• Advances in pediatric surgery
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• 제17권1호
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• pp.65-71
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• 2011

Currarino syndrome is a hereditary syndrome characterized by the triad of a sacral bony defect, presacral mass and anorectal malformation. We retrospectively reviewed 13 Currarino syndrome patients who were treated in our center between 1997 and 2010. Demographic data, initial symptoms, initial diagnosis. pathologic diagnosis of presacral mass, associated anomalies and managements were analyzed. There were 8 boys and 5 girls. Four patients were diagnosed as Currarino syndrome immediately after birth with failure of passage of meconium and abdominal distension. Four patients underwent surgery for imperforate anus immediately after birth and W8re diagnosed as Currarino syndrome later and underwent reoperation. Three patients were diagnosed during work-up and management with of the tentative diagnosis of Hirschsprung's disease. Diagnosis of the remaining two patients was at the age of 26 months and 9 years and anorectal malformation was not associated. Twelve patients showed hemi-sacrum and one patient showed bilateral sacral subtotal agenesis. Two patients without anorectal malformation underwent presacral mass excision, untethering of spinal cord and repair of myelomeningocele. Six out of 8 patients, excluding 3 that expired or were lost to follow up, with anorectal malformation underwent colostomy, presacral mass excision, untethering of spinal cord, repair of myelomeningocele, posterior sagittal anorectoplasty and colostomy repair. One patient underwent only posterior sagittal anorectoplasty after colostomy. One waits the scheduled operation only with Hegar dilatation. Pathologic examation of presacral masses showed myelomeningoceles in 4 patients, lipomyelomeningoceles in 3 patients and dermoid Cyst in one patient. Teratoma was combined in 2 patients. Eight patients needed neurosurgical operation for spinal cord problems. Seven patients had urologic anomalies and two of them underwent operation. Currarino syndrome should be considered as a differential diagnosis in pediatric patients with abdominal distension, constipation and anorectal malforlnation. For proper evaluation and treatment, a multi-disciplinary approach is recommended.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제10권1호
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• pp.44-50
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• 2007

목 적: 소아에서 기능성 변비는 소화기 증상 중 가장 흔한 증상이나 각 개인이 호소하는 증상이 다양하여 쉽게 정의하기 어렵다. 단순 복부 X-선 사진을 이용한 점수체계(scoring system)는 간단하고 비용이 적게 들어 변비의 정도를 평가하는데 사용하고 있다. 그러나 소아변비의 정도를 평가하는데 어떤 점수체계가 임상적으로 더 유용한지에 대한 연구는 매우 드물다. 따라서 저자들은 소아 변비 환자에서 단순 복부 방사선사진을 이용하여 Barr, Blethyn과 Leech 점수를 측정하여 유용성을 비교 연구하였다. 방 법: 2006년 1월부터 8월까지 조선대학교 병원 소아과 외래를 방문하거나 입원한 4세에서 15세 사이의 환아 77명(변비군 38명, 대조군 39명)을 대상으로 하였다. 네 명의 전공의가 77매의 동일한 단순 복부 X-선 사진을 보고 Barr, Blethyn과 Leech 점수체계를 사용하여 1주 간격으로 각각 2회 점수를 매겼으며 점수 측정시에 환자의 대한 사전 정보를 배제하였다. 각 점수체계 방법에 대한 검사자들의 유의성은 ${\kappa}$ 상수을 계산하여 평가하였다. 결 과: 대상아 77명 중 남자는 41명(53.2%), 여자는 36명(46.8%)이었고 변비군은 38명(49.4%), 대조군은 39명(50.6 %)이었다. 네 명의 검사자가 측정한 Barr 점수로 계산한 ${\kappa}$ 상수는 각각 0.75, 0.66, 0.68, 0.71이었고, Blethyn 점수로 계산한 ${\kappa}$ 상수는 0.61, 0.58, 0.55, 0.63이며, Leech 점수로 계산한 ${\kappa}$ 상수는 0.88, 0.92, 0.86, 0.89이었다. Barr 점수로 계산한 ${\kappa}$ 상수는 첫 번째 측정 시에 각각 0.66, 0.67, 0.69, 0.66이었으며, 1주후에 두 번째 측정 시에는 각각 0.68, 0.65, 0.71, 0.68로 측정 시기에 따라 유의한 차이가 없었다(p>0.05)(Fig. 1). 네명의 검사자가 측정한 Blethyn 점수로 계산한 ${\kappa}$ 상수는 첫 번째 측정 시에 각각 0.44, 0.55, 0.48, 0.33이었으며, 1주 후에 두 번째 측정 시에는 0.65, 0.34, 0.39, 0.46으로 측정 시기에 따라 유의한 차이가 있었으며(p<0.05), Leech 점수로 계산한 ${\kappa}$ 상수는 첫 번째 측정시에 각각 0.88, 0.91, 0.92, 0.86이고 두 번째 측정 시에 각각 0.81, 0.88, 0.89, 0.84로 측정 시기에 따라 유의한 차이가 없었다(p>0.05). Barr 점수의 평균치는 변비군은 $9.68{\pm}3.55$, 대조군은 $5.32{\pm}3.45$로 두 군 사이에 유의한 차이가 없었다(p>0.05). Leech 점수의 평균치는 변비군은 $10.42{\pm}3.12$, 대조군은 $6.28{\pm}3.56$으로 두 군 사이에 유의한 차이가 있었다(p<0.05). 네 명의 검사자가 측정한 Barr 점수의 특이도는 각각 0.51, 0.55, 0.54, 0.71이었고 민감도는 각각 0.68, 0.61, 0.69, 0.61이었다. Leech 점수의 특이도는 각각 0.88, 0.91, 0.92, 0.86이었고 민감도는 각각 0.90, 0.89, 0.91, 0.84였다. 결 론: 단순 복부 X-선 사진을 이용한 점수체계는 소아 변비의 정도를 평가하는 데 있어서 검사자의 경력과는 무관하게 사용할 수 있으며 그 중 Leech 점수체계가 임상적으로 가장 유용하다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제20권1호
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• pp.1-13
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• 2017

Functional gastrointestinal disorders (FGIDs) are common worldwide and cover a wide range of disorders attributable to the gastrointestinal tract that cannot be explained by structural or biochemical abnormalities. The diagnosis of these disorders relies on the symptom-based Rome criteria. In 2016 the Rome criteria were revised for infants/toddlers and for children and adolescents. In this review, we discuss the novel Rome IV criteria for infants and toddlers. The criteria for infant colic were drastically changed, whereas only minor changes were made for regurgitation, cyclic vomiting syndrome, functional diarrhea, infant dyschezia and functional constipation. In addition to this, the new Rome IV discusses underlying mechanisms of pain in infants and toddlers, including the neurodevelopment of nociceptive and pain pathways, the various factors that are involved in pain experience, and methods of pain assessment in infants and toddlers is essential for the clinician who encounters functional pain in this age group. Overall, the Rome IV criteria have become more distinctive for all disorders in order to improve the process of diagnosing pediatric FGIDs.

• Advances in pediatric surgery
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• 제19권2호
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• pp.156-161
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• 2013

Congenital segmental dilatation of the colon is a very rare entity of unknown etiology, characterized by a localized dilatation of a bowel segment of the colon of variable length and an abrupt transition between the normal and dilated intestine. It can affect any part of the colon, with the rectosigmoid colon being the most commonly affected site. The clinical and radiological features may resemble that of Hirschsprung disease, but differ in that the normal ganglion cells are found in the dilated and normal segment of the colon. We performed laparoscopic-assisted transanal endorectal pull-through for segmental dilatation of rectosigmoid colon in an 8-year-old boy with chronic constipation since the age of 5 months.

• Advances in pediatric surgery
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• 제12권1호
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• pp.115-125
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• 2006

Postoperative management of anorectal malformation consists of colostomy management and then management after definitive surgery. Colostomy management requires attention to certain details, i. e. complete decompression of the distal limb to avoid secondary megarectosigmoid and prevention and treatment of urinary tract infections in patients with rectourethral fistula. Management after definitive surgery requires the care of catheters placed in the rectourethral fistula or cloacal defect. Prevention and treatment of various complications after definitive operation, i. e. wound infection, anal stenosis or stricture, anal mucosal prolapse, and management of functional disorders, i.e. constipation, fecal incontinence and urinary incontinence are also necessary. In this review, recent trends for the prevention and treatment of the above mentioned problems after operation for anorectal malformation are presented.

• Advances in pediatric surgery
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• 제8권1호
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• pp.64-67
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• 2002

Since Swenson and Bill established the treatment principles of Hirshsprung's disease in 1948, there have been significant advances in the management of this disease. During the last decade, one-stage correction of Hirshsprung's disease without colostomy and primary laparoscopic pull-through procedure became popular with recent advances in the technology and refinement of the equipments. But the outcomes for Hirshsprung's disease are not always successful, and long-term follow-up isessential. Most children after corrective surgery show significant improvement in respect to fecal continence and constipation, that may not be apparent until late adolescence. The purpose of this study was to review the postoperative problems of Hirschsprung's disease and it's management.

• Clinical and Experimental Pediatrics
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• 제51권11호
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• pp.1136-1139
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• 2008

The genitourinary tract and gastrointestinal system are interdependent but share the same embryological origin, pelvic region, and sacral innervation. Although children with voiding disturbances often present with bowel dysfunction, this coexistence was considered coincidental until recently. However, it is now accepted that dysfunction in emptying of both systems is interrelated. Afferent impulses carrying sensory information are transmitted through the spinal cord and brainstem toward several cortical and subcortical areas, resulting in conscious control of the bladder and bowel. Alteration in these afferent pathways can result in dysfunction, including urinary and fecal incontinence. Distal gastrointestinal tract problems such as constipation might induce an inhibitory rectovesical reflex that interferes with normal voiding. Therefore, lower urinary tract function seems to be closely associated with distal gastrointestinal tract function.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권6호
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• pp.619-623
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• 2019

We report the case of a seven-year-old boy with an ingested foreign body, which was retained within the appendix for a known duration of ten months, ultimately requiring appendectomy. The ingested foreign body was incidentally discovered by abdominal x-ray at an emergency room visit for constipation. Despite four bowel cleanouts, subsequent x-rays showed persistence of the foreign body in the right lower quadrant. While the patient did not have signs or symptoms of acute appendicitis, laparoscopic appendectomy was performed due to the risk of this foreign body causing appendicitis in the future. A small metallic object was found within the appendix upon removal. This case highlights the unique challenge presented by foreign body ingestions in non-verbal or developmentally challenged children and the importance of further diagnostic workup when concerns arise for potential retained foreign bodies.

• Clinical and Experimental Pediatrics
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• 제53권7호
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• pp.741-744
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• 2010

Purpose: To evaluate the long-term safety of polyethylene glycol (PEG) 4000 in children with constipation, particularly the biochemical aspects of safety. Methods: Medical records were evaluated, and 100 children, who had been taking PEG 4000 for more than 6 months, and who had been under clinical and biochemical monitoring, were enrolled. Ages; $6.11{\pm}3.12$ years, Duration of therapy; $16.93{\pm}7.02$ months, dose of PEG 4000; $0.72{\pm}0.21g/kg/d$. Results: None of the children complained of clinical adverse effect. The first biochemical test was performed at 8.05 months after beginning of PEG 4000. Serum phosphate (SP) value was high in 10 children, and leucopenia was noted in one child. The second test was performed in 44 children at 7.57 months after the first test. The SP value was high in four children, including the three children whose initial SP value was high and one new child. Six out of 10 children with high initial SP value became normal and one was lost. Hypernatremia was noted in one child. The third test was done in 15 children at 7.5 months after the second test. The SP value of the new child from the second test was high, but became normal after finishing treatment. Two out of 3 children with high SP value at the second test became normal and one was lost. The fourth test was done in 2 children few months after the third test. All of the results were normal. There were no relation between duration of therapy and hyperphosphatemia, or between dose of PEG 4000 and hyperphosphatemia. Conclusions: PEG 4000 is safe for long-term therapy in children with constipation with respect to biochemical parameters.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제9권2호
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• pp.200-209
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• 2006

목 적: 만성 기능성 변비 환아의 임상 증상과 경과를 관찰하고, 장기적인 치료 성적을 평가하여 치료 결과에 영향을 미치는 요인을 분석함으로써 변비 치료에 도움을 얻고자 하였다. 방 법: 2001년 3월부터 2005년 6월까지 순천향대학교 부천병원에서 만성 기능성 변비로 진단받고 1개월 이상 치료받고 경과를 볼 수 있었던 63명의 환아를 대상으로 임상 증상, 치료에 따른 경과, 치료 결과 및 재발 여부 등을 조사하고 예후와 관련된 요인을 분석하였다. 결 과: 대상 환아들의 성별 분포는 남아가 35명(55.6%), 여아가 28명(44.4%)이었고 남아가 여아에 비해 유분증이 유의있게 많았다. 발병 연령은 평균 $21.1{\pm}23.5$ (1.9~84.0)개월이었으며 진단 당시 평균 연령은 $47.1{\pm}34.2$ (6.9~138.0)개월이었다. 치료 전 주당 배변 횟수는 평균 $3.2{\pm}2.3$ (0.5~10.0)회였고, 변비와 동반된 증상으로는 유분증이 34명(54.0%), 굵은 변이 30명(47.6%), 배변 횟수의 감소가 20명(31.7%), 배변 시 힘주기와 변 참기가 각각 19명(30.2%) 순이었다. 추적 관찰 기간은 평균 $34.2{\pm}14.6$ (3.6~60.0)개월이었으며 전화 통화 당시 변비 증상으로부터 회복된 환아는 44명(69.8%)이었고 증상이 남아있었던 환아는 19명(30.2%)이었다. 변비에서 회복된 환아들의 임상적 경과를 살펴보면 유분증이 있었던 환아들이 유분증이 없어진 시간은 치료 시작 후로 부터 평균 $4.3{\pm}2.4$ (1.0~36.0)개월이었으며, 배변시 힘을 많이 주었던 환아들이 변을 힘주지 않고 누게 된 시간은 평균 $5.0{\pm}1.4$ (0.8~36.0)개월이었고, 변을 참았던 환아들이 변을 참지 않게 된 시간은 평균 $5.0{\pm}3.1$ (1.0~36.0)개월이었다. 변비가 재발한 경우는 15명(23.8%)이었으며 남아가 9명(60%), 여아가 6명(40%)이었다. 치료 종료 후로부터 재발하기 까지의 기간은 평균 $2.9{\pm}1.9$ (1.0~6.0)개월이었으며 성별, 발병 나이, 변비의 증상, 치료 시작 전 증상의 지속 기간, 진단 당시 유분증 유무, 이유식 시작 시기와 대변 가리기 훈련 시기 등은 재발에 영향을 미치지 않았으며 치료 기간이 재발에 영향을 미치는 유일한 인자였다. 결 론: 대부분의 소아 만성 기능성 변비 환아가 치료 시작 후 5개월 경에 증상이 회복되었으나 치료 종료 후 약 3개월 내에 재발할 수 있으며 치료 기간이 재발 여부에 영향을 미치는 것으로 보아 충분한 기간 동안 치료하는 것과 정기적인 추적 관찰이 중요하다고 하겠다.