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.

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.

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.

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.

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.

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.

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.

Pulmonary infantile hemangioma presenting as a mass in a premature male infant: a case report focusing on pathological features.

Infantile hemangiomas are the most common benign neoplasm of infancy, with most occurring in the head and neck region. Predisposing factors include prematurity, low birth weight, multiple gestations, advanced maternal age, and chorionic villous sampling. In addition, white women, particularly those with a family history, are also at a higher risk. However, pulmonary infantile hemangiomas are exceedingly rare, with only a few case reports in the literature. Infantile hemangiomas should be considered in the differential diagnosis of a pulmonary mass in the early pediatric population. We present a case of pulmonary infantile hemangioma in a premature male infant successfully managed by surgical excision, with an emphasis on the pathogenesis and histologic features.

CD154-CD40 T-cell costimulation pathway is required in the mechanism of hepatic ischemia/reperfusion injury, and its blockade facilitates and depends on heme oxygenase-1 mediated cytoprotection.

BACKGROUND: Ischemia/reperfusion (I/R) injury remains an important clinical problem that affects both early and later allograft outcome. This study was designed to analyze the role of T cells and CD154-CD40 T- cell costimulation pathway in a mouse liver I/R model. METHODS AND RESULTS: Ninety minutes of warm ischemia followed by 4 h of reperfusion in wild-type (WT) mice resulted in a significant hepatic damage, as assessed by liver function (serum alanine aminotransferase [sALT] levels), local neutrophil accumulation (myeloperoxidase activity), and histology (Suzuki's score). In contrast, T-cell deficiency (in T-cell deficient [nu/nu] mice), disruption of the CD154 signaling (in knockout [KO] mice), or its blockade in WT recipients (after MR1 monoclonal antibody [mAb] treatment), virtually prevented hepatic I/R insult. Unlike CD154-deficient T cells, adoptive transfer of WT spleen cells fully restored hepatic I/R injury in nu/nu mice. Finally, the improved hepatic function in CD154 KO recipients, WT mice treated with CD154 mAb, or nu/nu mice infused with CD154-deficient cells resulted in consistently enhanced expression of heme oxygenase-1 (HO-1), a heat-shock protein with cytoprotective functions. CONCLUSION: This study confirms the importance of T cells, and documents for the first time the role of CD154 costimulation signals in the mechanism of hepatic I/R injury. We also show that CD154 blockade-mediated cytoprotection results and depends on HO-1 overexpression. Our data provide the rationale for human trials to target CD154-CD40 costimulation in hepatic I/R injury, particularly in the transplant patient.

A novel strategy against ischemia and reperfusion injury: cytoprotection with heme oxygenase system.

Much interest has recently been focused on the physiological/pathological role of the heme oxygenase (HO) system, the rate-limiting step in the conversion of heme, in inflammatory events. The HO system may be instrumental in mediating a number of cytoprotective effects, because of its end products, biliverdin, carbon monoxide (CO) and ferrous free iron (Fe2+). As each of the byproducts acts dependently and/or co-operatively with each other, their in vivo effects are complex. In general, the HO system is thought to exert three major functions in ischemia/reperfusion injury: (1) anti-oxidant effects; (2) maintenance of microcirculation; and (3) modulatory effects upon the cell cycle. The anti-oxidant functions depend on heme degradation, oxygen consumption and the production of biliverdin/ferritin via iron accumulation. On the other hand, the production of CO, which has vasodilatory and anti-platelet aggregative properties, can maintain tissue microcirculation. Strikingly, CO may also be instrumental in anti-apoptotic and cell arrest mechanisms. The HO system prevents early injury in the re-perfused organ, and inhibits the function of immune reactive cells, such as neutrophils, macrophages and lymphocytes. The role of the HO system as a novel strategy to mitigate an antigen-independent ischemia/reperfusion injury has been documented in a number of transplantation models.

Less extensive techniques for repair of pectus carinatum: the undertreated chest deformity.

BACKGROUND: Although patients with pectus carinatum (PC) often experience moderate to severe symptoms, there are sparse published data about the indications for correction, the newer techniques of surgical repair, and the results. This study reviews clinical experience with new, less extensive, open operative techniques for repair of PC. STUDY DESIGN: Since 1970, 154 patients (119 men and 35 women) with symptomatic PC (mean severity index 1.76) underwent correction at the UCLA Medical Center using modifications of the Ravitch repair. The last 60 consecutive patients had a less extensive open repair with resection of only small chips of cartilage medially and laterally, and suture reattachment of the remaining costal cartilages to the corrected sternum and ribs. For patients with chondromanubrial protrusion, two or three sternal osteotomies were used, and for those with concomitant lower sternal depression, a lower sternal wedge osteotomy was used. For most patients, a temporary support bar anterior to the sternum and cartilages was used for 6 months. RESULTS: Each of the 154 patients with reduced endurance or dyspnea with mild exercise experienced marked improvement within 6 months. There were no major recurrent deformities; six patients underwent minor surgical revision of localized persistent costal cartilage protrusion or depression. Postoperative complications in the last 60 patients were minor and less frequent, pain was less severe, hospitalization was shorter (mean 2.5 days), and postoperative results were better than when more extensive repairs were used in previous years. With a mean overall followup of 7.9 years, more than 97% of all patients experienced a very good or excellent result. CONCLUSIONS: New, less extensive, open techniques for repair of PC have low morbidity, short hospital stay, and very good physiologic and cosmetic results.

Combined laparoscopic-endoscopic placement of primary gastrojejunal feeding tubes in children: a preliminary report.

BACKGROUND: Placement of a primary gastrojejunal tube (GJT) can be technically challenging and often requires an open procedure to negotiate the tube past the duodenal sweep into the jejunum. The alternative approach is to first place a gastrostomy tube (GT), which is then changed to a GJT under endoscopic or fluoroscopic guidance after waiting 6-8 weeks to allow the stoma to mature. We report a case series of primary GJT placement using a combined laparoscopic-endoscopic approach. SUBJECTS AND METHODS: We retrospectively reviewed patients who underwent a combined laparoscopic-endoscopic primary GJT placement. Patients' demographics and relevant clinical information were analyzed. RESULTS: Six patients (4 male, 2 female) were identified. The median age at the time of operation was 30.2 months (range, 28 days-10 years). Five GJTs were successfully placed laparoscopically/endoscopically, and one procedure was converted to open. The mean operative time was 84 minutes (range, 63-102 minutes). Postoperative abdominal radiography confirmed post-pyloric tube position in all patients. Feedings were initiated on the first postoperative day. One intraoperative complication required conversion to an open procedure. No patients developed postoperative complications. CONCLUSIONS: Laparoscopic-endoscopic primary GJT placement is technically feasible and an excellent alternative in patients who require transpyloric feeding access.

Laparoscopic versus open treatment of congenital duodenal obstruction: multicenter short-term outcomes analysis.

BACKGROUND: Laparoscopic repair of congenital duodenal obstruction has become popularized over the past decade. Comparative data on outcomes, however, are sparse. We hypothesized that laparoscopic repair of congenital duodenal obstruction could be performed with similar outcomes to traditional open repair. PATIENTS AND METHODS: Medical records for all cases of congenital duodenal obstruction from 2005 to 2011 at three academic teaching hospitals were retrospectively reviewed. Patients were excluded from the analysis if they had confounding surgical diseases, did not have duodenoduodenostomy during the first hospital admission, had the repair performed before transfer from a referring hospital, or weighed less than 1.7 kg at the time of surgery. Analysis was performed as intention to treat, with laparoscopic converted to open cases included in the laparoscopic group. RESULTS: Sixty-four cases were included in the analysis (44 open, 20 laparoscopic). Baseline characteristics were similar between the two groups with the exception that the open group, on average, underwent repair later than the laparoscopic group (6 days versus 4 days, respectively). Seven laparoscopic cases were converted to an open procedure (35%), most commonly for difficulty in exposing the decompressed distal duodenum. Laparoscopic repair did take significantly longer than open repair (145 minutes versus 96 minutes, respectively), but clinical outcomes were similar. Complications were rare and were similar between methods of repair. Two patients in the laparoscopic group required subsequent open revision. CONCLUSIONS: Laparoscopic duodenoduodenostomy for congenital duodenal obstruction is a technically challenging procedure with a steep learning curve. Despite a relatively high conversion rate, clinical outcomes remained similar to the traditional open repair in selected patients.