• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제14권1호
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• pp.52-58
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• 2011

목 적: 극소저출생체중아 태변 장폐색은 장관 영양을 지연시키고 장폐색을 유발하는 주요 원인 중 하나로 고삼투압 수용성 조영제 관장이 진단과 치료의 유용성에 대한 원칙은 아직 논란 중이다. 본 연구는 태변 장폐색을 보인 극소저출생체중아의 임상 소견을 관찰하고, 고삼투압 수용성 조영 관장 후 장폐색 증상 호전, 장관영양의 진행, 합병증 등을 관찰하고자 하였다. 방 법: 극소저출생체중아 중에 태변 장폐색으로 고삼투압 수용성 조영제 관장을 시행한 14명의 임상 소견과 방사선 소견, 장관 영양의 진행, 고삼투압 수용성 조영제 관장 치료 후 호전 여부, 고삼투압 수용성 조영제 관장과 연관된 합병증 등을 관찰하였다. 이들의 주요임상 소견을 장폐색 증상 없이 수유 진행이 가능하였던 극소저출생체중아 18명과 비교 하였다. 결 과: 1) 태변 장폐색을 보인 극소저출생체중아에서 태변 장폐색이 없었던 극소저출생체중아에 비해 낮은 5분 Apgar 점수를 보였다(p<0.05). 또한, 출생 후 마지막 태변 배출시기(일), 첫 수유 시작 시기(일), 전 장관 영양 도달 시기(일)들도 의미 있게 지연되었다(p<0.05). 2) 고삼투압 수용성 조영제 관장은 14명에서 18 회를 시행하였다. 18회 중 12회에서 조영제가 회장 말단에 도달하였으며 이 중 11회에서 관장 후 다량의 태변 배출과 함께 장폐색 증상의 호전이 있었으나, 1예는 호전이 없어 회장절개술로 태변을 제거하였다. 3) 조영제가 회장 말단에 도달 못한 5명 중 3명에서 증상의 호전이 없어 조영제 관장을 재시행 하여 조영제가 회장말단에 도달하였고 장폐색 증상이 호전되었다. 4) 고삽투압 수용성 조영제 관장과 연관된 특별한 합병증은 없었다. 결 론: 극소저출생체중아 태변 장폐색에서 고삼투압수용성 조영제 관장은 비교적 안전하게 시행할 수 있는 치료 방법이었다.

• Advances in pediatric surgery
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• 제1권1호
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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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• 제28권1호
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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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• 제8권1호
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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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• 제1권2호
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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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• 제50권12호
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• pp.1252-1256
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• 2007

태변장폐색증은 낭성섬유증에서 가장 초기에 나타나는 임상 증상으로 태변이 장관내 축적되어 회장말단의 폐색을 일으키고 복부팽만, 담즙성 구토, 태변 배출 지연이 동반된다. 낭성섬유증은 7번 염색체 장완에 위치한 막전도조절유전자의 돌연변이로 야기되며 한국인에서는 드문 질환이다. 지금까지 대한민국에서 보고된 호흡기 질환에 동반된 낭성섬유증과 달리, 저자들은 태변장폐색증에 동반된 낭성섬유증이 유전자 검사로 진단된 1례를 경험하였기에 보고하는 바이다.

• Neonatal Medicine
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• 제25권1호
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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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• 제22권5호
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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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• 제51권2호
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• pp.219-221
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• 2008

저자들은 발열과 식욕저하를 주소로 패혈증 의증으로 전원 된 27일된 신생아에서 구토와 혈변 등은 없었으나, 입원 당시 복부팽만과 단순복부촬영에서 보인 소장 확장소견으로 개복술을 실시한 결과 선천성 밴드에 의해 내탈장된 소장이 압박되어 유발된 장폐색증 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Advances in pediatric surgery
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• 제17권1호
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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.