The Korean Association of Pediatric Surgeons (KAPS) performed a nationwide survey on sacrococcygeal teratoma in 2018.
The authors reviewed and analyzed the clinical data of patients who had been treated for sacrococcygeal teratoma by KAPS members from 2008 to 2017.
A total of 189 patients from 18 institutes were registered for the study, which was the first national survey of this disease dealing with a large number of patients in Korea. The results were discussed at the 34th annual meeting of KAPS, which was held in Jeonju on June 21–22, 2018.
We believe that this study could be utilized as a guideline for the treatment of sacrococcygeal teratoma to diminish pediatric surgeons' difficulties in treating this disease and thus lead to better outcomes.
This study aimed to compare the outcomes of open fundoplication (OF) and laparoscopic fundoplication (LF) in children with gastroesophageal reflux disease (GERD).
We retrospectively reviewed the electronic medical charts of pediatric patients who underwent fundoplication for GERD between January 2005 and May 2018 at the Korean tertiary hospital. Patient characteristics, operation type, associated diagnosis, operation history, neurologic impairment, postoperative complication, recurrence, and operation outcomes were investigated. The Mann-Whitney U test or Student's t-test was used to evaluate continuous data as appropriate. The χ2 test was used to analyze categorical data.
A total of 92 patients were included in this study; 50 were male and 42 were female. Forty-eight patients underwent OF and 44 patients underwent LF. Patient characteristics, such as sex ratio, gestational age, symptoms, neurological impairment, and history of the previous operation were not different between the two groups. A longer operative time (113.0±56.0 vs. 135.1±49.1 minutes, p=0.048) was noted for LF. There was no significant difference in operation time when the diagnosis was limited to only GERD, excluding patients with other combined diseases. Other surgical outcomes, such as intraoperative blood loss, transfusion rate, hospital stay, and recurrence rate were not significantly different between the 2 groups. The complication rate was slightly higher in the OF group than in the LF group; however, the difference was not significant (20.8% vs. 11.4%, p=0.344).
LF is as safe, feasible, and effective as OF for the surgical treatment of GERD in children.
Esophageal atresia (EA) is a diverse disease entity. We present a case of long gap EA without fistula corrected through totally laparoscopic and thoracoscopic esophageal replacement using gastric tube. A male baby weighing 3,000 g, with suspicion of EA, was born at gestational age of 37+6 weeks. Gastrostomy was made at an age of two days; seven months later, definite operation was planned. We determined to perform the gastric tube replacement due to long gap revealed by fluoroscopy. Gastric mobilization, gastric tube formation, and pyloroplasty were performed laparoscopically. An isoperistaltic 9 cm gastric tube was made using 2 Endo GIA 45, and interrupted end-to-end esophago-esophagostomy was performed thoracoscopically. With laparoscopy, gastropexy to the diaphragm was performed through the interrupted suture. Operation time was 370 minutes; there was no intraoperative event. Postoperative course was uneventful. He underwent esophageal balloon dilatation due to anastomosis stenosis in the months after surgery.
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.
Total proctocolectomy with ileal pouch-anal anastomosis (T-IPAA) in childhood is a surgical procedure mainly applied to familial adenomatous polyposis (FAP) or ulcerative colitis (UC), but it can be applied to non-FAP/non-UC disease (NFNU). Studies regarding the role of T-IPAA who underwent the operation in childhood, especially in terms of long-term gastrointestinal function, complications, and quality of life (QOL) are limited. The aim of this study was to evaluate the characteristics of patients receiving T-IPAA and to compare their bowel function outcomes and QOL.
Patients aged ≤18 years at the time of T-IPAA were included. Their medical records were retrospectively reviewed. Krickenbeck classification, Cleveland Clinic Incontinence (CCI) score, 36-item Short-form Health Survey Questionnaire, and Gastrointestinal Quality of Life Index were used for the evaluation of bowel function and QOL. The median follow-up period was 9.8 years.
Of the 25 patients, 9 had FAP, 9 had UC, and 7 had NFNU. NFNU include 3 of Hirschsprung disease, 2 of intestinal neuronal dysplasia, and 2 of imperforate anus. The median age at T-IPAA was 17.8, 14.2, and 9.3 years for FAP, UC, and NFNU, respectively (p=0.001). Bowel function was satisfactory in terms of voluntary bowel movement (VBM), soiling, and constipation. VBM and constipation were not different between the groups, but soiling was most in NFNU (100%, p=0.047). However, QOL was best in the NFNU group in surveys (p=0.034 and 0.004, respectively).
T-IPAA could be safely applied not only for FAP and UC but also for other diseases in selective cases, with caution.
Preoperative ultrasonography (USG) in pediatric inguinal hernia has controversy. In this study, we analyzed the cases of pediatric inguinal hernia with/without preoperative USG and discussed whether USG is necessary.
We reviewed medical records of 1,441 patients who underwent inguinal hernia repair in Seoul National University Children's Hospital between January 2011 and August 2016 retrospectively.
Male were 69.3% and age at operation was 37.8±36.5 months old. There were 150 patients (10.4%) performed USG preoperatively. The department ordered to perform USG included department of surgery (n=71), emergency medicine (n=42), pediatrics (n=26), urology (n=10) and outside hospital (n=1). The reasons of performing USG included evaluation for hernia laterality (n=82), incarceration (n=28), testis (n=15), request of parents (n=14), scrotal mass (n=6) and incidentally found during evaluation for another disease (n=5). Excepting 5 cases of incidental finding, of 145 cases with USG, 12 (8.3%) cases changed the surgical plan; change to bilateral repair from unilateral repair (n=5), emergency operation due to incarceration (n=4) which include 1 salpingo-oophorectomy, 1 open abdomen surgery and 2 hernia repair after reduction of ovary, change to co-operation of orchiopexy (n=2) and change to laparoscopic surgery from open surgery due to herniation of both ovaries into one inguinal canal (n=1). In group without USG (n=1,291), 5 patients (0.4%) had unexpected problems during operation; 2 co-operation of orchiopexy because of transverse testicular ectopia (n=1) and right undescended testis (n=1), 2 hypertrophy of major labia and 1 retroperitoneal lymphangioma at inguinal area misdiagnosed as inguinal hernia.
It was difficult to interpret the meaning of preoperative USG because not all patients had performed it. In this study, 10.4% of patients performed USG and 8.3% of them changed surgical plan. About 0.4% of patients without preoperative USG would have benefited from it for surgery if they had performed it. Since the percentage is too low, it is unreasonable to conclude that USG has diagnostic utility in inguinal hernia in this study.
An 18-year-old male patient with cerebral palsy and scoliokyphosis came to the emergency department with abdominal distension and vomiting. He was a situs inversus patient with a feeding gastrostomy tube. Sigmoid volvulus was initially suspected, so rectal tube insertion and endoscopic decompression were attempted, but failed. So he went through explorative laparotomy, and transverse colonic adhesion and twisting around the gastrostomy tube and gastric wall was identified. Adhesiolysis and resection with redundant transverse colon and end-to-end colocolic anastomosis was performed. He discharged with symptom free. Suspecting transverse colonic volvulus is important when the patient has anatomical anomalies and feeding gastrostomy tube. Timely diagnosis with proper radiologic imaging should be made. Surgical resection of the redundant colon is needed for successful management of transverse colonic volvulus.
Congenital esophageal stenosis (CES) is a rare disease that has been reported to occur once in every 25,000 to 50,000 births. According to its etiology, CES is divided into 3 subtypes, tracheobronchial remnants (TBR), fibromuscular hypertrophy (FMH) and membranous diaphragm (MD). Symptoms begin at the weaning period and the introduction of solid food around 6 months with dysphagia and vomiting. Esophagography is first screening test and endoscopic ultrasonography plays important roles to diagnose subtypes deciding therapeutic plan. TBRs were generally treated with surgical resection and end-to-end anasotomosis, whereas FMH and MD had good response rate to endoscopic or radiologic guided dilatation. This article reviews the literature on the etiology, clinical course, diagnosis and management of CES including recent opinion.
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.
Anorectal duplications account for only 5% of gastrointestinal duplications, and cases with involvement of the anal canal are much rarer. Nearly all anorectal duplications are posterior to the rectum; duplications located anterior to the normal rectum are highly unusual, and only a few cases have been reported. We report the case of an anterior anorectocolonic duplication presenting as a rectovaginal fistula in a 2-month-old infant. After diagnosis, the duplication was excised completely without further intestinal complications.
Thyroid cancer is a rare disease in pediatric population, but its incidence rate is increasing. The aim of this report is to present a single institution experience of pediatric thyroid cancer and to identify clinical features, predisposing factors, and postoperative course of pediatric thyroid cancer.
We retrospectively reviewed 35 pediatric patients who underwent operation due to thyroid cancer at Seoul National University Children's Hospital between May 1997 and January 2017. The median follow-up period was 70 months (range, 5–238 months).
The mean age at operation was 12.0±5.91 years and 27 patients were female. The underlying conditions in patients included history of chemoradiotherapy for previous other malignancies (n=4), hypothyroidism (n=3), history of chemotherapy (n=2), family history of thyroid cancer (n=1) and history of radiation therapy (n=1). The initial symptoms were palpable neck mass (n=21) and incidental findings (n=11). Total thyroidectomy (n=30) or unilateral lobectomy (n=5) were performed. There were 15 postoperative complications including transient hypocalcemia in 14 patients and Horner's syndrome in 1 patient. The most common pathologic cell type was papillary thyroid cancer (n=29). Extrathyroid extension and lymph node invasion were found in 25 patients and 27 patients, respectively. Thirteen patients showed multifocality. During follow-up period, 5 patients underwent additional operation because of tumor recurrence in lymph nodes. Lung metastasis was detected in 3 patients at the time of diagnosis and in 3 patients during follow-up period. The mortality rate was zero and mean disease-free survival was 83.7±47.9 months.
Pediatric thyroid cancer has lower mortality rate and recurrence rate as seen in this study despite the advanced stage at diagnosis. A thorough follow-up of patients with an underlying condition such as history of chemoradiotherapy and understanding new pediatric guideline can be helpful to maximize patients' survival and prognosis.
Minimally invasive surgery (MIS) in abdomen and thorax has been widely accepted for pediatric diseases. Thoracoscopic surgery has the advantage of less pain, better cosmetic outcomes and less musculoskeletal sequelae in comparison to open surgery. We would like to share our initial experience with thoracoscopic surgery performed by one pediatric surgeon.
We performed a retrospective review of patients who underwent thoracoscopic surgery by one pediatric surgeon between April 2010 and August 2017 in Department of Pediatric Surgery, Seoul National University Children's Hospital.
There were totally 18 cases; 8 cases for esophageal atresia, 3 cases for congenital diaphragm hernia, 2 cases for diaphragm eventration, 2 cases for esophageal duplication cyst, 2 cases for pleural mass and 1 case for esophageal bronchus. At the operation, median age was 9.5 months (range, 0-259 months) and median body weight was 9.4 kg (range, 1.9-49.4 kg). Median operative time was 157.5 minutes (range, 45-335 minutes). There was no case of open conversion and 2 cases of minor leakage at anastomosis site in case of esophageal atresia. Median follow-up month was 5 months (range, 0-87 months). During follow-up, 4 cases of esophageal atresia showed anastomosis site narrowing and average 2.5 times (range, 1-5 times) of esophageal balloon dilatation was done.
We performed thoracoscopic surgery in case of esophageal, diaphragm disease and pleural mass. Thoracoscopic surgery can be an effective and feasible option of treatment for well-selected pediatric patients of intra-thoracic disease including esophagus, diaphragm and mediastinum disease.
Soft tissue hemangioendothelioma (STHE) is a rare vascular tumor, which has a similar prognosis to borderline malignancy. The disease is poorly understood in pediatric cases because of its low incidence; therefore, we investigated treatment strategies for STHE in children.
We retrospectively analyzed 8 patients with STHE, who were pathologically confirmed between January 1995 and June 2015. The median duration of follow-up was 72 months.
Five were male and the median age at the time of surgery was 1.2 years. Six patients presented with a palpable mass, and 2 patients experienced facial paralysis. The median tumor size was 4.0 cm. The following tumor locations were observed head (2 patients), neck (2 patients), chest wall (1 patient), sacrococcyx (1 patient), upper limb (1 patient), and lower limb (1 patient). The patients underwent either microscopic complete resection (R0) (3 patients), macroscopic complete resection (R1) (2 patients), or macroscopic incomplete resection (R2) (3 patients). After histopathological examination, 6 patients were diagnosed with kaposiform hemangioendothelioma (HE), one with retiform HE, and one with epithelioid HE. Postoperative sequelae occurred as gait disturbance, hearing impairment, and vocal cord palsy. Tumor recurrence or regrowth occurred in 4 patients. These patients underwent reoperation and IFN therapy; however, in the patient with epithelioid HE, metastasis to the scalp occurred after these therapies. The patient with the head tumor who underwent R2 resection, underwent resection three more times, but died 11 months after the first surgery.
When treating STHE in children, R0 resection should be first considered, but recurrence and metastasis should be monitored depending on the size, pathology, and location of the lesion. When major sequelae are expected, function-preserving surgery could be considered, depending on tumor location, size, and nearby organs.
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.
It has been known that extramedullary hematopoiesis occurring after birth can be developed in various diseases, and it is often found in hematologic diseases. Among these, congenital dyserythropoietic anemia is a rare disease characterized with increase of ineffective hematopoiesis and morphological abnormalities of erythroblasts. In congenital dyserythropoietic anemia, extramedullary hematopoiesis is very rare and only a few cases have been reported. Although treatment is not required if there is no symptom in extramedullary hematopoiesis, surgery or radiation therapy is effective in case that there is symptom or unresponsive anemia despite blood transfusion. This case report is about surgical treatment for extramedullary hematopoiesis in 23-year-old patients diagnosed of congenital dyserythropoietic anemia.
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.
Farber disease (FD) is a rare lysosomal storage disorder that shows autosomal recessive inheritance. We report the case of a 58-month-old girl with FD, who was misdiagnosed with epithelioid hemangioendothelioma. The patient had undergone five surgeries for sacrococcygeal masses and three surgeries for scalp masses owing to misdiagnosis. Here, we describe this rare case of FD.
Number of pediatric cholecystectomy has been recently showing a gradually increasing trend. The purpose of this study was to investigate the clinical features of patients who underwent pediatric cholecystectomy, and the latest trend in cholecystectomy.
In the present study, we conducted a retrospective chart review on 47 patients who had undergone cholecystectomy at a single center. The entire patient population was divided into two groups, according to the time of cholecystectomy (early group, January 1999 to December 2006; late group, January 2007 to August 2014).
The comparison between the early and late groups showed that the number of cholecystectomy increased from 13 to 34 cases representing a 2.6-fold increase. The mean patient age also increased from 5.94±4.08 years to 10.51±5.57 years (p=0.01). Meanwhile, laparoscopic surgery also increased from 15.4% to 79.4%, respectively (p<0.001). However, sex, mean body mass index, comorbidities, indications of cholecystectomy, and previous total parenteral nutrition were not statistically significant.
The results of this study showed that pediatric cholecystectomy cases are increasing, particularly in the 10 to 19 years age group and laparoscopic cholecystectomies are also being performed at an increasing rate. When the patients were compared according to the time of cholecystectomy, there were no differences in other risk factors or indications for cholecystectomy.
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.
Abdominal actinomycosis is a rare and chronic progressive disease, especially in children. Clinically, it has non-specific symptoms and diagnostic findings as well as low prevalence, making it very difficult to diagnose prior to intraoperative pathological confirmation. For this reason, abdominal actinomycosis is commonly misdiagnosed as appendicitis. After the histopathological diagnosis of abdominal actinomycosis is made, patients should be administered an appropriate antibiotic such as penicillin. Here we describe a case of appendiceal actinomycosis in an 18-year-old girl who was initially diagnosed with acute appendicitis.
Nodular hidradenoma was diagnosed in a 29-month-old girl on her axilla. Hidradenoma, sometimes designated as acrospiroma, is a benign sweat gland neoplasm, which mostly occurs in adults. Very few cases of hidradenoma have been documented in children in their first decade of life. This case demonstrates that when a child develops a skin nodule, nodular hidradenoma can be a diagnostic option.
Neonatal neuroblastoma (NBL) is the most common malignant tumor in neonates, but there have been few studies about it. The purpose of this study was to investigate the clinical features of NBL and to compare prenatal and postnatal diagnosed groups.
Nineteen patients who were diagnosed with NBL prenatally or within 28 days after birth from February 1986 to February 2013 in Seoul National University Hospital were enrolled in the study. The patients were categorized according to the International Neuroblastoma Staging System (INSS) and Children's Oncology Group (COG). Retrospective medical-record reviews were performed on these patients. The operative date, complication, pathological stage, and overall survival of the prenatally diagnosed group and the postpartum diagnosed group were compared.
Tumor was detected via prenatal ultrasonography in 8 patients (42.1%), and 11 patients (57.9%) were diagnosed within 28 days after birth. Based on INSS, the patients were divided into the stage I (n=8), stage II (n=1), stage III (n=3), stage IV (n=4), and stage IVs (n=3) groups, respectively. Based on COG, on the other hand, the patients were divided into the low-risk (n=8), intermediate-risk (n=8), and high-risk (n=3) groups. The postoperative complication rate was 29%. One patient died from complications from chemotherapy. The other 18 patients' mean follow-up period was 77.7 months. The differences between the postoperative complication rate, proportion of early-stage tumor, and overall survival of the prenatal and postnatal groups were not statistically significant (p=0.446, p=0.607, p=0.414).
NBL showed favorable outcomes but relatively higher postoperative complications. There seem to be no significant statistical differences in the postoperative complications, proportion of early-stage tumor, and overall survival between the prenatally diagnosed group and the postpartum diagnosed group.
Debates exist about the appropriate treatment for pancreatic trauma in children. We intended to examine the safety of the operation of pancreatic trauma in children. This is a retrospective study of 13 patients, younger than 15, who underwent surgery for pancreatic trauma, between 1993 and 2011 in Seoul National University Children's Hospital. Medical records were reviewed for mechanism of trauma, clinical characteristics, radiological findings, operation and outcomes. Organ injury scaling from the AAST (American Association for Surgery of Trauma) was used. All injuries were caused by blunt trauma. Patients with grade III, IV, and those who were difficult to distinguish grade II from IV, underwent surgery due to severe peritonitis. Three patients with grade II were operated for reasons of mesenteric bleeding, tumor rupture of the pancreas, and progression of peritonitis. Distal pancreatectomy was performed in 10 patients and subtotal pancreatectomy and pylorus preserving pancreaticoduodenectomy in 1 patient each. The remaining one underwent surgical debridement because of severe adhesions. The location of injury, before and after operation, coincided in 83.3%. The degree of injury, before and after the operation, was identical in all the patients except for those who were difficult to tell apart grade II from grade IV, and those cannot be graded due to severe adhesion. Postoperative complications occurred in 23.1%, which improved with conservative treatment. Patients were discharged at mean postoperative 12(range 8~42) days. Even though patients with complications took longer in time from diagnosis to operation, time of trauma to operation and hospital stay, this difference was not significant. In conclusion, When pancreatic duct injury is present, or patient shows deterioration of clinical manifestation without evidence of definite duct injury, or trauma is accompanied by other organ injury or tumor rupture, operative management is advisable, and we believe it is a safe and feasible method of treatment.
Immature ganglion cell (IGC) is known for its relationship with intestinal motility and its impact on postoperative functional outcomes of Hirschsprung's disease (HD). There are few studies on the relationship between intestinal dysmotility and IGC in HD patients. 67 patients pathologically diagnosed with HD and who received definitive operation in Seoul National University Children's Hospital from 2010 to 2011 were included. 10 patients were excluded due to inadequate immunohistochemical staining results. The proximal end of resected ganglionic segment was evaluated with immunohistochemistry examination with MAP-2, a marker of ganglionic cells and bcl-2, a marker of IGCs The median age at operation was 155 (15-4678) day-old. 55 (96.5%) patients positive for bcl-2, were regarded as having IGC, and 2 (3.5%) patients positive for MAP-2 but negative for bcl-2, were regarded as having only mature ganglion cells. In the bcl-2 positive group, there were 7 patients (12.7%) with constipation, 15 patients (27.3%) with soiling, 3 patients (5.5%) with perianal excoriation and 6 patients (10.9%) with medication use. In bcl-2 negative group, intestinal dysmotility was not seen. There was no statistical significance in the two groups. Considering that HD is diagnosed at a young age, the rate of IGC present is very high and it might be inappropriate to relate IGC to functional outcome at young ages.
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