Biliary atresia (BA) is a major cause of extrahepatic biliary obstruction in children. Malnutrition is a significant clinical problem in children with BA. BA may induce the malabsorption of fat and fat-soluble vitamins, resulting in cholestasis and an impaired nutritional status. For the treatment of BA, it is most important to reconstruct the bile flow as early as possible by performing a Kasai portoenterostomy. After the Kasai operation, growth and nutrition are restored, but to follow normal growth and development, it is necessary to evaluate the nutritional status and support. Therefore, the purpose of nutritional support in children with BA is to normalize growth and development, prevent further liver damage and deterioration of the patient's nutritional status, avoid vitamin and mineral deficiencies, and improve the quality of life of patients.
Avitaminosis ; Bile ; Biliary Atresia* ; Child ; Cholestasis ; Growth and Development ; Humans ; Liver ; Malnutrition ; Miners ; Nutritional Status ; Nutritional Support* ; Quality of Life ; Vitamins

Mixed acinar-neuroendocrine carcinoma (MANEC) is a malignant pancreatic tumor that rarely occurs in children. It is diagnosed pathologically according to the proportion of neuroendocrine cells present, highlighting the need for surgical biopsy. A 13-year-old boy presented with a 10-cm palpable mass on CT. Surgical resection was performed, and the pathological diagnosis was MANEC. There were no postoperative complications, and the patient was discharged from the hospital 10 days after surgery. He is presently undergoing adjuvant chemotherapy. We reviewed historical MANEC cases published in the English literature. We concluded that pathological analysis of a surgically resected specimen is necessary for an accurate diagnosis of MANEC, and that publication of more cases is needed to determine the optimal management strategy for MANEC.
Adolescent ; Biopsy ; Carcinoma, Acinar Cell ; Carcinoma, Neuroendocrine ; Chemotherapy, Adjuvant ; Child ; Diagnosis ; Humans ; Immunohistochemistry ; Male ; Neuroendocrine Cells ; Postoperative Complications ; Publications ; Surgical Procedures, Operative

We experienced 3 cases of Candida esophagitis in infancy which were diagnosed by esophageal endoscopy. First case, 10 month-old boy with combined immune deficiency had suffered from oral thrush and poor feeding for more than 4 months. Esophageal endoscopy revealed multiple whitish creamy patches on the friable erythematous and necrotic mucosa of the esophagus. He was firstly treated with amphotericin-B but in vain. Then he was treated with fluconazole (5 mg/kg/day) and in a few days oral thrush nearly disappeared and endoscopy after 2 weeks revealed complete healing of the esophagitis. Second case, 6 month-old boy with some cellular immue defect also suffered from oral thrush, poor feeding and intermittent fever. He was treated with fluconazole and oral thrush was imporved. He was discharged without follow up endoscopy. Third case, 4 month-old girl with liver cirrhosis due to infantile cholestasis had Candida sepsis. Esophagitis was found incidentally during the endoscopic examination of esophageal varix. First 2 cases showed multiple small filling defects and decreased motility on esophagography. Candida antigen was not detected in the sera of all 3 cases of candidiasis. We conclude that Candidia esophagitis should be suspected when an infant has been suffering from long-term treatmet-resistant oral thrush and poor feeding and that esophageal endoscopy can be easily performed in infants also and useful in diagnosing esophagitis and assessing the outcome of treatment.
Candida* ; Candidiasis ; Candidiasis, Oral ; Cholestasis ; Endoscopy ; Esophageal and Gastric Varices ; Esophagitis* ; Esophagus ; Female ; Fever ; Fluconazole ; Humans ; Infant ; Liver Cirrhosis ; Male ; Mucous Membrane ; Sepsis

Lymph node enlargement is a common finding in children suggesting normal or benign. Palpable nodes which are large, hard or fixed must be examined carefully to rule out malignant diseases. In this case, a 15-year-old boy presented to our hospital to inspect the palpable mass at his post-auricular area being found 2 months ago. It was diagnosed as nodal marginal zone lymphoma (NMZL) through excisional biopsy and immunohistochemistry. NMZL is very rare, especially in children and young adults, but occurs locally in most cases with a good prognosis compared to adults. We described a rare case of NMZL diagnosed in adolescent.
Adolescent ; Adult ; Biopsy ; Child ; Humans ; Immunohistochemistry ; Lymph Nodes ; Lymphatic Diseases ; Lymphoma* ; Male ; Prognosis ; Young Adult

Gardner-associated fibroma (GAF) is a benign lesion of soft tissue which has recently been described and is exceedingly rare in children. GAF is associated with adenomatous polyposis coli gene mutation, familial adenomatous polyposis and desmoid. We report a case of patient with soft tissue tumor on her lower back which was turned out to be GAF. The patient was a 19-month-old female who visited out-patient clinic with palpable mass on her lower back and we performed surgical excision. The tumor was located at subcutaneous and we excised the tumor including surrounding soft tissue. She discharged without any complication on surgery day. The pathologic report showed dense collagenous tissue with spindle cell and adipose tissue, suggestive of GAF. We are planning to check gene study and to perform endoscopy and abdominal ultrasonography for at the age of 4.
Adenomatous Polyposis Coli ; Adipose Tissue ; Child ; Collagen ; Endoscopy ; Female* ; Fibroma* ; Fibromatosis, Aggressive ; Gardner Syndrome ; Humans ; Infant* ; Outpatients ; Ultrasonography

A baby was diagnosed with esophageal atresia (EA) with tracheoesophageal fistula (TEF) on the next day after birth, and end-to-end anastomosis of esophagus with TEF ligation was performed. The distance between proximal and distal esophageal pouch was checked as 3 vertebral body lengths and a 1 cm-sized bronchogenic cyst (BC) was identified near carina on the right side, just below the proximal esophageal pouch. This case report described the baby who have a BC was located between the both esophageal pouch and a longer esophageal gap than usual EA with distal TEF.
Bronchogenic Cyst* ; Esophageal Atresia* ; Esophagus ; Ligation ; Parturition ; Tracheoesophageal Fistula

Isolated cystic duct cysts are rare entities, with few cases having been reported. We present the case of a 4-month-old male patient presenting with abdominal pain and vomiting. Ultrasonography and magnetic resonance cholangiopancreatography revealed an isolated cystic duct cyst with associated stones. The patient underwent open cholecystectomy with complete cyst excision and cystic duct transection; there were no postoperative complications. While lesions like the one described herein are extremely rare, they should be included as a separate category in classifications of choledochal cysts.
Abdominal Pain ; Cholangiopancreatography, Magnetic Resonance ; Cholecystectomy ; Choledochal Cyst ; Classification ; Cystic Duct* ; Gallstones ; Humans ; Infant* ; Male* ; Postoperative Complications ; Ultrasonography ; Vomiting

Purpose: Cyst excision with hepaticojejunostomy has been the classic procedure for treating choledochal cysts, and the use of laparoscopic treatment has been favored recently. The purpose of this study was to compare the long-term biliary complication of laparoscopic operation with open surgery for choledochal cyst presenting in children. Methods: A retrospective study comparing the laparoscopic and open procedures was performed in 185 patients with choledochal cyst in a single children’s hospital. There were 109 patients who were operated with open surgery, and 76 patients operated with laparoscopic surgery. The primary outcome was long-term biliary complications and the secondary outcome included operative time, intraoperative transfusion, length of hospital stay, and other late postoperative complications. Results: In the patient’s demographics, there was no significant difference between the 2 groups. Notably, it was shown that the operative time was longer in the laparoscopic group. The number of patients requiring blood transfusion intraoperatively was lower in the laparoscopic group. It was noted that the hospital stay was not statistically different. The duration to resumption of diet and duration of drainage were longer in the laparoscopic group. Biliary complications were shown to be significantly higher in the open group. The risk factor for long-term biliary complications was noted with the intraoperative transfusion. Conclusion The use of a laparoscopic choledochal cyst excision with hepaticojejunostomy is a safe and feasible technique in a young patient. The long-term biliary complication was lower compared to open surgery, rendering this a good option for pediatric patients.

Background: Fifty to sixty percent of patients with anorectal malformation (ARM) have at least one associated anomaly (AA). We determined the incidence of AA with the subtypes of ARM classified in accordance with the Krickenbeck classification and analyzed differences in the incidence rates of major and minor AAs according to organ system. Methods: From January 1999 to May 2017, we retrospectively analyzed congenital anomalies in patients who underwent an anoplasty for ARM at our institution. The AAs were divided into nine organ systems. To analyze the difference in the incidence of AAs, we calculated odds ratios (ORs) using cases of perineal fistula as the base group. Results: Of the 460 patients, 256 (55.7%) were male, 299 (65%) had at least one anomaly, and 274 (59.6%) had major AAs. According to organ system, AAs were most common in the genitourinary (28%), cardiovascular (25%), and spinal/vertebral systems (22.6%). Major AA was most common in the cardiovascular (23%) and spinal/vertebral and genitourinary systems (19.3%). According to ARM subtype, AAs were common in the order of cloaca (93.9%), rectovaginal fistula (85.7%), and recto-bladder neck fistula (85%). For the incidence of AAs, cloaca (OR, 15.7) and recto-bladder neck fistula (OR, 5.74) showed significantly higher ORs. In the analysis of major AAs, the cloaca (OR, 19.77) showed the highest OR, followed by no fistula (OR, 4.78) and recto-bladder neck fistula (OR, 3.83). Conclusion A considerable number of patients with ARM had AAs. Our data are useful for predicting AAs in patients with ARM.

Purpose: Robotic surgery (RS) has the advantages of 3-dimensional view, optical magnification, motional scaling, and improved ergonomics and degree of freedom. Although RS has widely been performed on pediatric patients lately, there are still numerous restrictions and ambiguous indications. The purpose of this study was to report our early experience with RS on pediatric patients at a single center. Methods: Electronic medical records of patients who underwent RS with the da Vinci Xi surgical platform (Intuitive Surgical, Inc.) in Seoul National University Children Hospital from November 2019 to August 2021 were reviewed retrospectively. The median follow-up was 21.0 months (range, 12.3–31.8 months). An online survey was conducted to investigate satisfaction with robotic surgical scars. Results: Fifty-four patients underwent robotic surgeries (median age at operation, 11.1 years [range, 0.1–17.8 years]). In our hospital, patients had 20 different kinds of robotic surgeries, including choledochal cyst excision with hepaticojejunostomy, ovarian mass excision, and others. Median operation time and console time were 157.5 minutes (range, 45–505 minutes) and 40 minutes (range, 11–360 minutes), respectively. All cases were done without conversion into open or laparoscopic methods. Postoperative complications were found in 5 patients. According to an online survey, over half of patients (60.9%) answered that they felt satisfied with scars. Conclusion Our early experience demonstrated the safety and feasibility of RS in children with a range of diagnoses and complicated procedures. With more experience, RS could be an alternative to traditional open or laparoscopic operations in pediatric patients. Further studies are needed to clarify indications of pediatric RS.