Mediastinal infantile hemangioma with spinal canal extension and extensive gastrointestinal involvement complicated by respiratory failure.

Infantile hemangioma is the most common soft tissue tumor of infancy. Extensive organ involvement is rare. This report describes an infant with biopsy confirmed infantile hemangioma with diffuse organ involvement causing anemia and failure to thrive. Treatment was initiated with propranolol and led to initial improvement; however, course was complicated by several episodes of respiratory failure secondary to pulmonary edema. Propranolol therapy was interrupted for several months while patient was maintained on a diuretic regimen and treated with vincristine and high-dose corticosteroids. Patient was transitioned back to propranolol and is clinically thriving with objective improvement on radiographic imaging.

Kaposiform hemangioendothelioma of the bone in children and adolescents.

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor that mainly occurs during infancy or early childhood. Approximately 70% of cases are complicated by Kasabach-Merritt phenomenon. Although osseous extension of the primary lesion is relatively common, primary bone involvement by KHE is rare. Given the paucity of literature on primary KHE of the bone, we report a case series of primary KHE of the bone treated at our institution and describe the clinical presentation, radiologic and pathologic findings, management and outcomes.

Catastrophic congenital hemangioma with severe coagulopathy leading to fatal cardiac failure: Case report and review.

We present a complex case of a neonate, delivered urgently for hydrops fetalis, with a large vascular mass of the extremity, diagnosed postnatally as a congenital hemangioma. The patient suffered immediate cardiac compromise and severe coagulopathy atypical for the diagnosis and subsequently died from these complications. Treatment was imperative but challenging due to a lack of a standardized treatment approach and few historical reports of equally critically ill patients. In this report, we review potential medical and surgical interventions and discuss treatment considerations in similar, life-threatening cases of congenital hemangiomas.

In utero Fetal Intubation for a Large Neck Mass: A Minimally Invasive EXIT Option.

Fetuses with obstructive neck and orofacial lesions have been delivered via an ex utero intrapartum treatment (EXIT) procedure to facilitate securement of the airway while on placental circulation. Pregnancy-related cardiovascular changes and technical issues unique to an EXIT procedure increase fetal and maternal risks relative to a standard cesarean section. In order to circumvent such issues, fetal endoscopic intubation has been proposed. We report a case of a fetus with a large neck mass (mixed solid and multiloculated cystic lesion measuring 9.2 × 5.3 × 8.5 cm, neck hyperextension, protruding tongue, and serial gagging movements) that was successfully intubated in utero and delivered at 36 weeks and 0 days via standard cesarean section, thereby avoiding an EXIT procedure. The risks, benefits, and technical issues of in utero tracheal intubation are reviewed.

Doxycycline Sclerotherapy Is Superior in the Treatment of Pediatric Lymphatic Malformations.

PURPOSE: To evaluate efficacy of sclerotherapy with doxycycline versus sodium tetradecyl sulfate (STS) for treatment of macrocystic and mixed lymphatic malformations (LMs). MATERIALS AND METHODS: This single-center retrospective review identified 41 children (17 boys; 24 girls; age range, 1 month to 15.4 y) who underwent sclerotherapy with doxycycline (n = 32) or STS (n = 9) for macrocystic (n = 31) or mixed (n = 10) LMs. There were 114 treatments performed, averaging 2.8 treatments (range, 1-8 treatments) per patient. Average follow-up time was 10 months (range, 1-59 months). Clinical response was deemed excellent or moderate if > 90% or > 50% of LMs resolved based on visual estimate. RESULTS: With doxycycline, 87% of patients (28 of 32) had excellent or moderate response with an average of 2.8 treatments (range, 1-7 treatments); 13% required subsequent resection. With 3% STS monotherapy, only 55% of patients (5 of 9) had excellent or moderate response with an average of 2.8 treatments (range, 1-8 treatments), and 33% required subsequent resection. Significantly fewer patients treated with STS responded well compared with patients treated with doxycycline (P = .03). Patients treated with STS had significantly longer follow-up than patients treated with doxycycline (27 months vs 6 months, P = .0001). CONCLUSIONS: Doxycycline monotherapy resulted in a high rate of excellent clinical outcomes after a few treatments without increased need for subsequent operative resection. These results support use of doxycycline sclerotherapy as primary treatment for macrocystic and mixed LMs in children.

Evaluation of Postoperative Outcomes After Enterostomy Closure in Low Body Weight Infants: A Multi-Center Retrospective Analysis.

BACKGROUND: The minimum weight for enterostomy closure (EC) in infants remains debated with the current acceptable cut-off of >2 kg. As enterostomy-related complications or high enterostomy output (>30cc/kg/d) may prohibit a premature infant from reaching 2 kg, additional data is needed to evaluate the safety of EC in infants <2 kg. The objective of this study was to evaluate postoperative outcomes in low body weight (<2 kg) infants undergoing EC compared to larger infants. METHODS: We performed a multi-center retrospective analysis from 1/1/2012-12/31/2022 of all infants (age <1 year) who were <4 kg at time of EC. Primary outcomes included postoperative complications and 30-day mortality. Non-parametric analysis was performed using the Kruskal-Wallis one-way analysis of variance and chi-square tests. Univariable logistic regression was performed to identify factors associated with postoperative complications. RESULTS: Of 92 infants, 15 infants (16.3%) underwent EC at <2 kg, 16 (17.4%) at 2-2.49 kg, 31 (33.7%) at 2.5-2.99 kg, and 30 (32.6%) at ≥3 kg. Infants <2 kg at time of EC exhibited higher rates of hyperbilirubinemia (P = .030), neurologic comorbidities (P = .030), and high enterostomy output (P = .041). There was no difference in postoperative complications (P = .460) or 30-day mortality (P = .460) between the <2 kg group and larger weight groups. Low body weight was not associated with an increased risk for developing a postoperative complication (OR: 1.001, 95% CI: 1.001-1.001; P = .032). CONCLUSION: Our findings suggest that EC in infants <2 kg may be safe with comparable postoperative outcomes to larger weight infants. Thus, the timing of EC should be based on the infant's physiologic status, in contrast to a predetermined minimum weight cut-off.

Single-incision laparoscopic resection of ovarian masses in children: a preliminary report.

PURPOSE: Ovarian masses in the pediatric population are commonly resected with a three or four port laparoscopic approach. Single-incision laparoscopic (SIL) resection is an alternative approach. However, there is limited experience with this modality in ovarian mass resection. METHODS: We reviewed SIL ovarian mass resections performed by our group from 2010 to 2012. We evaluated patient demographics, surgery statistics, and hospital course. RESULTS: Six patients were identified with mean age of 14 years. Imaging studies showed cystic masses ranging 4-6 cm in five patients, and 20 cm in one patient. One patient presented with recurrent teratoma. Pathology revealed four benign teratomas, one benign cyst, and one serous cystadenoma. Average operating time was 75 min. All patients had an ovarian-preserving resection. Three patients had cyst spillage, including the one who presented with recurrence (this was the only patient with a subsequent recurrence). Hospital stay averaged 37 h. Narcotic use averaged 9.9 mg of morphine daily. All patients had excellent cosmetic results, and no postoperative complications. CONCLUSIONS: Ovarian cystic mass excision using the SIL approach carries a higher risk of tumor spillage. Although the incidence of malignancy is low, they cannot be conclusively excluded with our current preoperative evaluations. At this time, we recommend SIL resection only for simple cysts with low malignant potential; however, further experience with this procedure will likely improve the risk of tumor spillage in the future.

Colonic venous malformation and portal hypertension: association, management, and review of the literature.

We present a case of an adolescent with lower gastrointestinal bleeding caused by a colorectal venous malformation (VM) with concomitant portal hypertension. After an episode of massive gastrointestinal bleeding, we performed an extended right hemicolectomy and resection of the VM and selective portosystemic shunt. Here, we present the case and review the literature regarding portal hypertension and gastrointestinal vascular malformations. Additionally, we discuss the physiologic and hemodynamic effects of gastrointestinal vascular malformations on the portal system.

Pediatric single incision laparoscopic cholecystectomy: lessons learned in the first 25 cases.

PURPOSE: We are reporting our experience so far with single incision laparoscopic cholecystectomy in children. METHODS: After the approval of the institutional review board, we performed a retrospective chart review of our single port cases from 01/2008 to 10/2009. We used operating room (OR) times, length of stay, as well as IV narcotic use as our outcome measures. Pertinent clinical data were extracted. The single port procedure was performed using a single infra-umbilical incision whereby three 5-mm ports were placed. RESULTS: We identified 25 patients in the single port group (20 females and 5 males). 23 patients in the study group underwent cholecystectomy without intra-operative cholangiogram and one patient had an intra-operative cholangiogram performed. This additional procedure did not add to the overall OR time significantly as compared to simple cholecystectomies. Average OR time was 97.5 min as compared to 71.4 min in the traditional 4-port group. Blood loss was reported as minimal for all cases in both the groups (5-25 ml). There were no intra-operative complications in either group. Mean length of stay was 1.47 days in the study group. All patients in the study group had minimal (1-3 doses) need for intravenous narcotics during their inpatient stay except for one patient, who required more. All patients in the study group had excellent cosmetic results on postoperative follow-up. CONCLUSION: Single incision laparoscopic cholecystectomy is safe and feasible to perform in pediatrics, even in the setting of acute disease.

Minimally invasive esophagectomy and gastric pull-up in children.

PURPOSE: Minimally invasive esophagectomy and gastric pull-up is a widely accepted method in adults. However, the experience in the pediatric population is limited. Minimally invasive esophagectomy represents a new alternative technique to the conventional open approach. We wish to report our small case series of minimally invasive esophagectomy and gastric pull-up in pediatric patients. The aim of the study is to evaluate the feasibility, safety, and outcomes of the procedure. METHODS: Three patients (2 girls and 1 boy) with average age 46 months (34-57 months) and average weight 12.6 kg (11-15 kg) underwent the procedure. The indications for esophagectomy were esophageal stricture from caustic ingestion (2 patients) and failed repair of esophageal atresia (1 patient). RESULTS: Average operative time was 7 h (0519-0752 hours). There were no intraoperative complications with the average blood loss of 50 cc (5-125 cc). No anastomotic leaks were noted on the initial esophagrams that were obtained on postoperative day five or six. One patient developed a cervical wound infection on postoperative day seven due to a retained piece of Penrose, which required a neck exploration, removal of foreign body and repair of a small leak. One patient developed an anastomotic stricture at the 7-month follow-up. She was successfully treated with two balloon dilatations. One patient developed a delayed esophagogastric anastomotic leak at 3 months. The leak spontaneously closed after surgical drainage. At average of 22-month follow-up (15-36 months), all patients were eating regular food with excellent weight gain. CONCLUSION: Minimally invasive esophagectomy and gastric pull-up is technically challenging but feasible and safe with acceptable outcomes. However, further study is needed to further validate the approach.

Management of cervicofacial lymphatic malformations requires a multidisciplinary approach.

BACKGROUND/PURPOSE: Cervicofacial lymphatic malformations (CFLM) are rare, potentially life-threatening vascular anomalies, yet reports on multidisciplinary treatment strategies are lacking. We evaluated outcomes for CFLMs following sclerotherapy, surgical resection, and/or medical management. METHODS: We identified children with a CFLM at a vascular anomalies center from 2004 to 2019. EXCLUSION CRITERIA: retro-orbital malformations, untreated malformations, patients without follow-up. Primary clinical outcome was contour improvement, with significance defined as LM volume reduction of >50% by cross-sectional imaging. RESULTS: Sixty-three children met inclusion criteria: 35 with macrocystic CFLMs, six with microcystic CFLMs, and 22 with mixed-type malformations. Mean post-intervention follow-up was 27.5 months. Fifty-eight patients underwent sclerotherapy (median: two treatments). Doxycycline and/or bleomycin were used in 95% of patients. After sclerotherapy, 97% of macrocystic CFLMs improved significantly compared to 82% of mixed and 67% of microcystic lesions. Sixteen children underwent surgical resection with 75% significantly improving; two additional patients were successfully treated with sclerotherapy after debulking surgery. Six children received sirolimus for microcystic disease, of which 33% significantly improved. CONCLUSION: Sclerotherapy is very effective for macrocystic components of CFLMs, albeit less so for microcystic disease. Microcystic CFLMs frequently require surgical resection. Sirolimus is a helpful therapeutic adjunct, particularly for microcystic lesions, but more study is needed. LEVEL OF EVIDENCE: Level II, prognosis study.

A multidisciplinary approach to management of abdominal lymphatic malformations.

BACKGROUND/PURPOSE: Abdominal lymphatic malformations (LM) are a rare subset of vascular anomaly caused by abnormal development of the lymphatic system. They are classified as macrocystic, microcystic or combination macrocystic and microcystic. Surgical resection, percutaneous sclerotherapy, and medical therapy are all employed to treat these complex and often symptomatic lesions. No standardized treatment algorithm exists currently. The purpose of this study was to establish a multidisciplinary treatment approach to abdominal LMs. METHODS: A retrospective observational study was conducted from 2013 to 2019 on patients with abdominal LMs at a single tertiary children's hospital vascular anomalies center. Demographics, imaging, and treatment modality were recorded. Clinical and/or radiographic response to the primary treatment modality as well as complications was the outcomes measured. RESULTS: Nineteen patients (12 macrocystic, 5 microcystic and 2 combined) were identified, with a median age at diagnosis of 2.2 years (range 0.1-20.8 years). Sclerotherapy was the most common primary treatment, followed by surgical resection and sirolimus. No difference in clinical response (p = 0.58) or complications (p = 0.31) was observed based on primary treatment or subtypes. CONCLUSIONS: Based on our institutional experience, we propose an LM subtype-based treatment algorithm for abdominal LMs. It employs a multidisciplinary approach, and results in satisfactory patient outcomes with minimal complications. LEVEL OF EVIDENCE: Level III, retrospective comparative study.

Management challenges of a large upper extremity vascular malformation in a patient with capillary malformation-arteriovenous malformation syndrome.

We describe a 17-year-old boy with capillary malformation-arteriovenous malformation syndrome and a massive vascular malformation of the right chest wall, shoulder, and upper arm. Persistent growth of the malformation caused cutaneous ulcerations and recurrent massive bleeding episodes. We proceeded with a modified shoulder disarticulation preceded by ligation of the subclavian artery and innominate vein by median sternotomy. After a staged debulking resection of the residual chest wall arteriovenous malformation with rotational transverse rectus abdominis myocutaneous flap coverage, the patient was discharged home safely. This report demonstrates that a multidisciplinary approach is critical for management of life-threatening complications in capillary malformation-arteriovenous malformation patients.

Single-incision laparoscopic cholecystectomy in a pediatric population: a preliminary report.

Laparoscopic cholecystectomy has become a standard procedure for treatment of gallbladder diseases. The operation is performed through a four-trocar technique. Single-incision laparoscopy (SIL) has recently gained popularity. The purpose of our study was to review our cases of SIL cholecystectomy and to evaluate the safety and feasibility of this technique. After the approval of the Institutional Review Boards, we performed a retrospective chart review of our SIL cholecystectomy cases performed between January 2008 and August 2009. Pertinent clinical data were extracted. The outcomes were reported as operating room time, intraoperative and postoperative complications, length of stay, and intravenous narcotic use. We identified 24 patients (19 females and five males) with a mean age of 15 years. Most patients (67%) had a diagnosis of symptomatic cholelithiasis. Two patients had gallstone pancreatitis, three had acute cholecystitis, and one had a hydropic gallbladder. Two patients had an intraoperative cholangiogram performed. Average operating room time was 97.5 +/- 34.5 minutes (range, 65 to 145 minutes). There were no intraoperative complications. All patients had minimal (one to three doses) need for intravenous narcotics. All patients have had excellent cosmetic results on postoperative follow-up. SIL cholecystectomy in children is safe and feasible, even in the setting of acute cholecystitis and the need for cholangiogram.

Tezosentan, a novel endothelin receptor antagonist, markedly reduces rat hepatic ischemia and reperfusion injury in three different models.

This study investigated the effects of dual endothelin (ET) receptor blockade in rat models of liver ischemia and reperfusion injury (IRI). Three models of IRI were used: (1) in vivo total hepatic warm ischemia with portal shunting for 60 minutes with control (saline) and treatment groups (15 mg/kg tezosentan intravenously prior to reperfusion), (2) ex vivo hepatic perfusion after 24 hours of cold storage in University of Wisconsin solution with control and treatment groups (10 mg/kg tezosentan in the perfusate), and (3) syngeneic liver transplantation (LT) after 24 hours of cold storage in University of Wisconsin solution with control and treatment groups (10 mg/kg tezosentan intravenously prior to reperfusion). Tezosentan treatment significantly improved serum transaminase and histology after IRI in all 3 models. This correlated with reduced vascular resistance, improved bile production, and an improved oxygen extraction ratio. Treatment led to a reduction in neutrophil infiltration and interleukin-1 beta and macrophage inflammatory protein 2 production. A reduction in endothelial cell injury as measured by purine nucleoside phosphorylase was seen. Survival after LT was significantly increased with tezosentan treatment (90% versus 50%). In conclusion, this is the first investigation to examine dual receptor ET blockade in 3 models of hepatic IRI and the first to use the parenterally administered agent tezosentan. The results demonstrate that in both warm and cold IRI tezosentan administration improves sinusoidal hemodynamics and is associated with improved tissue oxygenation and reduced endothelial cell damage. In addition, reduced tissue inflammation, injury, and leukocyte chemotactic signaling were seen. These results provide compelling data for the further investigation of the use of tezosentan in hepatic IRI.

Thoracoscopic pneumonectomy for severe bronchiectasis in a 9-year-old female.

INTRODUCTION: Thoracoscopic total pneumonectomy has not been previously described in the pediatric surgical literature. In this paper, we describe a case of pneumonectomy performed through a minimally invasive approach in a 9-year-old female with Down's syndrome and gastroesophageal reflux disease. CASE REPORT: The patient suffered from multiple recurrent aspiration pneumonias, which progressed to bronchiectasis of the entire left lung. As a result, the patient was hypoxemic and required continuous supplemental oxygen. Preoperative perfusion scans showed diminished perfusion of the left lung. Thoracoscopy was performed by using 3-5 mm trocars and one 12-mm trocar. Insufflation pressure was maintained at 5 mm Hg. Dissection was performed at the hilum by using hook electrocautery and the LigaSure device (ValleyLab, Boulder, CO). The pulmonary artery, veins, and left mainstem bronchus were sequentially divided by using a 35-mm ENDO GIA vascular stapler (Ethicon Endo-Surgery, Cincinnati, OH). There were no intraoperative complications. Eight months following surgery, her health is improved and she no longer requires supplemental oxygen. CONCLUSION: Thoracoscopic pneumonectomy is a safe, technically feasible approach for severe bronchiectasis in children.

Tubularized Gastric Conduit is More Desirable in Pediatric Patients Treated with Minimally Invasive Esophagectomy and Gastric Pull-Up.

INTRODUCTION: Conditions requiring an esophagectomy and esophageal replacement are rare in children. The preferred method and ideal replacement organ continue to be debated. We present long-term outcomes in children treated with esophagectomy and gastric pull-up. METHODS: We conducted a retrospective review of all the patients who underwent a esophagectomy and gastric pull-up at two major pediatric institutions from 2004 to 2015. Follow-up data were obtained for children when available, including any postoperative complications, need for dilation of strictures, and current feeding method. RESULTS: Minimally invasive procedures were performed on 7 patients (5 female and 2 male) with a median age of 3 years (range 2-20, standard deviation = 8). Three patients successfully underwent laparoscopic transhiatal esophagectomy and cervical gastric pull-up, and three patients successfully underwent combined laparoscopic and right thoracoscopic (Ivor-Lewis) esophagectomy and cervical gastric pull-up. We identified an additional 3 patients who had an open esophagectomy and gastric pull-up. Seven patients had tubularized gastric conduits, six without pyloroplasty and one with pyloroplasty. For those patients with tubularized conduits, the average time to achieve full oral feeds was 16 days, with 1 patient with pyloroplasty who took 27 days. Of the three whole-stomach conduits, one reached oral independence at 19 days and the other two had yet tolerated anything per os. Follow-up data were available for all patients. At the average 5 years follow-up (ranging from 1 month to 7 years), all but two were thriving well with full oral feeds. CONCLUSIONS: Minimally invasive esophagectomy and gastric pull-up is a good alternative in managing pediatric patients in need of esophagectomy and replacement; it offers acceptable early and long-term outcomes. Tubularized conduit appears to be superior to using the whole stomach and potentially avoids pyloroplasty. Ongoing study is needed to validate our findings.

Management of pediatric intramuscular venous malformations.

BACKGROUND: Intramuscular venous malformations (VMs) are rare, but can be highly symptomatic. There are few reports on outcomes, particularly pain, functional limitations, and muscle contractures. We aimed to compare results of medical management, sclerotherapy, and surgical resection. METHODS: We retrospectively reviewed 45 patients with an extremity or truncal intramuscular VM between June 2005 and June 2015 at a single institution. Outcomes were compared between treatment modalities with ANOVA and χ2 tests. RESULTS: Six patients (13%) were treated with medical management, 4 (9%) with surgical resection, 23 (51%) with sclerotherapy, and 12 (27%) with both surgery and sclerotherapy. Sclerotherapy alone decreased pain in 72%. Only 20% of patients presented with muscle contracture. For these patients, 33% resolved with sclerotherapy, physical therapy, and aspirin; 22% resolved with surgery, and 45% had persistent contracture. 40% of patients treated with sclerotherapy then surgery developed new muscle contractures, compared to 4% of sclerotherapy only patients and 0% of surgery only patients (p=0.04). CONCLUSIONS: Medical management, surgery and sclerotherapy are effective treatments for intramuscular VMs. Observation and supportive care can be a primary treatment for patients with minimal symptomatology and no functional limitations. Sclerotherapy is more effective for treating pain than contractures and when used alone, rarely causes a new muscle contracture.

Routine contrast enema is not required for all infants prior to ostomy reversal: A 10-year single-center experience.

INTRODUCTION: The incidence of intestinal stricture is low for most conditions requiring a primary small bowel stoma in infants. Routine performance of contrast enemas (CE) prior to stoma closure adds cost and radiation exposure. We hypothesized that routine CE prior to ostomy reversal is not necessary in all infants, and sought to identify a subset of patients who may benefit from preoperative CE. METHODS: Medical records of infants under age 1 (N=161) undergoing small bowel stoma reversal at a single institution between 2003 and 2013 were retrospectively reviewed. Student's T-test was used to compare groups. RESULTS: Contrast enemas were performed on 80% of all infants undergoing small bowel ostomy reversal during the study period. Infants with necrotizing enterocolitis (NEC) were more likely to have a CE than those with intestinal atresia (p=0.03) or those with all other diagnoses combined (p=0.03). Nine strictures were identified on CE. Of those, 8 (89%) were in patients with NEC, and only 4 were clinically significant and required operative resection. The overall relevant stricture rate was 2.5%. No patient that underwent ostomy takedown without CE had a stricture diagnosed intraoperatively or an unrecognized stricture that presented clinically after stoma takedown. CONCLUSIONS: Routine CE is not required prior to small bowel ostomy reversal in infants. We recommend judicious use of enema studies in patients with NEC and high likelihood of stricture.