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Robot-Assisted Hepatectomy in Pediatrics: Two Case Reports of Single Center

Jueun PARK ; Suhyeon HA ; Hyunhee KWON ; Jung-Man NAMGOONG ; Dae Yeon KIM

Advances in Pediatric Surgery.2024;30(2):73-79. doi:10.13029/aps.2024.30.2.73

Since the first introduction of robotic surgery systems in Korea in 2005, there has been a gradual increase in the number of robotic surgeries performed. However, robotic liver resection is one of the most complex procedures, and its application, especially to children, is still limited. Therefore, in this study, we aim to present our experiences with 2 pediatric patients who underwent robotic liver resection in Asan Medical Center and discuss the safety and feasibility of robot-assisted hepatectomy in pediatrics.

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National Survey of Gastroschisis and Omphalocele by Korean Association of Pediatric Surgeons

Yeon Jun JEONG ; Dayoung KO ; Eun-Jung KOO ; Hyunhee KWON ; Dae Yeon KIM ; Soo-Hong KIM ; Wontae KIM ; Hae-Young KIM ; Hyun Young KIM ; Seong Chul KIM ; Younghyun NA ; Jung-Man NAMGOONG ; So Hyun NAM ; Sungjoo PARK ; Junbeom PARK ; Jinyoung PARK ; Tae-Jun PARK ; Jeong-Meen SEO ; Ji-Young SUL ; Joonhyuk SON ; Hyun Beak SHIN ; Joohyun SIM ; Jung-Tak OH ; Chaeyoun OH ; Joong Kee YOUN ; Sanghoon LEE ; Ju Yeon LEE ; Cheolgu LEE ; Kyong IHN ; Eunyoung JUNG ; Jae Hee CHUNG ; Yong-Hoon CHO ; Yun Mee CHOE ; Soo Jin Na CHOI ; Seok Joo HAN ; In Geol HO

Advances in Pediatric Surgery.2024;30(2):39-51. doi:10.13029/aps.2024.30.2.39

Purpose: This study provides insights into the prevalence at birth, clinical characteristics, and outcomes of gastroschisis and omphalocele in Korea over the past decade, addressing the lack of localized data despite advanced healthcare capabilities. Methods: The study retrospectively analyzed data from 20 pediatric surgical centers in Korea from January 2012 to December 2021, including 269 patients diagnosed with gastroschisis or omphalocele. Data variables included gender, gestational age, birth weight, associated anomalies, type of defect, surgical interventions, and outcomes. Results: The study covered 269 patients, with 80 gastroschisis and 189 omphalocele cases. Gastroschisis prevalence at birth remained stable at 2.15 per 100,000 live births, while omphalocele increased to 5.08 per 100,000. Both conditions had similar gender ratios (0.95). Gastroschisis patients had lower birth weights (2,463.90±505.50 g) and smaller head circumferences (31.97±1.86 cm) compared to omphalocele patients (2,757.65±761.24 g, 32.78±2.64 cm). Omphalocele cases had more associated anomalies, especially cardiovascular issues. Prenatal diagnosis rates were high: 93.7% for gastroschisis and 86.4% for omphalocele. About 96.3% of gastroschisis and 84.1% of omphalocele patients were born in their treatment hospitals. Gastroschisis patients underwent surgery sooner (average 3.5 days) and started feeding later (16.5 days) than omphalocele patients (average 56.5 days to surgery, 6.6 days to start feeding). Hospital stays and follow-up durations were similar, averaging around 782.6 days for gastroschisis and 800.3 days for omphalocele patients. Survival rates were 89.7% for gastroschisis and 87.1% for omphalocele. Conclusion The study highlights the need for early diagnosis, centralized care, and specialized surgical approaches to optimize outcomes for gastroschisis and omphalocele patients in Korea. Enhanced prenatal screening and surgical protocols are recommended to improve these patients' prognosis.

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Clinical and Imaging Diagnosis of Vascular Anomaly: Experience of 137 Patients From a Single Center

Sujin GANG ; Hyunhee KWON ; Jueun PARK ; Dae Yeon KIM

Advances in Pediatric Surgery.2024;30(2):52-63. doi:10.13029/aps.2024.30.2.52

Purpose: The International Society for the Study of Vascular Anomalies (ISSVA) classification is crucial in diagnosing vascular anomalies (VAs), surpassing the International Classification of Diseases 10th Revision. This study aims to reevaluate diagnoses using ISSVA criteria and explore diagnostic patterns. Methods: Analyzing 138 pediatric VA patients diagnosed via magnetic resonance imaging from 2018 to 2023 at Asan Medical Center, we reviewed clinical, imaging, pathology, and genetic data. Diagnoses were revised per 2018 ISSVA criteria, assessing discrepancies. Results: Among 133 VA cases, 125 were malformations and eight were tumors, mostly in the head and neck. Clinical and imaging diagnoses disagreed in 51 cases. Some initially complex malformations were simplified. Lymphatic malformation cases shifted to VAs and vascular tumors were identified post-initial diagnosis. Conclusion Accurate diagnosis of VAs is essential for prognosis, treatment planning, and predicting outcomes. However, 14.2% of patients showed discordance between clinical diagnoses and imaging findings. Capillary malformations were often overlooked in imaging but became evident with relevant clinical findings. Adopting a multidisciplinary approach and a unified diagnosis based on ISSVA classification is crucial for clearly defining VAs.

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Clinical Characteristics That Led to Diagnosis of Crohn’s Disease After Anal Fistula Surgery in Patients Aged 10–19 Years: A Single Center, Retrospective Study

Ah Young KANG ; Ki-Yun LIM ; Keehoon HYUN ; Heecheol CHANG

Advances in Pediatric Surgery.2024;30(2):64-72. doi:10.13029/aps.2024.30.2.64

Purpose: Crohn’s disease (CD) is mainly presenting gastrointestinal symptoms but also may involve perianal diseases, with anal fistula being the most common. Anal fistula surgery performed without recognition that the patient has CD increases the complications such as delayed wound healing and anal sphincter injury. This study aimed to investigate clinical symptoms and surgical findings that could allow early diagnosis of CD in patients aged 10 to 19 years who underwent anal fistula surgery. Methods: Among the 320 patients under the age of 19 who underwent anal fistula surgery, those who were diagnosed with tuberculosis fistula were excluded, medical records of 316 patients were examined. We investigated the characteristics of anal fistula and postoperative wounds as well as the colonoscopic and surgical findings. Results: Compared to 272 patients not diagnosed with CD (non-CD group), 44 patients diagnosed with CD (CD group) showed significantly higher levels of C-reactive protein as well as anal discharge, family history of inflammatory bowel disease, history of anal fistula, diarrhea, abdominal pain, weight loss, complex anal fistula, recurred anal fistula, delayed wound healing and friable/edematous/granulation of surgical site. Conclusion For patients aged 10–19 years with anal fistulas, emphasizing early suspicion and an active diagnostic work-up is essential for early diagnosis of CD.

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Comparative Analysis of Two National Surveys on Esophageal Atresia With or Without Tracheoesophageal Fistula: A Retrospective Study by the Korean Association of Pediatric Surgeons

Jinyoung PARK ; Dae Yeon KIM ; Seong Chul KIM ; Hyun-Young KIM ; So Hyun NAM ; Jeong-Meen SEO ; Jung-Tak OH ; Myung-Duk LEE ; Suk-Koo LEE ; Soo Min AHN ; Hye Kyung CHANG ; Sung Eun JUNG ; Yeon Jun JEONG ; Eunyoung JUNG ; Jae Hee CHUNG ; Yong Hoon CHO ; Soon Ok CHOI ; Seung Hoon CHOI ; Yun Mee CHOE ; Seok Joo HAN ; Jeong HONG ; Nam-Hyuk LEE

Advances in Pediatric Surgery.2024;30(1):1-8. doi:10.13029/aps.2024.30.1.1

Purpose: The Korean Association of Pediatric Surgeons (KAPS) conducts annual nationwide surveys on various aspects of pediatric surgical diseases, with the results being discussed during KAPS’s annual spring meetings. Methods: KAPS conducted two national surveys, in 1995 and 2016, to investigate esophageal atresia (EA) with or without tracheoesophageal fistula (TEF). The authors analyzed data from these surveys to identify differences or changes in the annual occurrence, demographic characteristics, clinical presentation, preoperative diagnostic methods, anatomical type, associated anomalies, surgical treatment, and postoperative outcomes among patients with EA/TEF treated by KAPS members. Results: The first and second national surveys included 148 and 211 patients with EA/TEF, respectively. Excessive salivation was the most prevalent clinical symptom in both surveys. Type C was the most common form of EA/TEF in both surveys. The first survey included 126 patients, all of whom underwent open surgery. In the second survey, 152 (78.4%) of 194 patients underwent open surgery, while 34 (17.5%) underwent thoracoscopic surgery. Primary esophageal repair was performed on 96 (76.2%) of 126 patients in the first survey and on 160 (82.5%) of 194 patients in the second survey. Anastomotic strictures developed in 21.4% and 32.5% of patients, anastomotic leakage in 22.2% and 10.3%, recurrent fistula in 2.4% and 4.2% during the first and second surveys, respectively. The respective survival rates for group A were 90.2% and 98.3% in the first and second surveys. For group B, the rates were 73.9% and 98.1%, and for group C, they were 34.5% and 68.1%, respectively, according to the Waterston classifications. Conclusion These nationwide surveys provide comprehensive information on the status, detailed treatment, and outcomes for Korean pediatric patients with EA/TEF. They are anticipated to be an invaluable resource and guide for pediatric surgeons seeking to expand their knowledge on EA/TEF and its treatment options.

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Hirschsprung Disease Associated With Pelvic Neuroblastoma: A Rare Neurocristopathic Association

Sravanthi VUTUKURU ; Nitin J PETERS ; Shailesh SOLANKI ; Amita TREHAN ; Kirti GUPTA ; Monika BAWA

Advances in Pediatric Surgery.2024;30(1):32-37. doi:10.13029/aps.2024.30.1.32

Hirschsprung disease (HSCR) is a genetic disorder with an incidence of 1:5000, seen in the pediatric age group. The association between HSCR and neuroblastoma (NBL), ends of the neurocristopathy spectrum is rare. Less than 10 cases of this association are reported in the literature and the association between the Phox gene and Sox10 gene in the pathophysiology of these is being studied. We report a one-year-old baby, who presented to us, with chronic constipation on regular enemas and laxative usage. There was a history of delayed passage of meconium. At the time of Duhamel’s pull through a well-defined, bilobed hard presacral mass, was encountered. Excision and coccygectomy were done and the pull was completed. The histopathology showed a well-differentiated NBL. Fludeoxyglucose positron emission tomography scan and the N-Myc amplification were negative and the patient was managed with expectant treatment. She is doing well over a 3-year follow-up with no recurrence and good resolution of bowel functions.

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Sirolimus Treatment of Complicated Vascular Anomalies: A Single Center’s Experience Over 2 Years

Jueun PARK ; Suhyun HA ; Hyunhee KWON ; Jung-Man NAMGOONG ; Hee Mang YOON ; Pyeong Hwa KIM ; Dae Yeon KIM

Advances in Pediatric Surgery.2024;30(1):14-22. doi:10.13029/aps.2024.30.1.14

Purpose: Complicated vascular anomalies, characterized by encasing vital organ or diffusely locating unresectable lesion, pose therapeutic challenges with limited response to conventional treatment such as surgical resection or sclerotherapy. Sirolimus, an mammalian target of rapamycin inhibitor, has shown promising therapeutic effects in patients with vascular anomalies by inhibiting vascular endothelial growth factor, as reported in several studies. Here, we analyzed the treatment outcomes of patients who received sirolimus for complicated vascular anomalies at our institution. Methods: Patients treated with sirolimus at the Department of Pediatric Surgery, Asan Medical Center from January 2018 to December 2021 were included. Sirolimus was administered twice daily at a dose of 0.8 mg per body surface area (BSA), with dose adjustments to achieve a target drug concentration of 8–12 ng/mL. Adverse drug effects and therapeutic responses were periodically assessed. Treatment efficacy was evaluated based on clinical findings pre- and post-sirolimus administration, absolute volume reduction of lesions through imaging tests (magnetic resonance imaging; MRI), and relative volume reduction adjusted to the patient's BSA. Results: There were 16 females (50.0%) and 16 males (50.0%), with a median follow-up period of 41 months after sirolimus administration. Vascular anomaly types included lymphatic malformations (41%), venous malformations (28%), lymphovenous malformations (19%), and others (12.5%). The most common adverse effect was oral ulcer (6 patients). MRI volumetry revealed volume decreases in 17 patients (53.1%) with 22 patients (71%) exhibited lesion decreases relative to BSA. Notably, 9 patients (28.1%) had markedly decreased volume reduction based on absolute volume, and 12 (38.7%) based on volume compared to BSA. Conclusion Over a 2-year follow-up, sirolimus was effective in treating patients with complicated vascular anomalies, when administered with cautious consideration of side effects. A multidisciplinary approach is needed for evaluating treatment outcomes in these patients, necessitating further long-term research on adverse effects.

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The First Case of Endoscopic Balloon Dilatation for a Newborn With a Congenital Duodenal Web in Korea

Seo-Hee KIM ; Ju Yeon LEE

Advances in Pediatric Surgery.2024;30(1):23-27. doi: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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Morgagni Hernia Masquerading as a Congenital Heart Disease in a Neonate: A Case Report

Sheetal UPRETI ; Shailesh SOLANKI ; Shivani DOGRA ; Jai K MAHAJAN

Advances in Pediatric Surgery.2024;30(1):28-31. doi:10.13029/aps.2024.30.1.28

Morgagni hernia (MH) is a type of congenital diaphragmatic hernia that is rare and without any distinctive presentation. Chest radiographs can miss the diagnosis when solid organs instead of bowel loops are herniated. Echocardiography can perplex the diagnosis instead of aiding if MH is not suspected. We are here discussing the presentation and management of a neonate with MH, which was referred to our institute as a congenital heart disease.

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Perinatally Detected Subhepatic Cysts: Choledochal Cyst? or Cystic Biliary Atresia?

Hee Jin YEON ; Sung Min LEE ; In Geol HO ; Kyong IHN ; Seok Joo HAN

Advances in Pediatric Surgery.2024;30(1):9-13. doi:10.13029/aps.2024.30.1.9

Purpose: Most patients with perinatally detected subhepatic cysts receive information suggestive of a suspected diagnosis of choledochal cyst (CC). However, it is not uncommon to be finally diagnosed with cystic biliary atresia (CBA), a disease with a more unfavorable prognosis. This study aimed to investigate the distribution of the final diagnoses of perinatally detected subhepatic cysts and to compare patients diagnosed with CC and CBA.Method: We performed a retrospective review of patients with subhepatic cysts detected using ultrasonography during perinatal period, between January 2012 and December 2022.Result: This retrospective study included 52 patients with perinatal subhepatic cysts. Of these, 71.2% (37/52) were diagnosed with CC, 15.4% (8/52) with CBA, and 5.8% (3/52) with duplication of the alimentary tract. Only 1.9% (1/52) of the patients were diagnosed with biliary atresia, gallbladder duplication, mesenteric lymphatic malformation, or were normal. Of all patients, 86.5% (45/52) were diagnosed with CC or CBA, with CBA accounting for 17.8% (8/45). There were no statistically significant differences between the CC and CBA groups regarding the gestational age at which the cyst was first detected and the final size of the cyst measured on prenatal ultrasound. Conclusion Subhepatic cysts detected during the perinatal period are typically diagnosed as CC. However, this study revealed that 15.4% of all patients were diagnosed with CBA, despite no significant differences in prenatal ultrasound findings. Therefore, it is essential to consider the possibility of CBA in cases of perinatally detected subhepatic cysts.

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