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Management of complex esophageal problems in children is challenging. We report our experience with the use of esophageal stents in three children with esophageal strictures, leaks, or airway-esophageal fistulae refractory to conventional treatment. The stent played a key role in allowing extubation of a child with a large tracheo-esophageal-pleural fistula and in the resolution of pulmonary infection in a child with esophago-bronchial fistula, both followed by surgery. In the third child, with stricture, stents were complicated with migration, esophageal erosion, and esophago-bronchial fistula. In our experience, esophageal stents were useful mainly as a bridge to definitive surgical repair.

Phosphatase and tensin homolog (PTEN) hamartoma tumor syndrome (PHTS) is characterized by formation of recurrent benign tumors described as PTEN hamartoma of soft tissue that may contain fast flow vascular anomalies (FFVA). The purpose of this study is to review the temporal evolution and management of FFVA in PHTS. A retrospective review of 22 patients (9 males), age 1 to 18 (median 9) years diagnosed with PHTS at a tertiary care pediatric hospital between October 2002 and August 2017 revealed 4 patients with FFVA. Imaging, management, and treatment complications were reviewed. During median follow-up of 8 (range: 4–13) years, ultrasound and magnetic resonance imaging performed for recurrent pain, showed progressive increase in the size of hamartomas and development of new FFVA in three-fourth patients. Medical management included pain medications, oral sirolimus, and physical and psychiatric therapy. Surgical excision of hamartoma (n = 1) resulted in recurrence within 3 months. Between 4 and 24 (average 1.5/year) embolizations were performed per patient. Pain related to FFVA responded well to embolization. Pain secondary to PTEN hamartoma responded poorly to percutaneous sclerosant injection, but demonstrated improvement with sirolimus. There was no correlation between serum sirolimus levels and frequency/timing of recurrence of FFVA/hamartoma. Complications included sclerosant migration into digital arteries (n = 1), subclavian vein stenosis due to glue migration (n = 1), oral mucositis (n = 4), and elevated triglycerides (n = 4). Patients with PHTS present with recurrent pain requiring life-long management with a multi-disciplinary team. Pain due to FFVA responds to embolization, and pain due to hamartoma responds to sirolimus. This improves quality of life, but does not prevent disease progression.

Children with vascular malformations are best managed with a multidisciplinary team of specialists. Interventional radiology may deliver primary treatment such as staged sclerotherapy and embolization for malformations that are poor candidates for primary surgical resection or play a supportive role such as preoperative or intraoperative embolization. A thorough understanding of vascular morphology and flow dynamics is imperative to choosing the best treatment tool and technique. In this review, the author discusses the selection of techniques and tools used to treat vascular malformations based on their angiographic morphology.

Vascular anomalies (VAs) comprise a large variety of individual diagnoses that in different phases of treatment require a diverse number of medical specialists to provide optimal care. Medical therapies include agents usually associated with cancer chemotherapy, such as vincristine, as well more immunomodulatory types of drugs, such as glucocorticoids and sirolimus. These immunomodulating drugs are being successfully applied in cases that are typically categorized as vascular tumors, including kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), as well as some of the more invasive types of vascular malformations (i.e., microcystic lymphatic malformations and blue rubber bleb nevus syndrome (BRBNS). These therapies need to be combined with good supportive care, which often involves anticoagulation, antimicrobial prophylaxis, and comprehensive pain and symptom-relief strategies, as well as appropriate drug monitoring and management of side effects of medical treatment. The optimal care of these patients frequently involves close collaboration between surgeons, interventional and conventional radiologists, medical subspecialists, and nurses.

A 15-year-old girl presented with gallstone pancreatitis. Subsequently, a pseudopancreatic cyst developed that was diagnosed on computed tomographic scan. She underwent a laparoscopic and gastroscopic transgastric cystogastrostomy. In the following report, we describe our novel approach and technique for the above condition.

Background Placing a ventriculo-peritoneal shunt in children with hydrocephalus is the standard of care. Many of these children will require revision of this portion of the shunt for a variety of reasons. Previously, it was thought that in a child with multiple previous ventriculo-peritoneal shunt (VPS) revisions, laparoscopy was contraindicated. This study aims to show that laparoscopy can be used safely and effectively in children with multiple previous ventriculo-peritoneal shunt surgeries. Materials and methods Laparoscopically assisted placement of the peritoneal portion of the ventriculo-peritoneal shunt in children with multiple previous VPS revisions was performed in 8 consecutive children (4 female) with ages ranging from 7 months to 18 years between May 2003 and September 2007. All eight children had undergone more than two previous VPS operations. All shunts were placed in areas free of adhesions and flow was observed under direct visualization. Results All of the procedures were successful; none needed conversion to the standard mini-laparotomy approach. No obvious or occult injury to the abdominal components was noted during hospitalization or during follow-up. Six of 8 patients required lysis of adhesions at the time of the revision. Average length of hospital stay was 2.6 days and no revisions of the abdominal portion of the VPS have been required by any of the 8 patients after laparoscopic revision. Previously unknown complications of shunt surgery were corrected in 1 of 8 children. Conclusions Laparoscopic placement of the peritoneal portion of a ventriculo-peritoneal shunt can be done safely and effectively in children with multiple previous VPS revisions due to improved visualization and placement of the shunt tip in a virgin area of the abdomen. Additionally, any known or unknown complications from previous VPS surgeries can be corrected with the laparoscopic approach. When combined with the reduction in pain, shorter hospital stay, and fewer immediate and future complications, this is the procedure of choice for patients requiring revision VPS surgeries in our hospital.