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Case Reports

[English]
The First Case of Endoscopic Balloon Dilatation for a Newborn With a Congenital Duodenal Web in Korea
Seo-Hee Kim, Ju Yeon Lee
Adv Pediatr Surg 2024;30(1):23-27.   Published online May 31, 2024
DOI: https://doi.org/10.13029/aps.2024.30.1.23
Duodenal web (DW) is a rare congenital anomaly of the intestinal tract that can lead to severe dehydration and electrolyte imbalance. A 14-day-old boy presented with recurrent vomiting and weight loss and was diagnosed with DW. Duodenoscopy identified a pinhole structure in the second portion of the duodenum, prompting a subsequent endoscopic balloon dilatation procedure. Following the procedure, vomiting and abdominal distension resolved, and the patient was discharged on a regular diet. No symptoms recurred during follow-up.
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[English]
Recurrent Congenital Milium of the Nipple
Hyun Beak Shin, Yeon-Jun Jeong
Adv Pediatr Surg 2019;25(2):60-62.   Published online December 12, 2019
DOI: https://doi.org/10.13029/aps.2019.25.2.60

Congenital milia of the nipple are extremely rare, and standard treatment has not yet been established because nipple preserving excision is problematic due to the location. Although most reports show excisional biopsy resulting in good outcomes without recurrence, there is a lack of consensus about treatment modality, with several studies suggesting that incisional evacuation by needle, or a ‘wait and see’ approach represent sufficient treatment. This case report is about a recurrent case after incisional evacuation for congenital milium of the nipple. We recommend nipple preserving excision with exfoliation of the milial capsule as being the most appropriate treatment modality for congenital milium of the nipple.

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Review Article

[English]
Enterostomy Closure after Acute Abdomen in Neonate and Infant
Hee-Beom Yang, Hyun-Young Kim
Adv Pediatr Surg 2018;24(2):35-43.   Published online December 24, 2018
DOI: https://doi.org/10.13029/aps.2018.24.2.35

The risk of acute abdomen in neonates and infants is generally increased due to advanced maternal age and neonatal intensive care unit care development. Enterostomy is a safe procedure for acute abdomen in neonates and infants. However, there is no consensus for the optimal timing of enterostomy closure (EC). A few considerations should be reviewed for deciding the timing of stoma closure to obtain the best outcome. Distal loopography is commonly performed upon examination to ascertain the existence of a distal passage after EC, detect signs of disease-specific complications, and assess the need of surgery in addition to EC. Pathology review is also one important pre-closure consideration. When the incidence of a hypoganglionosis or an aganglionosis combined with acute abdomen is observed in neonates and infants, thorough examinations should be performed to conclusively determine whether there is no passage disturbance of the distal bowel. Refeeding not only provides information about chance to grow, electrolyte imbalance correction, and proximal and distal bowel size match, but also provides information about distal bowel passage when there is a doubtful distal loopography or pathology result. Early closure enables growth spurt with the correction of water/electrolyte imbalance. It potentially reduced medical costs, less discrepancy between proximal and distal bowel size. Some factors favoring a late stoma closure may be due to less postoperative complications, early recovery, shorter time of total parenteral nutrition after EC, and decreased length of hospitalization after the EC. Some studies have shown controversial results. In summary, a pre-closure evaluation is imperative to assure the safety of an EC. The optimal timing of an EC remains controversial. For this reason, individualized approach is needed after reviewing the general condition of each patient. Further prospective study on optimal timing of stoma closure including a randomized clinical trial is needed.

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Original Article

[English]
Single Stage Transanal Endorectal Pull-through Operation for Hirschsprung’s Disease in Neonate: A Single Center Experience
Ju Yeon Lee, Jung Hyun Choi, Jung-Man Namgung, Dae Yeon Kim, Sung-Cheol Kim
J Korean Assoc Pediatr Surg 2016;22(2):38-41.   Published online December 22, 2016
DOI: https://doi.org/10.13029/jkaps.2016.22.2.38
Purpose

The single stage transanal pull-through (SSPT) for Hirschsprung’s disease is becoming the most popular procedure. This single center study compared the result of single stage operation with two-stage operation for Hirschsprung’s disease in neonates.

Methods

We retrospectively reviewed medical records of all patients who were diagnosed as Hirschsprung’s disease and underwent SSPT or two-stage operation operation in Asan Medical Center between January 2003 and July 2014.

Results

There were 17 SSPT and 28 two-stage operation. The mean age of SSPT group was 14.2±7.1 days, and the mean age of two-stage operation group was 15.4±8.6 days for stomy formation, and 188.6±36.3 days for Duhamel operation. The operation time of SSPT was shorter than Duhamel operation (145.0±37.0 minutes vs. 193.0±36.0 minutes, p<0.001). The mean follow-up period of SSPT and two-stage operation was 35.5±34.9 months (range, 2-132 months) and 56.6±35.5 months (range, 1-121 months), respectively. Defecation problem rate such as fecal soiling or fecal impaction showed no significant difference between the two groups (p=0.719). Two SSPT patients required botulinum toxin injection due to rectal stenosis. Three patients of SSPT group underwent re-do endorectal pull-through due to remnant aganglionic or hypoganglionic bowel.

Conclusion

The SSPT showed shorter hospital days. However, few patients experienced rectal stenosis, but were manageable with botulinum toxin injection. The SSPT requires experienced-pathologist, as well as surgeon, because intra-operation pathology reading is critical for appropriate SSPT. SSPT is a feasible and reasonable option to treat Hirschsprung’s disease.

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Review Article

[English]
Neonate Congenital Bochdalek Hernia: A National Survey of Its Members by Korean Association of Pediatric Surgeons
D Y Kim, S C Kim, S H Kim, H Y Kim, H Y Kim, S H Nam, K W Park, J B Park, J Y Park, Y J Boo, J M Seo, S M Ahn, J T Oh, S K Lee, S C Lee, E Y Jang, H K Jang, S E Jung, S M Jung, Y J Jung, E Y Jung, M J Cho, S O Choi, S J Han, Y J Hong, J Hong
J Korean Assoc Pediatr Surg 2016;22(1):6-9.   Published online June 30, 2016
DOI: https://doi.org/10.13029/jkaps.2016.22.1.6

This is a survey on congenital posterolateral diaphragmatic hernia, conducted by Korean Association of Pediatric Surgeons (KAPS). A registration form for each patient during the 5-year period between 2010 and 2014 and a questionnaire were sent to each member. Twenty-six members in 16 institutions returned completed forms. Total patients were 219. Prenatal diagnoses were done in 181 cases (82.6%). Preoperative mortality was 11.4%. Minimal invasive surgery was done in 61 cases (31.8%). Risk factors related with death were Apgar score, oxygenation index, preoperative pH and bicarbonate, O2 saturation, the presence of hernia sac, and the size of defect. The neonatal survival and 1-year survival of total patients were 77.6% and 75.3%.

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Original Articles

[English]
Associated Factors with Parenteral Nutrition Associated Cholestasis in Neonates
Kyoung Eun Kim, Hyon Hui Lee, Mi Lim Chung, Woon Won Kim, So Hyun Nam
J Korean Assoc Pediatr Surg 2015;21(1):1-6.   Published online June 24, 2015
DOI: https://doi.org/10.13029/jkaps.2015.21.1.1
Purpose

Long time total parenteral nutrition (TPN) can induce irreversible liver damage. In this study, we investigated the associated factors of parenteral nutrition associated cholestasis (PNAC) in neonates.

Methods

We retrospectively reviewed 227 neonates (male:female=110:117) those who had received TPN over 2 weeks from March 2010 to February 2014. PNAC was defined as direct bilirubin was higher than 2.0 mg/dL without any cause except TPN.

Results

Overall incidence was 28.6%. PNAC was frequently developed in younger gestational age with lower birth weight. Episodes of sepsis, underlying bronchopulmonary dysplasia, history of necrotizing enterocolitis, and experience of gastrointestinal surgery increase the incidence of PNAC. PNAC was directly associated the duration of TPN and long period to full enteral feeding, reaching 60 and 150 mL/kg/day. Overall mortality rate was 9.7%. It was higher in PNAC group despite PNAC was not the primary cause of death. All survivors were recovered from cholestasis with encourage of enteral nutrition.

Conclusion

PNAC in neonate was associated with younger gestational ages and lower birth weights, duration of TPN, or who experienced sepsis, necrotizing enterocolitis, gastrointestinal surgery or bronchopulmonary dysplasia.

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[English]
Anorectal Malformations: Diagnosis and Management in Neonatal Period
Nam Hyuk Lee
J Korean Assoc Pediatr Surg 2006;12(1):99-106.   Published online June 30, 2006
DOI: https://doi.org/10.13029/jkaps.2006.12.1.99

Anorectal malformations comprise a spectrum of disease and the majority of patients have one or more abnormalities that affect other systems. In evaluating a newborn with anorectal malformation, the decision regarding the need for a colostomy and detection and management of any life threatening associated anomalies are thetwo most important considerations. Perineal inspection provides the clue to the surgical approach in about 80-90% of male and 90% of female newborn baby. The remaining patients who do not show any clinical evidence need radiologic evaluation to decide whether a colostomy should be performed. In most cases the decision to make a colostomy should not be made until the baby is 20 to 24 hours old and evaluation to rule out the presence of associated anomalies completed. A divided colostomy at the junction of the descending and sigmoid colon is recommended for anorectal malformations.

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[English]
Anatomical Variations of the Hymen and Congenital Anomalies of the External Genitalia in Female Newborns
Tae Woo Kim, Nam Hyuk Lee, Sang Youn Kim
J Korean Assoc Pediatr Surg 1997;3(2):108-116.   Published online December 31, 1997
DOI: https://doi.org/10.13029/jkaps.1997.3.2.108

The anatomical variations of the external genitalia including the hymen were observed prospectively in 1,500 female infants born between March, 1992 and July, 1992 at the Taegu Fatima Hospital. Careful inspection of the external genitalia was done within 24 hours after birth, and abnormal findings photographed. Almost all hymenal tissue appeared to be redundant. The hymen was annulus in 89.1 % of patients. Ninteen point nine percent had a central orifice and the remainder a ventral orifice. External ridges, intravaginal ridges, and clefts were present in 71.5 %, 50.7 % and 40.5 %, respectively. Congenital anomalies of the external genitalia were found in 20 patients(1.4 %). There were sixteen cases of hymenal cysts, two hymenal polyps, one imperforate hymen, and one Skene's duct cyst. Seven of the 16 hymenal cysts with stalks were excised and those without stalk were aspirated. Hymenal polyps were excised. Imperforate hymen was incised and drained, and Skene's duct cyst was aspirated successfully. The surgical procedure were done without anesthesia, and the results have been good. In conclusion, routine physical examination of the female newborn infant detects frequent developmental anomalies. The significance of those anomalies will be clarified after longitudinal long-term followup studies.

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Review Article

[English]
Jejunoileal atresia (JIA) is a rare congenital disorder in newborns leading to intestinal obstruction, necessitating urgent surgery. A delay in diagnosis can result in severe complications like sepsis, bowel perforation, and even death. Over the years, improvements in neonatal care, surgical techniques, and nutrition have led to a marked decrease in mortality rates for JIA patients. While the exact cause remains uncertain, it is thought to result from an ischemic insult during later in utero. JIA is categorized into 4 types, detailing the structure and implications of each type. Clinically, mothers may exhibit polyhydramnios, and infants usually present with symptoms like bilious vomiting and abdominal distension. A significant percentage of affected infants do not pass meconium within the first day of life. Diagnosis is commonly through abdominal radiographs, with some cases requiring contrast enemas. Once diagnosed, immediate surgery is advised, with the primary goal to restore intestinal function and length. Nowadays, the survival rate for JIA patients exceeds 90%. This improvement is attributed to a better understanding of the condition, advancements in surgical techniques, and the introduction of total parenteral nutrition. The prognosis varies, with short bowel syndrome being a significant factor determining the outcome. Short bowel syndrome’s prognosis depends on the remaining small bowel length, the ileocecal valve’s presence, and dependence on long-term nutrition. In conclusion, the treatment and prognosis for JIA have seen substantial improvements due to advances in medical care. Early diagnosis and intervention are crucial. While many patients lead a normal life, those with complications like short bowel syndrome may require long-term care. Future efforts should emphasize refining surgical techniques, gaining a deeper understanding of JIA, and enhancing postoperative care. There's also a need for more research into JIA’s genetics and etiology.
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Case Reports

[English]
Ileal Duplication in a Neonate With Jejuno-Ileal Atresia, Midgut Malrotation and Volvulus
Dudhani, Shreyas , Suman, Bijay , Singh, Ramjeewan , Sinha, Amit Kumar , Kumar, Bindey
Adv Pediatr Surg 2023;29(1):40-44.
DOI: https://doi.org/10.13029/aps.2023.29.1.40
Jejuno-ileal atresia is often associated with various other gut pathologies. A 5-day male presented with bilious vomiting, abdominal distension and non-passage of stools. Exploratory Laparotomy showed a type IIIA ileal atresia with midgut volvulus and atypical malrotation for which Ladd’s Procedure and anastomosis of atretic bowel was done. A fleshy tubular structure extracted from distal bowel showed ileal duplication. In JIA, the vascular theory of pathogenesis is widely accepted with multiple studies to suggest the same. The mechanism being mesenteric ischemia leading to intestinal necrosis, resorption, and atresia. It has been found that abdominal duplication cyst often occurs simultaneously with short bowel, intestinal atresia, stenosis and enteric duplication for which a common vascular pathogenetic mechanism has been suggested. Our case serves as an in vivo description of the proposed theory where the findings may be described elegantly. We describe and discuss the mesenteric ischemia theory in bowel atresia and hope to further our understanding about the same.
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[English]
Haddad Syndrome: A Case of Congenital Central Hypoventilation Syndrome Combined with Hirschsprung Disease
Jeong, So Young , Shin, Hyun Beak , Jeong, Yeon Jun , Kim, Jin Kyu
Adv Pediatr Surg 2020;26(2):72-76.
DOI: https://doi.org/10.13029/aps.2020.26.2.72
Congenital central hypoventilation syndrome (CCHS) with Hirschsprung disease (HD), also known as Haddad syndrome, is an extremely rare disorder. Recent studies have identified the paired like homeobox 2b (PHOX2B) gene as the major gene involved in the development of CCHS. The syndrome is diagnosed when gene analysis confirms a mutation in the involved gene, but making an early diagnosis is difficult because of the rarity of the disease. In this study, we report the case of a newborn male with recurrent hypoventilation and bowel distension. HD was suspected on barium enema, and loop ileostomy was performed. After surgery, the abdominal symptoms gradually improved, but extubation was not possible owing to recurrent respiratory failure. These clinical manifestations were indicative of Haddad syndrome, and genetic testing confirmed the presence of a PHOX2B mutation. The patient was diagnosed with Haddad syndrome on the 11th day after birth.
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