• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.14 no.1
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• pp.52-58
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• 2011

Purpose: Mecnoium obstruction in very low birth weight infants (VLBWI), which delays enteral feeding and is one of the major causes of bowel obstruction, can be diagnosed and treated with hyperosmolar water-soluble contrast enema. The purpose of this study was to observe the clinical findings of meconium obstruction, the improvement of small bowel obstruction after contrast enema, and the complications related to the enema. Methods: Hypersolmolar water-soluble contrast enemas were performed in 14 VLBWIs with meconium obstruction. Clinical findings, radiologic findings, feeding intolerance, effectiveness, and complications of enemas were observed. Also, clinical findings related to meconium obstruction were compared with 18 VLBWIs without meconium obstruction. Results: 1) Fourteen VLBWIs with meconium obstruction had significantly lower 5 minutes Apgar scores than 18 VLBWIs without meconium obstruction (p<0.05). Moreover, the day of last meconium passing, and the day of the first trial and full enteral feeding were delayed significantly. 2) A total of 18 enemas were performed in the 14 infants. The contrast medium passed the ileocecal valve and reached the terminal ileus in 12 enemas. Of the 12 enemas, 11 were successful, but 1 infant underwent an ileotomy, even though the contrast medium reached the terminal ileum. 3) Intestinal obstruction was not relieved in three of five infants, in whom the contrast medium failed to pass the ileocecal valve. Obstruction was relieved after repeated enemas in which the contrast medium reached the terminal ileum. 4) No complications associated with water-soluble contrast enemas were observed. Conclusion: Hyperosmolar water-soluble contrast enema is considered to be safe and therapeutic for meconium obstruction in VLBWIs.

• Advances in pediatric surgery
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• v.1 no.1
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• pp.59-62
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• 1995

Meconium peritonitis is defined as an aseptic, chemical or foreign-body peritonitis caused by spill of meconium in the abdominal cavity related to the prenatal perforation of the intestine. Perforation is usually caused by obstruction from meconium ileus, intestinal atresia, stenosis, volvulus, internal hernia, congenital peritoneal bands, intussusception, or gastroschisis. Less commonly, no evidence of distal obstruction exists. Here, we present two cases of generalized meconium peritonitis of antenatal diagnosis. The first case, detected at 8 months of gestational age, had a perforation of the proximal blind pouch of jejunal atresia, associated with respiratory distress due to severe abdominal distension. This case was successfully treated with resection and anastomosis and brief period of postoperative ventilatory support. The second case had a distal ileal perforation with thick meconium in the terminal ileum. In this case, there was no dilatation of ileum proximal to the perforation site. Resection and anastomosis was performed and postoperative course was uneventful.

• Neonatal Medicine
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• v.28 no.1
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• pp.22-28
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• 2021

Purpose: Meconium obstruction of prematurity (MOP) predisposes premature infants to intestinal perforation and prolonged hospitalization if not diagnosed and treated promptly. A standard contrast enema is less effective to treat infants with distal ileal obstructions because the contrast may not reach the obstructed areas. In an effort to avoid risky surgery, we administered oral contrast media to seven clinically diagnosed patients with MOP whose obstructions were not relieved via conventional sonography-guided contrast enema. We retrospectively evaluated whether oral nonionic water-soluble contrast media relieves MOP. Methods: Seven of 67 premature infants with MOP were administered oral contrast media from June 2015 to January 2019. Patients were followed-up radiographically for bowel distention and evacuation of contrast media after oral administration. We recorded radiographic improvements, meconium evacuation, time to first feeding after oral contrast media administration, maternal history, and neonatal clinical factors. Results: We evaluated five male and two female infants. The median gestational ages and body weights at birth were 27⁺⁵ weeks and 890 g, respectively. Radiography in five infants revealed multiple distended intestinal loops without air-fluid interfaces. Two infants had gasless abdomens, in which only stomach gas was visible. Oral contrast media (median, 2.5 mL) were administered at a median age of 7 days; five infants (5/7, 71.4%) responded to this treatment. The remaining two infants, who had ileal stenosis and hypoganglionosis, were surgically managed. Five infants (5/7, 71.4%) had maternal risk factors, and two (28.6%) were small for gestational age. Conclusion: Nonionic oral water-soluble contrast medium can serve as a valuable adjunct treatment in premature infants with meconium obstruction.

• Advances in pediatric surgery
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• v.8 no.1
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• pp.54-61
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• 2002

Hirschsprung's disease (HD) is usually diagnosed in the newborn period and early infancy. The common presentation of HD in newborns consists of a history of delayed passage of meconium within the first 48 hours of life. The differential diagnosis in newborns is one of the clinical challenges of this disorder. A number of medical conditions which cause functional obstruction of the intestines are easily excluded. Neonates with meconium ileus, meconium plug syndrome, distal ileal atresia and low imperforate anus often present in a manner similar to those with HD in the first few days of life. Abdominal radiographs may help to diagnose complete obstruction such as intestinal atresia. Microcolon on contrast enema can be shown in cases with total colonic aganglionosis, ileal atresia or meconium ileus. Suction rectal biopsy or frozen section biopsy at operation is essential for differential diagnosis in such cases. HD is also considered in any child who has a history of constipation regardless of age. Older children with functional constipation may have symptoms that resemble those of HD and contrast enema is usually diagnostic. However, children with other motility disorders generally referred to as chronic idiopathic intestinal pseudoobstruction present with very similar symptoms and radiographic findings. These disorders are classified according to their histologic characteristics.; visceral myopathy, visceral neuropathy, intestinal neuronal dysplasia (IND), hypoganglionosis, immature ganglia, internal sphincter achalasia. Therefore, the workup for motility disorders should include rectal biopsy not only to confirm the presence of ganglion cells but also evaluate the other pathologic conditions.

• Advances in pediatric surgery
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• v.1 no.2
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• pp.177-180
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• 1995

A 2-day-old male (Premie, Large for gestational age(LGA), Intrauterine period(IUP) 33 weeks, birth weight 2,955 gram) was transferred with marked abdominal distention, bilious return via the orogastric tube, respiratory difficulty, and generalized edema (hydrops fetalis). He was born by cesarean section to a 36 year-old mother. Antenatal ultrasonogram at IUP 31 weeks demonstrated multiple dilated bowel loops suggestive of intestinal obstruction. There was no family history of cystic fibrosis. Simple abdominal films disclosed diffuse haziness and suspicious fine calcifications in the right lower quadrant. Barium enema demonstrated a microcolon. Sweat chloride test was not available in our institution. At laparotomy, there noted 1) a segmental volvulus of the small bowel with gangrenous change, associated with meconium peritonitis, 2) an atresia of the ileum at the base of the volvulus, and 3) the terminal ileum distal to the volvulus was narrow and impacted with rabbit pellets-like thick meconium. These findings appeared to be very similar to those of a complicated meconium ileus. In summary, the ileal atresia and meconium peritonitis seemed to be caused by antenatal segmental volvulus of the small intestine in a patient with probable meconeum ileus.

• Clinical and Experimental Pediatrics
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• v.50 no.12
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• pp.1252-1256
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• 2007

Meconium ileus (MI) is the earliest clinical manifestation of cystic fibrosis (CF) in infants. It arises from the intraluminal accumulation of highly viscid, protein-rich meconium, typically present in the terminal ileum as a neonatal intestinal obstruction. Therefore, the clinical symptoms include abdominal distension, bilious vomiting and delayed passage of meconium. CF is caused by mutations in the transmembrane conductance regulator gene (CFTR) located in the long arm of chromosome 7. CF is common in Caucacians, but is a rare disorder in Asian countries, including Korea. We experienced a case of CF combined with MI. Compared with the previous reports of CF in Korea which presented respiratory problems, this is the first case genetically diagnosed as CF with MI during the newborn period.

• Neonatal Medicine
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• v.25 no.1
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• pp.37-43
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• 2018

Purpose: Meconium-related ileus is common in preterm infants. Without proper management, it can cause necrotizing enterocolitis and perforation requiring emergent operation. This study was conducted to describe the efficacy and safety of ultrasound-guided Gastrografin enema at bedside for preterm infants with meconium-related ileus. Methods: Between March 2013 and December 2014, this study enrolled preterm infants with birth weight <1,500 g, who were diagnosed with meconium-related ileus requiring ultrasound-guided Gastrografin enema refractory to glycerin or warm saline enemas. Gastrografin was infused until it passed the ileocecal valve with ultrasound guidance at bedside. Results: A total of 13 preterm infants were enrolled. Gestational age and birth weight were 28.6 weeks (range, 23.9-34.3 weeks) and 893 g (range, 610-1,440 g), respectively. Gastrografin enema was performed around postnatal day 8 (range, day 3-11). The success rate was 84.6% (11 of 13 cases). Three of these 11 infants received a second procedure, which was successful. Among 2 unsuccessful cases, one failed to pass meconium while the other required surgery due to perforation. The time required to pass meconium was $2.8{\pm}1.5hours$ (range, 1-6 hours). The time until radiographic improvement was $2.8{\pm}3.4days$ (range, 1-14 days) after the procedure. Conclusion: Ultrasound-guided Gastrografin enema at bedside as a first-line treatment to relieve meconium-related ileus was effective and safe for very low birth weight infants. We could avoid unnecessary emergent operation in preterm infants who have high postoperative morbidity and mortality. This could also avoid transporting small preterm infants to fluoroscopy suite.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.5
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• pp.487-492
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• 2019

Waardenburg syndrome (WS) type IV is characterized by pigmentary abnormalities, deafness and Hirschsprung's disease. This syndrome can be triggered by dysregulation of the SOX10 gene, which belongs to the SOX (SRY-related high-mobility group-box) family of genes. We discuss the first known case of a SOX10 frameshift mutation variant defined as c.895delC causing WS type IV without Hirschsprung's disease. This female patient of unrelated Kuwaiti parents, who tested negative for cystic fibrosis and Hirschsprung's disease, was born with meconium ileus and malrotation and had multiple surgical complications likely due to chronic intestinal pseudo-obstruction. These complications included small intestinal necrosis requiring resection, development of a spontaneous fistula between the duodenum and jejunum after being left in discontinuity, and short gut syndrome. This case and previously reported cases demonstrate that SOX10 gene sequencing is a consideration in WS patients without aganglionosis but with intestinal dysfunction.

• Clinical and Experimental Pediatrics
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• v.51 no.2
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• pp.219-221
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• 2008

Intestinal obstruction is not uncommon in infants. The common causes of intestinal obstruction in the neonatal period are Hirschsprung disease, intestinal atresia, meconium ileus, and intussusception. However, small bowel obstruction caused by a congenital band is very rare. We report a 27-day-old baby who was admitted with abdominal distension and fever. The abdominal X-ray revealed massive bowel dilatation and the contrast gastrografin enema suggested a distal small bowel obstruction. The explolaparotomy showed small bowel entrapment due to an unusual anomalous congenital band.

• Advances in pediatric surgery
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• v.17 no.1
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• pp.15-22
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• 2011

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 $1.45{\pm}0.90kg$ and $31.1{\pm}4.6$ weeks, respectively. Thirteen neonates (65 %) diagnosed with MO weighed less than 1.5 kg and 10 neonates (50 %) weighed less than 1 kg. 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 1.5 kg, the group that weighed less than 1.5 kg 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 1.5 kg 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.