Santulli Enterostomy: A Considerable Method for Patients Who Require Proximal Enterostomy / 소아외과

PURPOSE@#Santulli enterostomy has been used for various surgical abdominal conditions that require temporary diversion of bowel during a neonatal period. The aim of this study was to report clinical outcomes of Santulli enterostomy and to evaluate its usefulness.@*METHODS@#Between January 2000 and December 2016, 40 neonates who underwent Santulli enterostomy were enrolled; Santulli enterostomies were performed for 25 patients without previous laparotomy (primary Santulli group) and 15 patients with previous laparotomy (secondary Santulli group).@*RESULTS@#Small bowel atresia is the first common indication of Santulli enterostomy (22/40, 55.0%), and luminal discrepancy between proximal and distal bowel was the most common determinant factor of Santulli enterostomy (17/40, 42.5%). The median age at surgery and mean birth weight were 2 days and 2,480 g respectively in the primary group, and 71 days, 2,340 g respectively in the secondary group. Operation time was significantly longer in the secondary group than the primary group (156±48 minutes vs. 224±95 minutes, p=0.019), and there was no difference in the time taken to initiation of oral feeding between the two groups. Santulli enterostomy closure was performed at median 65 days after Santulli enterostomy for primary group and 70 days for secondary group. Six complications (15.0%) were found after Santulli enterostomy, and nine complications (24.3%) after Santulli enterostomy closure (p=0.302). The incidence of complications was significantly higher in secondary group than in primary group (4.5% vs. 53.3%, p=0.001), and the reoperation rate was also significantly higher in the secondary group (4.5% vs. 46.7%, p=0.004).@*CONCLUSION@#Santulli enterostomy could be applied as a temporary enterostomy in neonatal patients with various surgical abdominal diseases. Considering the high complication rate after secondary Santulli enterostomy closure, decision making on the timing of enterostomy closure should be done with caution.

Santulli Enterostomy: A Considerable Method for Patients Who Require Proximal Enterostomy

PURPOSE: Santulli enterostomy has been used for various surgical abdominal conditions that require temporary diversion of bowel during a neonatal period. The aim of this study was to report clinical outcomes of Santulli enterostomy and to evaluate its usefulness. METHODS: Between January 2000 and December 2016, 40 neonates who underwent Santulli enterostomy were enrolled; Santulli enterostomies were performed for 25 patients without previous laparotomy (primary Santulli group) and 15 patients with previous laparotomy (secondary Santulli group). RESULTS: Small bowel atresia is the first common indication of Santulli enterostomy (22/40, 55.0%), and luminal discrepancy between proximal and distal bowel was the most common determinant factor of Santulli enterostomy (17/40, 42.5%). The median age at surgery and mean birth weight were 2 days and 2,480 g respectively in the primary group, and 71 days, 2,340 g respectively in the secondary group. Operation time was significantly longer in the secondary group than the primary group (156±48 minutes vs. 224±95 minutes, p=0.019), and there was no difference in the time taken to initiation of oral feeding between the two groups. Santulli enterostomy closure was performed at median 65 days after Santulli enterostomy for primary group and 70 days for secondary group. Six complications (15.0%) were found after Santulli enterostomy, and nine complications (24.3%) after Santulli enterostomy closure (p=0.302). The incidence of complications was significantly higher in secondary group than in primary group (4.5% vs. 53.3%, p=0.001), and the reoperation rate was also significantly higher in the secondary group (4.5% vs. 46.7%, p=0.004). CONCLUSION: Santulli enterostomy could be applied as a temporary enterostomy in neonatal patients with various surgical abdominal diseases. Considering the high complication rate after secondary Santulli enterostomy closure, decision making on the timing of enterostomy closure should be done with caution.

Relationship between Anomalous Pancreaticobiliary Ductal Union and Pathologic Inflammation of Bile Duct in Choledochal Cyst / 대한소아소화기영양학회지

PURPOSE: Choledochal cyst is a cystic dilatation of common bile duct. Although the etiology is presently uncertain, anomalous pancreaticobiliary ductal union (APBDU) is thought to be a major etiology of choledochal cyst. In this study, we analyzed the clinical and anatomical characteristics and pathologies of patients diagnosed with choledochal cyst in a single institute for 25 years. METHODS: A total of 113 patients, diagnosed with choledochal cyst and who received an operation in Severance Children's Hospital from January 1988 to May 2013, were included. Medical records were reviewed, including clinical and demographic data, surgical procedures. Abdominal ultrasonography, magnetic resonance cholangiopancreatography, and intraoperative cholangiography were used as diagnostic tools for evaluation and classification of choledochal cyst and the presence of anomalous pancreaticobiliary ductal union. Todani's classification, and relationship between APBDU and surgical pathology. RESULTS: Among 113 patients, 77 patients (68.1%) presented symptoms such as hepatitis, pancreatitis and/or cholecystitis. Eighty three patients (73.5%) had APBDU, and 94 patients (83.2%) showed inflammatory pathologic changes. APBDU, pathologic inflammation, and serological abnormalities such as hepatitis or pancreatitis showed a statistically significant correlation to one another. CONCLUSION: APBDU is thought to be one of the etiologic factors of choledochal cyst. It is related to the inflammatory changes in bile duct that can lead to the cystic dilatation.

Clinical Characteristics and Management of Benign Transient Non-Organic Ileus of Neonates: A Single-Center Experience

PURPOSE: The term benign transient non-organic ileus of neonates (BTNIN) is applied to neonates who present symptoms and plain radiographic findings of Hirschsprung's disease, but do not have aganglionic bowel and are managed well by conservative treatment. It can often be difficult to diagnose BTNIN because its initial symptoms are similar to those of Hirschsprung's disease. The aim of this study is to evaluate the clinical characteristics and proper treatment of BTNIN. MATERIALS AND METHODS: A retrospective review was made on the clinical data of 19 neonates who were treated for BTNIN between January 2008 and December 2011 at a single facility. RESULTS: Abdominal distension occurred in every patient (19/19). Other common symptoms included emesis (5/19), explosive defecation (5/19), and constipation (4/19). The vast majority of patients (15/19) experienced the onset of symptoms between 2 and 4 weeks of age. Radiograph findings from all of the patients were similar to Hirschsprung's disease. A barium study showed a transition zone in 33.4% (6/18) of the patients. However, rectal biopsy revealed ganglion cells in the distal rectum in 88.2% (15/17) of the patients, and anorectal manometry showed a normal rectoanal inhibitory reflex in 90% (9/10). All patients responded well to conservative treatment. Symptoms disappeared at the mean age of 4.9+/-1.0 months, and the abdominal radiographs normalized. CONCLUSION: BTNIN had an excellent outcome with conservative treatment, and must be differentiated from Hirschsprung's disease. A rectal biopsy and anorectal manometry were useful diagnostic tools in the differential diagnosis.

Feasibility of a Laparoscopic Approach for Generalized Peritonitis from Perforated Appendicitis in Children

PURPOSE: This study evaluated the feasibility of a laparoscopic approach in children with generalized peritonitis secondary to perforated appendicitis. MATERIALS AND METHODS: We retrospectively analyzed the medical records of patients who underwent laparoscopic appendectomy with drainage for generalized peritonitis secondary to perforated appendicitis at our hospital between September 2001 and April 2012. Laparoscopic outcomes were compared with outcomes of an open method for perforated appendicitis. RESULTS: Ninety-nine patients underwent laparoscopic appendectomy (LA) for generalized peritonitis from perforated appendicitis, and 87 patients underwent open appendectomy (OA) for perforated appendicitis. Wound infection was more common in the OA group (12.6%) than in the LA group (4.0%; p=0.032). The incidence of intestinal obstruction during long-term follow-up was significantly higher in the OA group (4.6% vs. 0.0% in the LA group; p=0.046). LA was possible in most patients for whom LA was attempted, with a conversion rate of 10.8%. Conversion to OA was affected by the preoperative duration of symptoms and the occurrence of intraoperative complications. CONCLUSION: LA is feasible for use in children with generalized peritonitis from perforated appendicitis, with reasonable open conversion and perioperative complication rates comparable to those of the OA group.

Minimally invasive surgery in infants with congenital diaphragmatic hernia: outcome and selection criteria / 대한외과학회지

PURPOSE: The aim of the study was to determine clinical indications for performing minimally invasive surgery (MIS) with acceptable results by reviewing our experience in congenital diaphragmatic hernia (CDH) repair and comparing outcomes of MIS with open surgery. METHODS: Medical records of patients who underwent CDH repair were reviewed retrospectively between January 2008 and December 2012, and outcomes were compared between MIS and open repair of CDH. RESULTS: From 2008 to 2012, 35 patients were operated on for CDH. Among these patients, 20 patients underwent open surgery, and 15 patients underwent MIS. Patients with delayed presentations (60.0% [9/15] in the MIS group vs. 20.0% [4/20] in the open surgery group; P = 0.015) and small diaphragmatic defect less than 3 cm (80.0% [12/15] in the MIS group vs. 0.0% [0/20] in the open surgery group; P < 0.001) were more frequently in the MIS group than the open surgery group. All 10 patients who also had other anomalies underwent open surgery (P = 0.002). Moreover, nine patients who needed a patch for repair underwent open surgery (P = 0.003). Patients in the MIS group showed earlier enteral feeding and shorter hospital stays. There was no recurrence in either group. CONCLUSION: CDH repair with MIS can be suggested as the treatment of choice for patients with a small sized diaphragmatic defect, in neonates with stable hemodynamics and without additional anomalies, or in infants with delayed presen tation of CDH, resulting in excellent outcomes.

Significance of Contrast Enema in One-stage Transanal Endorectal Pull-through Operation for Hirschsprung Disease

In one-stage transanal endorectal pull-through operation (TERPT) for Hirschsprung disease, preoperative evaluation by contrast enema (CE) is important tool in aspect of planning of surgical procedure as well as diagnosis. This study was to evaluate the significance of CE for identifying the extent of aganglionic bowel. A retrospective analysis was performed in 40 patients who underwent TERPT between 2003 and 2011. The authors reviewed the CE studies and their correlation with pathologic extent of aganglionosis. Total 66 contrast enemas were performed in 40 patients. Twenty patients underwent single CE, but 20 patients required multiple CEs. In single CE group, 17 had clear radiographic transition zone, but 3 had less definite transition zone. In multiple CE group, 17 patients who had equivocal finding in first or second CE had definite radiographic transition zone, but 3 patients of this group had less definite radiographic transition zones. Overall, 34 patients (85%) had clear radiographic transition zone by single or repeated CE. One (2.9%) out of 34 patients with clear radiographic transition zone had discordance between radiographic and pathologic transition zone. In contrast 4 (66.7%) out of 6 patients with equivocal radiographic transition zone had discordance between radiographic and pathologic transition zone. Observation of clear radiographic transition zone is important in preparation of TERPT, and repeated CE is helpful to reduce the discordance between radiographic and pathologic transition zone. Awareness of the possibility of discordance is also important if radiographic transitional zone is not clear.

Clinical characteristics and treatment of esophageal atresia: a single institutional experience / 대한외과학회지

PURPOSE: Treatment for esophageal atresia has advanced over several decades due to improvements in surgical techniques and neonatal intensive care. Subsequent to increased survival, postoperative morbidity has become an important issue in this disease. The aim of our study was to analyze our experience regarding the treatment of esophageal atresia. METHODS: We reviewed and analyzed the clinical data of patients who underwent surgery for esophageal atresia at Severance Children's Hospital from 1995 to 2010 regarding demographics, surgical procedures, and postoperative outcomes. RESULTS: Seventy-two patients had surgery for esophageal atresia. The most common gross type was C (81.9%), followed by type A (15.3%). Primary repair was performed in 52 patients. Staged operation was performed in 17 patients. Postoperative esophageal strictures developed in 43.1% of patients. Anastomotic leakages occurred in 23.6% of patients, and recurrence of tracheoesophageal fistula was reported in 8.3% of patients. Esophageal stricture was significantly associated with long-gap (> or =3 cm or three vertebral bodies) atresia (P = 0.042). The overall mortality rate was 15.3%. The mortality in patients weighing less than 2.5 kg was higher than in patients weighing at least 2.5 kg (P = 0.001). During the later period of this study, anastomotic leakage and mortality both significantly decreased compared to the earlier study period (P = 0.009 and 0.023, respectively). CONCLUSION: The survival of patients with esophageal atresia has improved over the years and the rate of anastomotic leakage has been significantly reduced. However, overall morbidities related to surgical treatment of esophageal atresia still exists with high incidence.

Second Serial Transverse Enteroplasty Procedure in an Infant with Extreme Short Bowel Syndrome

The serial transverse enteroplasty (STEP) procedure is a novel technique to lengthen and taper the bowel in patients with short bowel syndrome. The advantages of STEP include not only technical ease and simplicity, but also the ability to repeat the procedure. Herein, we report a case of extreme short bowel syndrome that was successfully treated by the second STEP procedure. A 3-day old newborn girl underwent STEP because of jejunal atresia with the small bowel length of 15 cm, but her bowel elongation was not enough to escape from short bowel syndrome. At the age of 6 months, she underwent a second STEP procedure. The bowel lengthening by the second STEP made her tolerable to enteral feeding with body weight gain and rescued her from short bowel syndrome. This case showed that second STEP is very helpful in treatment of extreme short bowel syndrome.

Inflammatory Myofibroblastic Tumor in a 3 Months Old Infant / 임상소아혈액종양

Benign intestinal tumors are rare in infancy, however the authors describe an inflammatory myofibroblastic tumor (IMT) of the mesentery in a 3-month-old infant who was diagnosed incidentally. During operation, huge mass which was attached to proximal jejunum was resected, from which a histological diagnosis of IMT was made. A review of the literature for this rare condition was done to delineate the natural history of this tumor. Because of the risk of local recurrence and malignant transformation, IMT cases needed a long-term follow up.

High Incidence of Hiatal Hernia in Esophageal Atresia and Its Etiologic Factors

Hiatal hernia is a very rare disease in the pediatric population. However information from our esophageal atresia postoperative follow-up program has hypotheses; "Hiatal hernia may more frequently occur in postoperative esophageal atresia patients (EA group) than in the general pediatric population (GP group)" and "The tension on the esophagus after esophageal anastomosis may be an important etiologic factor of hiatal hernia in EA group". To prove the first hypotheses, we compared the incidence of hiatal hernia in the GP group with the incidence in the EA group. The Incidence in the GP group was obtained from national statistic data from Statistics Korea and Health Insurance Review and Assessment Service of Korea. The incidence in the EA group was obtained from the medical record and the imaging studies of our esophageal atresia postoperative follow-up program. To prove the second hypothesis, the presumptive risk factors for the development of hiatal hernia in EA group, such as the type of esophageal atresia, degree of esophageal gap, the stage operation and the redo-operation with resection and re-anastomosis of esophagus were analyzed statistically. The total number of patients in the EA group was ninety-nine and there were 5 hiatus hernias. The incidence of EA group (5 %) is significantly higher than incidence of GP group (0.024 %). (p=0.0001) The statistical analysis of the presumptive risk factors for hiatal hernia development in EA group failed to show any evidence of correlation between postoperative esophageal tension and the hiatal hernia. This study shows that the postoperative patients with esophageal atresia have high occurrence of hiatal hernia and should be followed up carefully to detect hiatal hernia.

Meconium Obstruction in Neonates-Clinical Characteristics and Treatment

Meconium obstruction (MO) in neonates arises from highly viscid meconium and the poor motility of the premature gut. Recently the incidence of the MO in neonates has been increasing, but, the diagnosis and treatment of this disease have not yet been clarified. Between March 2004 and April 2010, 24 neonates were treated for MO at Severance Children's Hospital. Their clinical characteristics and treatment were reviewed retrospectively. Twenty neonates were diagnosed with MO and 4 neonates were diagnosed with Hirschsprung's disease (HD). The mean birth weight and gestational age of the 20 neonates with MO were 1.45+/-0.90kg and 31.1+/-4.6 weeks, respectively. Thirteen neonates (65%) diagnosed with MO weighed less than 1.5kg and 10 neonates (50%) weighed less than 1kg. Half of the neonates with MO were treated by non-operative methods and the other half were treated by operative methods. Compared with the group that weighed over 1.5kg, the group that weighed less than 1.5kg were more frequently operated upon (61.5% vs. 28.5%), and contrast enemas were performed later and more frequently. Also the group that weighed less than 1.5kg had a higher mortality rate (15.4% vs. 0%). Three of the four neonates with HD were diagnosed with long-segment aganglionosis. In conclusion, MO occurred in very low birth weight neonates more often and must be differentiated from HD. Also, MO in very low birth weight neonates should be treated with special attention due to more a complicated clinical course.

Inflammatory Myofibroblastic Tumor in a 3 Months Old Infant / 임상소아혈액종양

Benign intestinal tumors are rare in infancy, however the authors describe an inflammatory myofibroblastic tumor (IMT) of the mesentery in a 3-month-old infant who was diagnosed incidentally. During operation, huge mass which was attached to proximal jejunum was resected, from which a histological diagnosis of IMT was made. A review of the literature for this rare condition was done to delineate the natural history of this tumor. Because of the risk of local recurrence and malignant transformation, IMT cases needed a long-term follow up.

Initial Experience of Robot-assisted Resection of Choledochal Cyst in Children

Although laparoscopic surgery for hepatobiliary disease in children is possible, it is technically challenging. In an attempt to overcome these difficulties, the da Vinci Robotic Surgical System(R) was used to facilitate the minimally invasive treatment of choledochal cyst in six children. In early consecutive three cases, we experienced three complications; a case of laparotomy conversion, a case of late stenosis of the hepaticojejunostomy, and a case of leakage from a hepaticojejunostomy. However, in the last three cases the complete resection of the choledochal cyst and Roux-en-Y hepaticojejunostomy were performed using the robotic surgical system without complication. We think robot-assisted choledochal cyst resection in children appears safe and feasible, and may increase the variety of complex procedures in pediatric surgical fields.

Anesthetic management for neonate with giant cystic hygroma involved upper airway: A case report / 대한마취과학회지

Significant differences exist between neonatal and adult airways. Anesthetic management of the airway may be challenging in neonate and young infant with large neck mass because these patients are at risk for sudden complete airway occlusion resulting in hypoventilation and hypoxemia. We experienced a 30-day-old baby presented with large cystic hygroma on the left side of neck. This mass was infiltrated in pharynx and large enough to disturb swallowing and breathing, and was not reduced despite of sclero-therapy. Therefore he was decided to get surgical removal. During the gaseous induction with sevoflurane, spontaneous respiration was maintained because difficulty was encountered with intubation. Intraoperatively, the endotracheal tube was dislodged unexpectedly because vigorous surgical traction. Postoperatively the baby was extubated 2 day after operation, and suffered from transient facial nerve palsy and continuous discharge from surgical wound. He was administered ICU for a long time.

Infantile Vulvar Abscess with a Normal Anus: A Suspicious Sign of Rectovestibular Fistula

PURPOSE: We investigated whether infantile vulvar abscesses are predictable features of rectovestibular fistula with a normal anus. MATERIALS AND METHODS: A retrospective analysis of five infants with vulvar abscesses and rectovestibular fistulae with normal anuses was performed. RESULTS: Four cases had a left vulvar abscess, and in one case the vulvar abscess was on the right side. All caregivers reported passage of stool from the vagina. The fistulae were almost uniformly located from the vestibule to the rectum above the anal dentate line, observable by visual inspection and probing under anesthesia. The first two cases were treated with division and closure of the fistulae after a diverting loop colostomy, and the remaining three cases with fistulotomy and curettage. There was no recurrence during the median follow-up period of 38 months. CONCLUSION: This unique rectovestibular fistula should be suspected in female infants with vulvar abscesses, especially when parents report passage of stool from the vagina. Fistulotomy and curettage may be an initial treatment and effective as a temporary diverting colostomy and delayed repair of the fistula.

Isolation and Identification of Respiratory Cells from Human Amniotic Fluid

Recently, amniotic fluid has gained attention as one of the potential sources for cell therapy and tissue engineering because it has characteristics of multipotent stem cells. However, current knowledge about what types of cells are naturally found in amniotic fluid is still limited. In this study, we aimed to investigate whether human amniotic fluid contains cells that have characteristics of respiratory cells. Samples of human amniotic fluid (5 mL per sample) obtained from amniocenteses were cultured with small airway growth medium (SAGM). Cells were grown until the third passage and the presence of type II alveolar cells were characterized by inverted microscopy, immunofluorescence, and reverse transcription polymerase chain reaction (RT-PCR). On inverted microscopy, cultured cells showed typical polygonal and cobblestone-like epithelial morphology. The morphology of cells was not changed after selection and passing. Immunofluorescence analysis demonstrated that the isolated cells stained positive for surfactant protein C (SPC), specific marker for type II alveolar cells. Cells also stained positive for TTF-1 protein but negative for CD 31 and vimentin. RT-PCR analysis of cells showed expression of SPC mRNA. This study has demonstrated that respiratory cells can be isolated and identified from human amniotic fluid cultured in SAGM medium. Our results may provide the basis for further investigations of amniotic fluid.

Postpyretic Gastroparesis

Gastroparesis is a clinical term for gastric dysmotility or paralysis that presents without mechanical obstruction, but with functional obstruction. Nausea, vomiting, abdominal discomfort and abdominal distension may result from the functional obstruction of gastroparesis. Gastroparesis is frequently associated with such systemic diseases as diabetic mellitus and scleroderma or with certain operations such as vagotomy. Yet gastroparesis is rarely described in older children after viral infection. The authors observed a case of gastroparesis after pyretic symptoms. We report here on this case and its clinical consequences.

A Case of Alagille Syndrome with Atresia of the Hepatic Duct / 대한소아소화기영양학회지

A two-month-old baby had acholic stool, neonatal hyperbilirubinemia and congenital heart disease. Atresia of the hepatic duct was confirmed by open cholangiography, which showed a non-opacified intrahepatic bile duct. Liver biopsy and the Kasai operation were performed. Because the liver biopsy pathology revealed a paucity of intrahepatic bile ducts, the patient was diagnosed with the Alagille syndrome. We report the case of an infant diagnosed with the Alagille syndrome with atresia of the hepatic duct.

One-Stage Operation for Cantrell's Pentalogy

Cantrell's pentalogy may be defined as a failure of fusion of the midline from the sternum to the umbilicus. Thus, this malady consists of multiple anomalies of the sternum, heart, pericardium, diaphragm and umbilicus or anterior abdominal wall. According to the degrees of each anomaly, various operations can be planned as a one-stage operation or as a multi-stage operation and then palliative or corrective operations. The authors experienced a case of Cantrell's pentalogy that consisted of a bifid sternum, ventricular septal defect, atrial septal defect, ventricular diverticulum, dextrocardia, pericardial defect, anterior diaphragmatic defect and diastasis recti; all of these problems were corrected by a one-stage operation.