Purpose
Anorectal malformations (ARMs) represent a wide spectrum of anomalies with various presentations and associated anomalies. The management of ARMs is still controversial and various managements for ARMs have been proposed. The aim of this study was to identify the status of ARMs in Korea and to analyze the data regarding classification, treatment and functional outcomes of ARMs among the Korean Association of Pediatric Surgeons (KAPS) members.
Methods
The KAPS has conducted a national survey for ARMs in 1999 and 2015 to analyze the differences and changes in the classification, treatment, and functional outcomes of ARMs.
Results
A total of 295 and 619 ARMs patients were enrolled in the second and third national survey, respectively. The most common type among male and female was the low type in the second national survey and low defects (cutaneous fistula, anal stenosis, membrane) in the third national survey. Most common associated anomalies were genitourinary system in the second national survey and cardiovascular system in the third national survey. Various surgical options including anoplasty, posterior sagittal anorectoplasty (PSARP) and laparoscopic surgery with or without colostomy were performed. Unfortunately, comparison regarding functional outcomes between 2 national surveys was not possible due to the use of different classification and functional assessment criteria.
Conclusion
We suggest making an ARMs registry at a national level so that multicenter functional outcome data can be collected for a better understanding and management of this rare anomaly.

Purpose
To report the clinical progress and findings seen in pediatric airway surgeries performed by a single pediatric surgeon.
Methods
We retrospectively reviewed 32 pediatric patients with various airway diseases who underwent surgical correction performed by a single pediatric surgeon between January 2005 and March 2021. Surgical indications depended on the presence and severity of respiratory symptoms. Surgical results were considered good if symptoms improved and normal respiration was possible without dependence on a ventilator or tracheostomy, without postoperative complications, at the last postoperative follow-up.
Results
A total of 32 patients who underwent airway surgery were diagnosed with 3 types of surgical airway diseases: tracheomalacia (n=19), subglottic stenosis (n=10), and tracheal stenosis (n=3). In the treatment for tracheomalacia, surgical efficacy was 88.2% for aortopexy and 100.0% for tracheopexy. The postoperative mortality rate of slide thyrocricotracheoplasty for subglottic stenosis and slide tracheoplasty for tracheal stenosis was 20.0%. The age at the time of all airway surgeries ranged from 1.0 to 127.6 months (median, 4.8 months). The postoperative follow-up period ranged from 1.2 months to 16.6 years (median, 4.5 years).
Conclusion
Severe pediatric airway diseases with obstructive symptoms are life-threatening and require surgical treatment. Pediatric airway surgery performed in this study by a single pediatric surgeon had similar or better outcomes than in previous studies. However, more accurate diagnostic methods and improved surgical procedures for pediatric airway diseases require development.

Purpose
Although total parenteral nutrition (TPN) is essential for patients with intestinal failure, intensive monitoring and management are required to provide sufficient nutrition and prevent complications. The adverse effects of prolonged TPN >5 years were evaluated in pediatric patients, and preventative methods and treatments were analyzed.
Methods
Pediatric surgery patients at a single center who had received parenteral nutrition (PN) for more than 5 years at a single center were analyzed retrospectively. Adverse effects and treatments were determined by review of their medical records. Complications were defined as catheter-related, metabolic, or nutritional.
Results
Seven patients receiving long-term PN had short bowel syndrome (SBS), including two each with necrotizing enterocolitis, congenital atresia, and pseudo-obstruction, and one with Hirschsprung’s disease. Six patients had a mean 6.4 ± 4.4 line infections (median: 6, range: 3–15). Central venous catheters were replaced a mean 7.3 ± 4.5 times (median: 8.5, range: 1–12). None of these patients had bilirubin concentrations above 2 mg/mL, and none experienced acute or chronic kidney injuries. Six children received vitamin D supplements, and one experienced rickets. Laboratory tests revealed a temporary lack of zinc or selenium, but no associated clinical symptoms. None had a vitamin B12 or folate deficiency. All seven patients were hospitalized for electrolyte imbalance a median 2.5 times (range: 1–17); all recovered with hydration and TPN. At the time of this writing, all seven patients remained alive, with below-average growth, and two were treated with growth hormones.
Conclusion
Appropriate catheter insertion, guardian education and management, as well as active responses to complications, are important-required to safely maintain long-term parenteral nutrition.

Approximately 5% of Wilms tumors are bilateral. Multidisciplinary care including surgery, chemotherapy, and radiation has greatly improved the survival rates. With long-term survivorship, treatment should be planned to ensure quality survival. Nephron-sparing surgery (NSS) is now a recommended alternative to bilateral nephrectomy with renal replacement therapy in bilateral Wilms tumors. However, the feasibility of bilateral NSS is dependent on multiple factors like the size, location of the tumor, and response to chemotherapy. In advanced bilateral tumors, a) prolonging preoperative chemotherapy to achieve optimum response for performing a salvageable renal surgery and b) administering chemotherapy in between the staged nephrectomies, is an effective strategy. Response assessment with imaging will help in a) selection of appropriate surgery, b) timing and c) sequencing of surgery. We present a patient with bilateral Wilms tumor managed with neoadjuvant chemotherapy followed by staged bilateral NSS followed by adjuvant chemotherapy at our institute. The patient is alive with a follow-up of 5 years.

Entomophthoromycosis is a rare fungal infection, infecting the immune competent children, in the tropical and sub-tropical areas. It is caused by Basidiobolus ranarum, which belongs to the order Entomophthoral. The diagnosis is delayed, as it presents with non-specific symptoms. The disease occurs following a trivial injury or insect bites and therefore presents as subcutaneous nodules over the trunk, buttock and thighs. Gastrointestinal (GI) infection and invasive spread are very rare. Very few cases of GI disease and liver entomophthoromycosis have been reported in the English literature. The diagnosis is confirmed on histopathology and fungal cultures. We discuss a case of liver entomophthoromycosis in a 3-year-old child, who presented with non-specific symptoms and palpable lump in the epigastric region. The child was worked up for malignancy and underwent left hepatectomy. Later, the histopathology report revealed entomophthoromycosis. Delay in diagnosis can be fatal. Entomophthoromycosis is an emerging fungal infection and should be an important differential in patients presenting with vague symptoms and lump abdomen.

Hydatid disease and tuberculosis are both endemic in India. The involvement of the kidney in any of these diseases is rare and co-occurrence of both pathologies at the same time is extremely rare. The common characteristics of both conditions i.e., remain asymptomatic for a long duration and present with non-specific symptoms make the diagnosis challenging. Moreover, the treatment of both pathologies at the same time requires multifaceted medical management and surgical intervention at the optimal time. Here, we are describing a case of renal hydatid with tuberculosis in a child, its presentation and management along with the review of pertinent literature.