia, 4 as Ic, and 5as IVa. Six patients had an anomalous pancreaticobiliary duct union. The mean operative time was 319.4 minutes. There were no surgery-related complications. Sips of water were allowed from mean postoperative day 2.4 and regular diet from mean postoperative day 3.4. The mean hospital stay was 6.5 days. CONCLUSION: Laparoscopic excision of choledochal cyst and hepaticojejunostomy in children is feasible with favorable cosmesis.

The Usefulness of Calretinin Immunohistochemistry for Rectal Suction Biopsy to Diagnose Hirschsprung’s Disease / 소아외과

PURPOSE: 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. METHODS: 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. RESULTS: 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%. CONCLUSION: We concluded that calretinin immunohistochemistry is a very useful and valuable method to diagnosis HD patient.

An Isolated Tubular Intestinal Loop in a Neonate with Type II Intestinal Atresia / 대한주산의학회잡지

An isolated tubular intestinal loop (ITIL) means an anatomical or vascular communication with rest of the bowel loop and may provide an insight into the pathogenesis of intestinal atresia. We experienced a case of an ITIL identified in omentum of a 4-day-old neonate with type-II intestinal atresia. To our knowledge, this association has never been reported in the English literature. Omental wrapping of the incompletely resorbed ischemic bowel segment may explain this phenomenon in a case of congenital intestinal atresia.

Early Experience of Laparoscopic Adhesiolysis in Children with Postoperative Intestinal Obstruction

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.

Hemorrhagic Complication of Protruding Preperitoneal Fat Presenting as an Incarcerated Inguinal Hernia in a Patient with Clinically Occult Inguinal Hernia

No abstract available.

Tuberculous Iliopsoas Muscle Abscess Associated with Multiple Intraabdominal and Thoracic Abscesses in 9-year-old Boy

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.

Comparison of Clinical Findings of Gastric Perforation between Preterm and Term Neonates / 대한주산의학회잡지

PURPOSE: The purpose of this study was to compare the clinical findings and mortality of gastric perforation between preterm and term infants. METHODS: The medical records of neonates, admitted to the neonatal intensive care unit of Dongsan Medical Center for gastric perforation between July 1992 and June 2012, were reviewed retrospectively. The admission records of clinical findings and mortality were reviewed and statistically analyzed between preterm and term infants. RESULTS: Nine infants were diagnosed with neonatal gastric perforation. Of the nine infants, the number of term infants was five and the number of male was eight. Of the four infants diagnosed with spontaneous gastric perforation, the number of preterm and term infants was three and one respectively. The anatomical location of perforation was greater curvature in all four preterm infants. However, various sites such as greater curvature (three infants) and antrum (two infants) were observed in five term infants. Mortality rate was tended to be lower in preterm infants compared to term infants, without statistical significance (25.0% vs. 40.0%, P>0.1). There was no mortality in four infants diagnosed with spontaneous gastric perforation. However, two infants diagnosed with necrotizing enterocolitis (NEC) all died. CONCLUSION: There was no significant difference in clinical findings and mortality of gastric perforation between preterm and term infants. The prognosis of spontaneous gastric perforation was good, however, mortality rate was tended to be higher in NEC than other causes (P=0.083).

MRI Findings of Long-term Survivals after Kasai Portoenterostomy

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 (p 0.427). Serum total and direct bilirubin level were 4.2mg/dL and 2.1mg/dL in patients with BDD negative group compare to 1.1mg/dL and 0.5mg/dL in BDD positive group (p 0.281). The odds ratio to have cholangitis in the patient with MRN was 2.3 and 0.53 in patient with BDD in their MRI findings. MRN in liver MRI may suggest high bilirubin level and more chance to have cholangitis, but the findings of BDD may related to low bilirubin level and less change to have cholangitis.

Effective Management of Gastroparesis with Erythromycin after Laparoscopic Nissen Fundoplication: A case report

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.

Haddad Syndrome with PHOX2B Gene Mutation in a Korean Infant

Congenital central hypoventilation syndrome with Hirschsprung's disease, also known as Haddad syndrome, is an extremely rare disorder with variable symptoms. Recent studies described that congenital central hypoventilation syndrome had deep relation to the mutation of the PHOX2B gene in its diagnosis and phenotype. We report a newborn male infant with clinical manifestations of recurrent hypoventilation with hypercapnea and bowel obstruction. These clinical manifestations were compatible with congenital central hypoventilation syndrome and Hirschsprung's disease, and polyalanine 26 repeats in the PHOX2B gene supported the diagnosis of congenital central hypoventilation. We described a first case of Haddad syndrome in Korean and its clinical and genetic characteristics were discussed.

Associated Anomaly of Esophageal Atresia

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.

Hypertrophic Pyloric Stenosis Occurring in Fasting State with Gastrostomy After Surgery for Esophageal Atresia with Tracheoesophageal Fistula

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.

Late complications and current status of long-term survivals over 10 years after Kasai portoenterostomy / 대한외과학회지

PURPOSE: Whereas the Kasai portoenterostomy (KPE) is an accepted first line of surgery for bile drainage in infants with biliary atresia, its long-term effectiveness is not clear because its etiology and pathogenesis remains unknown. This study was aimed to investigate the late complications occurring in long-term survivors and the current status of living patients who survived over 10 years after KPE. METHODS: A retrospective analysis of the medical records of 32 patients who underwent KPE from 1990 to 2000 was done. We analyzed 10-year survival rates with the Kaplan-Meier method and the current status of the long-term survivors. RESULTS: The overall 10-year survival rate by Kaplan-Meier method after KPE was 76.2%. Eight (25%) patients had died, including 4 who were transplanted. Nineteen (59.4%) patients survived over 10 years. Among them, 6 (31.6%) patients had portal hypertension, and 5 (26.3%) had episodes of cholangitis. Two had intrahepatic cyst and 2 had intestinal obstruction. Six (31.6%) patients have been well without any complications. CONCLUSION: The long-term survival rate of biliary atresia is slightly improving. However, two thirds of patients suffer from various complications. One-third of survivors go on without any complication. As biliary atresia is known as a progressive inflammatory disease, careful life-long follow- up is needed in long-term survivals after KPE.

Clinical Analysis of Metachronous Inguinal Hernia in Children

PURPOSE: The purpose of this study is to identify the incidence of metachronous contralateral inguinal hernia (MCH) and how early patients visit hospital upon discovering the presence of a lump on the contralateral side after initial hernia repair. METHODS: This is a retrospective study of 2,169 patients with inguinal hernia between January 2001 and January 2010 at a single institution. We evaluated the occurrence of MCH among 1,689 consecutive unilateral inguinal hernia (UIH) patients who were treated in our department. We also analyzed and compared the time lag (number of days) between noticing the presence of hernia and hospital visit between UIH and MCH. RESULTS: A total of 102 patients with MCH underwent initial UIH repair during the study period. The incidence of MCH was 6.0% and was slightly higher in males (6.7%) than in females (3.5%). The patients with left inguinal hernia (7.3%) had higher incidence of developing MCH than those with right (5.2%). When we analyzed the patients with MCH, 69.6% of them were under age 5 and 74.6% of MCH occurred within 2 years after initial UIH repair. Seventy-three percent of the patients with MCH, whose time lag was over one month when the first episode of UIH occurred, visited hospital later as MCH occurred. CONCLUSION: The incidence of MCH within the study period is 6.0%. It is slightly higher in males and in patients with left inguinal hernia. Most MCH occurred under age 5 and within 2 years after initial UIH repair.

Umbilical Cord Hernia Containing Vermiform Appendix Adherent to the Sac

This is a case report of umbilical cord hernia containing vermiform appendix, which was not reduced to the abdominal cavity by fibrous adhesion to the inner surface of the sac. Appendectomy is required to reduce the herniated bowel and to excise the hernia sac.

Primary segmental volvulus of the ileum mimicking meconium plug syndrome / 대한외과학회지

Primary segmental volvulus (PSV) of the ileum in neonate occurs rarely but shows an aggressive clinical course. Thus, early diagnosis is important to prevent necrosis and perforation of the intestine. We report a case of PSV of the ileum in a 2-day-old female neonate whose clinical features and radiologic findings appeared to be meconium plug syndrome.

Comparison of Clinical Finding and Mortality Rate in Neonatal Gastrointestinal Perforation due to Necrotizing Enterocolitis and Other Causes / 대한주산의학회잡지

PURPOSE: This study was conducted to compare the clinical features and outcome of neonatal gastrointestinal perforation due to necrotizing enterocolitis (NEC) and other etiologic diseases (non-NEC). METHODS: The medical records of neonates, admitted to the neonatal intensive care unit of Dongsan Medical Center for gastrointestinal perforation between January 1999 and December 2009, were reviewed retrospectively. The admission records for clinical findings and mortality were reviewed and statistically analyzed for both groups. RESULTS: Among 28 neonates, NEC group was 35.7% and the other group (intestinal atresia, malrotation, meconium peritonitis, etc) was 64.3%. The mean gestational age was significantly shorter (32.8+/-4.6 weeks vs. 36.8+/-2.7 weeks, P=0.028) and the mean diagnostic day was significantly later (16.3+/-9.7 days vs. 2.2+/-1.8 days, P=0.001) in the NEC group than that of the non-NEC group. The mortality rate was markedly higher in the NEC group (50%) than that of the non-NEC group (5.6%)(P=0.013). By simple logistic regression analysis, gestational age (OR 0.69, 95% CI: 0.51-0.95, P=0.022) and NEC (OR 17.00, 95% CI: 1.60-181.36, P=0.019) were the significant risk factors to increase the mortality rate. Multiple logistic regression analysis showed NEC (OR 7.70, 95% CI: 0.55-108.06, P=0.130) and gestational age (OR 0.79, 95% CI: 0.58-1.09, P=0.151) were not the significant independent risk factors. CONCLUSIONS: This study found that gestational age was shorter and mortality rate was higher in the NEC group than the non-NEC group. However, after multiple logistic regression analysis, NEC or lower gestational age itself did not increase the mortality rate significantly.

Conservative Management of Tracheal Injury After Endotracheal Intubation in a Neonate with Subglottic Stenosis and Esophageal Atresia with Tracheoesophageal Fistula

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.

Five-year Survival and Prognostic Factors after Kasai Portoenterostomy for Biliary Atresia

PURPOSE: Long-term survival of biliary atresia patients after Kasai's portoenterostomy is being increasingly reported. Although extended survival has been achieved for many patients, factors influencing outcome have not been defined clearly. The authors investigated 5-year survival rates and prognostic factors for survival after Kasai's portoenterostomy using univariate and multivariable methods. METHODS: The authors analyzed 5-year survival rates and prognostic factors in 56 patients who underwent Kasai's portoenterostomy during the period from 1991 to 2005 by the Kaplan-Meier model and Cox proportional hazards model, respectively. Both clinical factors and histological features of the liver and periportal fibrous mass were analyzed as prognostic factors. RESULTS: Younger ages of less than 90 days at surgery, clearance of jaundice and absence of post-Kasai cholangitis in 6 months were predictive of a favorable outcome. A significant difference between the live and the dead was found with regard to an average of 7 or more bile ductules in periportal fibrous mass in 200 HPF (P=0.013). The external diameter of the longest bile ductule in the periportal fibrous mass is not correlated to the prognosis (P=0.49). Independent factors for good prognosis were jaundice-free in 6 months and over 7 bile ductules density in periportal fibrous mass. CONCLUSION: Favorable outcome after Kasai's portoenterostomy for biliary atresia is determined by bilirubin clearance in 6 months after Kasai operation and seven or more bile ductules in periportal fibrous mass. The 5-year post-Kasai survival rate is 77.2%.

Primary Segmental Volvulus of the Ileum in 6 Day-old Premature Newborn / 대한주산의학회잡지

Primary segmental volvulus of small bowel which is not associated with intestinal malrotation is very rare in neonates and is considered as surgical emergency. Most of the primary segmental volvulus of small bowel occurs in ileum but the cause of volvulus is still not known. We report a 6 day-old-premature boy who was born weighing 1,720 g at 31th week of gestation. He was transferred to our hospital because of aggravated abdominal distension and bilious drainage through orogastric tube. Free intra-abdominal air was noted and emergency laparotomy confirmed primary segmental volvulus of the ileum with perforation of the necrotic bowel.