• Advances in pediatric surgery
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• v.3 no.2
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• pp.168-171
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• 1997

Appendiceal perforation is uncommon in the neonate. Diess reported the first case in 1908. Approximately 111 additional cases have been reported since that time. However, with exclusion of neonatal appendicitis associated with inguinal or umbilical hernias, necrotizing enterocolitis, meconium plug, and Hirschsprung's disease, there are only 36 cases of primary neonatal appendicitis. We treated a 12 days old boy with perforation of the appendix. The infant was 3000 g at birth and had a normal spontaneous vaginal delivary at 35 weeks of gestation. The mother was 31-year-old and had premature rupture of membrane. After normal feeding for the first 5 days of life, the infant had emesis of undigested milk, decreased activity and jaundice. The baby was admitted to the Pediatrics. Progressive abdominal distension, fever, decreased activity, and vomitting developed over the next six days. Erect abdominal radiography showed pneumoperitoneum. At exploratory laparotomy, a $0.8{\times}0.6$ cm sized perforation was noted at antime-senteric border of midportion of the appendix. Trasmural inflammation and the presence of ganglion cells were noticed on histology.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.20 no.1
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• pp.55-60
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• 2017

Intestinal hypoganglionosis is a rare innervation disorder that provides numerous nutritional, medical and surgical challenges. In this case report, we present a case of a newborn with intestinal hypoganglionosis leading to intestinal failure and intestinal failure-associated liver disease who responded to $Omegaven^{TM}$, a fat emulsion comprised of omega-3 fatty acids. $Omegaven^{TM}$ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with $Omegaven^{TM}$ was seen in this patient with a clear decrease in aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and complete resolution of cholestasis with a direct bilirubin of zero within two weeks of initiation of $Omegaven^{TM}$. No current guidelines for the diagnosis and management of hypoganglionosis are available. We recommend a multidisciplinary approach and the use of novel therapies such as fat emulsions composed of omega-3 fatty acids for improved patient outcomes. Appropriate compassionate use protocols should be obtained from the Food and Drug Administration prior to initiation of $Omegaven^{TM}$.

• Advances in pediatric surgery
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• v.8 no.2
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• pp.113-118
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• 2002

The etiology of several motility disorders, including persistent megacolon after definitive surgery for Hirschsprung's disease, meconium ileus which is not associated with cystic fibrosis and idiopathic megacolon, is still unclear. Interstitial cells of Cajal (ICC) are thought to modulate gut motility as gastrointestinal pace maker cells. The aim of this study was to evaluate the role of ICC in the bowel walls of the patients (n=15) who had variable motility disorders. The ICC were identified by immunohistochemical staining using an anti-C-Kit antibody and the results were compared with control specimens (n=2). The control group (G1) showed evenly distributed ICC in their bowel walls. The second group (G2, n=5) who had normal bowel movements after Duhamel procedures and the third group (G3, n=4) who had persistent megacolon after Duhamel procedures showed absent or scarcely distributed ICC in their aganglionic bowels. The ICC were immunohistochemical staining using an anti-C-Kit antibody and the results were compared with control specimens (n=2). The control group (G1) showed evenly distributed ICC in their bowel walls. The second group (G2, n=5) who had normal bowel movements after Duhamel procedures and the third group (G3, n=4) who had persistent megacolon after Duhamel procedures showed absent or scarcely distributed ICC in their aganglionic bowels. Whereas ICC were evenly distributed in the ganglionic bowels of G2, they were not seen or scarecely distributed in the ganglionic bowels of G3. Two patients (G4) who suffered from idiopathic megacolon showed absence or decrease of ICC in spite of presence of ganglion cells in their colons. Four neonates (G5) who underwent ileostomy because of meconium obstruction showed absent or markedly decreased ICC in the the colon at the time of ileostomy and the distribution of ICC was changed to a normal pattern at the time of ileostomy closure between 39-104 days of age and their bowel motility were restored after that. The results suggest that lack of ICC caused reduce motility in the ganglionic colons and it may be responsible for the development of various motility disorders. Delayed maturity of ICC may also playa role in the meconium obstruction of neinates.

• Advances in pediatric surgery
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• v.2 no.1
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• pp.26-32
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• 1996

To understand the current status of neonatal surgery in Korea, a survey was made among the 27 members of Korean Association of Pediatric Surgeons. Response rate among surgeons was 78 percent, eighteen hospitals participated in this study. Five hundred and three cases of neonatal surgical patients were analyzed. In Korea, about 50% of cases were treated at the hospital in the capital city area. Regional number of patients were closely related to the regional population. Imperforate anus(19%), atresia/stenosis of gut(12%), and Hirschsprung's diseases(12%) were sitting at the top in the list. Majority of operation was done within the first week of life, especially during the first 24 hour period. Eighty per cent was major or so called index cases. Mortality in so-called index cases was 17%. High mortality was observed in patients with diaphragmatic hernia(47%), gastrointestinal perforation(65%) and esophageal atresia(28%). Low birth weight babies showed higher mortality in gastrointestinal perforation, esophageal atresia and abdominal wall defect. These were compared to 1993 survey of Japanese Society of Pediatric Surgeons.

• Advances in pediatric surgery
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• v.7 no.1
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• pp.46-53
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• 2001

To understand the current status of neonatal surgery in Korea, a survey was made among the 36 members of the Korean Association of Pediatric Surgeons. The response rate was 75 % (26 surgeons in 20 hospitals). Five hundred fifty three neonatal surgical patients treated in 1999 were analyzed. Regional numbers of patients were closely related to the regional population in most areas. Ano-rectal malformations (17 %), pyloric stenosis (16 %), Hirschsprung's disease (13 %), atresia/stenosis of the gut (11 %), esophageal atresia (8 %) were the most common anomalies treated. The majority of operations were done within the first week of life. Seventy one per cent of cases were major life threatening or so-called neonatal index cases. Over-all mortality was 8 per cent. Higher mortality was observed in patients with diaphragmatic hernia (26 %), gastro-intestinal perforation (18 %), NEC (18 %), and esophageal atresia(14 %). Higher mortality was observed in patients with extremely low birth weight (33%) and low birth weight (18 %). Associated anomalies were observed in 20 %. Prenatal ultrasound was performed in 36 per cent with sensitivity of 20 %. Result of this study was compared to the previous report (1994) and that of Japan (1998).

• Advances in pediatric surgery
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• v.3 no.1
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• pp.41-46
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• 1997

Perforation of the gastrointestinal tract in neonatal period has been associated with a grim prognosis. Recently there has been some improvement in survival. To evaluate the remaining pitfalls in management, 19 neonatal gastrointestinal perforation cases from May 1989 to July 1996 were analysed retrospectively. Seven patients were premature and low birth weight infants. Perforation was most common in the ileum(56.3%). Mechanical or functional obstruction distal to the perforation site was identified in 7 cases; Hirschsprung's disease 3, small bowel atresia 3, and anorectal malformation 1. These lesions were often not diagnosed until operation. Five cases of necrotizing enterocolitis and 1 of muscular defect were the other causes of perforation. In six cases, the cause of the perforation was not identified. Perinatal ischemic episodes were associated in five cases. Overall mortality was 15.1%. Because a considerable number of gastrointestinal perforations resulted from distal obstruction, pediatric surgeon should be alert for early identification and intervention of gastrointestinal obstruction, particularly in patients that are premature and have a history of ischemia.

• Advances in pediatric surgery
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• v.6 no.1
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• pp.27-31
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• 2000

Fecal incontinence is not rare in children who have been operated upon for anorectal malformation, Hirschsprung's disease, and meningomyelocele. Incontinence can negatively impact on the emotional and social development of these children. Antegrade continence enema (ACE) was utilized in 9 patients with meningomyelocele from January 1998 to June 1999. Cleanliness and post-operative complications were evaluated. Three operative techniques were employed; reversed appendicocecostomy, in situ appendicostomy and neoappendicostomy with cecal flap. All of the patients achieved complete fecal cleanliness. Four patients had post-operative complications; two with stoma leakage, one with abdominal pain on irrigation and one with stoma stenosis. The leakage was minimal and the pain on irrigation improved spontaneously. The stoma stenosis required revision 6th month postoperatively. The ACE has been shown to be safe and highly effective.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.2
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• pp.137-145
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• 2020

Purpose: The Oral-anal Transit Test (OTT) is a simple method of obtaining information about colonic transit. We aim to assess the correlation of OTT with the neuromuscular integrity of the colon determined by colonic manometry (CM). Methods: All patients who had OTT followed by CM were evaluated. Less than 6 of 24 markers remaining on OTT was considered normal. CM was performed per previously published guidelines. A normal CM was defined as at least one High Amplitude Propagating Contraction progressing from the most proximal sensor through the sigmoid colon. Results: A total of 34 patients underwent both OTT and CM (44% male, age 4-18 years, mean 11.5 years, 97% functional constipation +/- soiling, Hirschsprung's Disease). Of normal and abnormal OTT patients, 85.7% (6/7) and 18.5% (5/27) respectively had normal CM. When all markers progressed to at least the sigmoid colon, this was 100% predictive against colonic inertia. Greater than 50% of patients with manometric isolated sigmoid dysfunction had markers proximal to the recto-sigmoid. Conclusion: OTT and CM are both valuable studies that assess different aspects of colonic function. OTT can be used as a screening test to rule out colonic inertia. However, the most proximal extent of remaining markers does not predict the anatomical extent of the manometric abnormality, particularly in isolated sigmoid dysfunction.

• Advances in pediatric surgery
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• v.1 no.1
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• pp.68-72
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• 1995

Segmental dilatation of the colon is a very rare disease entity of unknown etiology and may mimic Hirschsprung's disease. It is characterized by dilatation of a segment of the colon of variable length with obstruction due to lack of peristalsis in a normally innervated intestine. Recently authors experienced a case of segmental dilatation of the sigmoid colon in a 6 month-old male, who presented with severe constipation, abdominal distention, and abdominal mass since 2 months of age. Down's syndrome and congenital nystagmus were associated. Barium enema demonstrated focal dilatation of the sigmoid colon, but the rectum and descending colon proximal t o the affected colon were of normal caliber. Rectal suction biopsy with acetylcholinesterase staining was normal and anorectal manometry showed normal rectosphincteric reflex. At operation, there was a massively dilated and hypertrophied sigmoid colon with increased tortuous serosal vessels, measuring 15 cm in length and 10 cm in width. Teniae coli were identifiable in the affected segment. Frozen section biopsies at the proximal, affected, and distal colon showed ganglion cells. Descending loop colostomy was constructed initially and segmental resection and end to end colocolostomy were carried out 3 months later. Final histologic examination showed 1) normal colonic mucosa with ganglion cells, 2) prominent submucosal fibrosis and marked muscular hypertrophy, 3) unremarkable acetylcholinesterase activity and immunohistochemical findings against S-100 protein. On 8 months follow-up, he has been doing well and moves bowels 1-2 times daily.

• Advances in pediatric surgery
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• v.15 no.1
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• pp.44-51
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• 2009

This study is a retrospective analysis of 1244 cases of the inguinal hernia in children under the age of fifteen years who were operated at the department of pediatric surgery, Inje University Busan Paik Hospital from March, 1997 to February, 2007. The ratio of male to female was 3.6:1. The type of hernia was indirect in all of the cases. The hernia was on the right side in 656 cases (53.9 %), left side in 467 cases (37.5 %), and bilateral in 121 cases (9.7 %). The hernia presented most frequently in infants under age 12 months; 364 cases (29.2 %). Fifty-nine cases (21.7 %) were in female and 305 cases (31.3 %) in male. There were 428 cases (33.6 %) in 1-3 years age group, 295 cases (23.7 %) in 4-6 years, 112 cases (9.0 %) in 7-9 years, 39 cases (3.1 %) in 10-12 years and 16 (1.2 %) in 13-15 years. The content of hernia sac was small bowel (59 %), omentum (31 %) in males and the ovary and tube (54 %) and small bowel (26 %) in female. The incidence of combined operation at the time was 3.2 %, and consisting of orchiopexy (67.5 %), frenulotomy (12.5 %), appendectomy (10 %), circumcision (5 %), and fistulotomy (5%). The incidence of combined disease was 2.8 % and consisting of undescended testis, Hirschsprung's disease, idiopathic hypertrophic pyloric stenosis, imperforate anus, and congenital heart disease. After unilateral inguinal hernia repairs, contralateral hernias developed in 34 patients. The laterality of the primary site of hernias were left in 19 cases (55.8 %), and right 15 cases (44.1 %). The 936 cases (75.2 %) were operated under general anesthesia; Mask bagging 663 cases (53.2 %), endotrachea intubation 257 cases (20.6 %), and laryngeal mask 16 cases (1.2 %). The remainder 308 cases (24.7 %) were operated under regional caudal anesthesia.