C. Jason Smithers

Blue Rubber Bleb Nevus Syndrome: Surgical Eradication of Gastrointestinal Bleeding

Objective:We report the largest clinical experience to date of surgically treated patients with blue rubber bleb nevus syndrome (BRBNS). Summary Background Data:BRBNS is a rare congenital disorder presenting with multifocal venous malformations of the skin, soft tissues, and gastrointestinal (GI) tract. Patients with BRBNS develop anemia from chronic GI bleeding, and require lifelong treatment with iron and blood transfusions. An aggressive surgical approach to treat the GI venous malformations of BRBNS has been considered unlikely to be successful because of the large number of lesions, their position throughout the GI tract, and the likelihood of recurrence. Based on our belief that eradicated lesions would not recur, we undertook the removal of all GI tract lesions in an effort to eliminate bleeding. Methods:Ten patients with BRBNS were treated from 1993 to 2002. Lesions were identified using complete GI endoscopy. The multiple venous malformations were removed by a combination of wedge resection, polypectomy, suture-ligation, segmental bowel resection, and band ligation. Results:Patient ages ranged from 2 to 36 years, and patients received an average of 53 prior blood transfusions. A mean of 137 focal GI venous malformations per patient were resected at operation (range 4–557), with a mean operative duration of 14 hours (range 7–23 hours). Only 1 patient who had a less extensive procedure developed recurrent GI bleeding. The mean follow-up period was 5.0 years (range 2.9–10.3 years). Conclusions:We believe that an aggressive excisional approach is indicated for the venous anomalies that cause GI bleeding in BRBNS.

Surgical approaches to aortopexy for severe tracheomalacia

PURPOSE The purpose of this study was to determine the outcomes among three different surgical approaches for performing an aortopexy to treat severe tracheomalacia (STM). METHODS A retrospective review was performed for all patients who underwent an aortopexy by pediatric surgeons at a single institution during 1997-2012. Data collected included details of the operative approaches and clinical results. The data were analyzed using Chi-square and Fisher exact test. RESULTS Forty-one patients underwent an aortopexy. The operation was chosen by the surgeon and not randomized. Exposure was by partial sternotomy (PS) (20), open thoracotomy (12), or thoracoscopic approach (7). Only the PS approach was done by a single team. All groups showed improvement in work of breathing, prevention of severe respiratory distress, and acute life threatening events. These effects were more dramatic for the PS group, especially regarding oxygen and/or ventilator dependence and the ability to undergo tracheostomy decannulation. Among the sixteen patients with failure-to-thrive before successful aortopexy by any technique, ten demonstrated significant improvement in their growth (p=0.025). The recurrence rate for the thoracoscopic approach was 38%, and there were no recurrences in the partial sternotomy and the thoracotomy groups, 38% vs 0% vs 0%, p=0.005. Simultaneous bronchoscopy was utilized more commonly in the PS group compared to the thoracotomy and thoracoscopic group, 95% vs 62% vs 38%. CONCLUSIONS In this series, the partial sternotomy technique had the most reliable resolution of symptoms and no recurrence requiring reoperation. The PS approach to STM has the technical advantages of an improved exposure with equal access to the vessels over the right and left mainstem bronchi, as well as the trachea and a more specific elevation of the arteries, including suspension of the pulmonary arteries and trachea itself when desirable. Simultaneous bronchoscopy during aortopexy and an experienced team also likely contribute to improved outcomes. The variations in populations, follow-up, and use of continuous intraoperative bronchoscopy, however, make firm conclusions difficult.

Externally removable stents in the treatment of benign recalcitrant strictures and esophageal perforations in pediatric patients with esophageal atresia

BACKGROUND We investigated whether removable stents, such as self-expandable plastic stents (SEPSs) and fully covered self-expandable metal stents (FCSEMSs) could provide an alternative treatment for recalcitrant strictures and esophageal perforations after esophageal atresia (EA) repair. OBJECTIVE The primary aim of our study was to evaluate technical feasibility. Secondary aims were to evaluate safety and procedural success. DESIGN Retrospective study. SETTING Tertiary-care referral center. PATIENTS A total of 24 children with EA. INTERVENTIONS Retrospective review of all children with EA who underwent dilation and esophageal stent placement from January 2010 to February 2013 at our institution. MAIN OUTCOME MEASUREMENTS Healing of perforation and stricture resolution at 30 and 90 days. RESULTS A total of 41 stents (SEPSs 14, FCSEMSs 27) were placed in 24 patients with EA during the study period, including 14 who had developed esophageal leaks. Procedural success of esophageal stent placement in the treatment of refractory strictures was 39% at 30 days and 26% at 90 days. The success rate was 80% for closure of esophageal perforations with stent therapy after dilation and 25% for perforations associated with surgical repair. Adverse events of stent placement included migration (21% of SEPSs and 7% of FCSEMSs), granulation tissue (37% of FCSEMSs), and deep ulcerations (22% of FCSEMSs). LIMITATIONS Retrospective study with small sample size. CONCLUSION SEPSs and FCSEMSs can be placed successfully in small infants and children with a history of EA repair. The stents appear to be safe and beneficial in closing esophageal perforations, especially post-dilation. However, a high stricture recurrence rate after stent removal may limit their usefulness in treating recalcitrant esophageal anastomotic strictures.

Categorization and repair of recurrent and acquired tracheoesophageal fistulae occurring after esophageal atresia repair

PURPOSE Recurrent trachea-esophageal fistula (recTEF) is a frequent (5%-10%) complication of congenital TEF (conTEF) and esophageal atresia (EA) repair. In addition, postoperative acquired TEF (acqTEF) can occur in addition to or even in the absence of prior conTEF in the setting of esophageal anastomotic complications. Reliable repair often proves difficult by endoluminal or standard surgical techniques. We present the results of an approach that reliably identifies the TEF and facilitates airway closure as well as repair of associated tracheal and esophageal problems. METHODS Retrospective review of 66 consecutive patients 2009-2016 (55 referrals and 11 local) who underwent repair via reoperative thoracotomy or cervicotomy for recTEF and acqTEF (IRB P00004344). Our surgical approach used complete separation of the airway and esophagus, which reliably revealed the TEF (without need for cannulation) and freed the tissues for primary closure of the trachea and frequently resection of the tracheal diverticulum. For associated esophageal strictures, stricturoplasty or resection was performed. Separation of the suture lines by rotational pexy of the both esophagus and the trachea, and/or tissue interposition were used to further inhibit re-recurrence. For associated severe tracheomalacia, posterior tracheopexy to the anterior spinal ligament was utilized. RESULTS The TEFs were recurrent (77%), acquired from esophageal leaks (26%), in addition to persistent or missed H-type (6%). Seven patients in this series had multiple TEFs of more than one category. Of the acqTEF cases, 6 were esophagobronchial, 10 esophagopulmonic, 2 esophagotracheal (initial pure EA cases), and 2 from a gastric conduit to the trachea. Upon referral, 18 patients had failed endoluminal treatments; and open operations for recTEF had failed in 18 patients. Significant pulmonary symptoms were present in all. During repairs, 58% were found to have a large tracheal diverticulum, and 51% had posterior tracheopexy for significant tracheomalacia. For larger esophageal defects, 32% were treated by stricturoplasty and 37% by segmental resection. Rotational pexy of the trachea and/or esophagus was utilized in 62% of cases to achieve optimal suture line separation. Review with a mean follow-up of 35months identified no recurrences, and resolution of pulmonary symptoms in all. Stricture treatment required postoperative dilations in 30, and esophageal replacement in 6 for long strictures. There was one death. CONCLUSION This retrospective review of 66 patients with postoperative recurrent and acquired TEF following esophageal atresia repair is the largest such series to date and provides a new categorization for postoperative TEF that helps clarify the diagnostic and therapeutic challenges for management.

Hematologic outcomes after total splenectomy and partial splenectomy for congenital hemolytic anemia.

PURPOSE The purpose of this study was to define the hematologic response to total splenectomy (TS) or partial splenectomy (PS) in children with hereditary spherocytosis (HS) or sickle cell disease (SCD). METHODS The Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium registry collected hematologic outcomes of children with CHA undergoing TS or PS to 1 year after surgery. Using random effects mixed modeling, we evaluated the association of operative type with change in hemoglobin, reticulocyte counts, and bilirubin. We also compared laparoscopic to open splenectomy. RESULTS The analysis included 130 children, with 62.3% (n=81) undergoing TS. For children with HS, all hematologic measures improved after TS, including a 4.1g/dl increase in hemoglobin. Hematologic parameters also improved after PS, although the response was less robust (hemoglobin increase 2.4 g/dl, p<0.001). For children with SCD, there was no change in hemoglobin. Laparoscopy was not associated with differences in hematologic outcomes compared to open. TS and laparoscopy were associated with shorter length of stay. CONCLUSION Children with HS have an excellent hematologic response after TS or PS, although the hematologic response is more robust following TS. Children with SCD have smaller changes in their hematologic parameters. These data offer guidance to families and clinicians considering TS or PS.

Posterior tracheopexy for severe tracheomalacia

PURPOSE In severe tracheomalacia, aortopexy addresses anterior vascular compression, but does not directly address posterior membranous tracheal intrusion. We review patient outcomes of posterior tracheopexy for tracheomalacia with posterior intrusion to determine if there were resolution of clinical symptoms and bronchoscopic evidence of improvement in airway collapse. METHODS All patients who underwent posterior tracheopexy from October 2012 to March 2016 were retrospectively reviewed. Clinical symptoms, tracheomalacia scores based on standardized dynamic airway evaluation by anatomical region, and persistent airway intrusion were collected. Data were analyzed by Wald and Wilcoxon signed-ranks tests. RESULTS 98 patients (51% male) underwent posterior tracheopexy at a median age of 15months (IQR 6-33months). Median follow-up was 5months (range 0.25-36months). There were statistically significant improvements in clinical symptoms postoperatively, including cough, noisy breathing, prolonged and recurrent respiratory infections, transient respiratory distress requiring positive pressure, oxygen dependence, blue spells, and apparent life-threatening events (p<0.001), as well as ventilator dependence (p=0.04). Tracheomalacia scores on bronchoscopy improved significantly in all regions of the trachea and bronchi (p<0.001). 9.2% had persistent airway intrusion requiring reoperation, usually with aortopexy. CONCLUSIONS Posterior tracheopexy is effective in treating severe tracheomalacia with significant improvements in clinical symptoms and degree of airway collapse on bronchoscopy. LEVEL OF EVIDENCE Level III, treatment study.

Advances in Pediatric Gastrostomy Placement

Placement of gastrostomy tubes in infants and children has become increasingly commonplace. A historical emphasis on use of open gastrostomy has been replaced by less invasive methods of placement, including percutaneous endoscopic gastrostomy and laparoscopically assisted gastrostomy procedures. Various complications, ranging from minor to the more severe, have been reported with all methods of placement. Many pediatric patients who undergo gastrostomy tube placement will require long-term enteral therapy. Given the prolonged time pediatric patients may remain enterally dependent, further quality improvement and education initiatives are needed to improve long-term care and outcomes of these patients.

The Impact of Natural Disaster on Pediatric Surgical Delivery: A Review of Haiti Six Months Before and After the 2010 Earthquake

Little is known about pediatric surgical disease in resource-poor countries. This study documents the surgical care of children in central Haiti and demonstrates the influence of the 2010 earthquake on pediatric surgical delivery. Methods. We conducted a retrospective review of operations performed at Partners in Health/Zanmi Lasante hospitals in central Haiti. Results. Of 2,057 operations performed prior to the earthquake, 423 were pediatric (20.6%). Congenital anomalies were the most common operative indication (159/423 operations; 33.5%). Pediatric surgical volume increased significantly after the earthquake, with 670 operations performed (23.0% post-earthquake v. 20.6% pre-earthquake, p=.03). Trauma and burns became the most common surgical diagnoses after the disaster, and operations for non-traumatic conditions decreased significantly (p<.01). Conclusion. Congenital anomalies represent a significant proportion of baseline surgical need in Haiti. A natural disaster can change the nature of pediatric surgical practice by significantly increasing demand for operative trauma care for months afterward.

Does the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure change morbidity outcomes for high-risk congenital diaphragmatic hernia survivors?

PURPOSE In high-risk congenital diaphragmatic hernia (CDH), significant barotrauma or death can occur before extracorporeal membrane oxygenation (ECMO) can be initiated. We previously examined ex utero intrapartum treatment (EXIT)-to-ECMO in our most severe CDH patients, but demonstrated no survival advantage. We now report morbidity outcomes in survivors of this high-risk cohort to determine whether EXIT-to-ECMO conferred any benefit. METHODS All CDH survivors with <15% predicted lung volume (PPLV) from September 1999 to December 2010 were included. We recorded prenatal imaging, defect size, and pulmonary, nutritional, cardiac, and neurodevelopmental outcomes. RESULTS Seventeen survivors (8 EXIT-to-ECMO, 9 non-EXIT) had an average PPLV of 11.7%. Eight of 9 non-EXIT received ECMO within 2days. There were no significant defect size differences between groups, mostly left-sided (13/17) and type D (12/17). Average follow-up was 6.7years (0-13years). There were no statistically significant differences in outcomes, including supplemental oxygen, diuretics, gastrostomy, weight-for-age Z scores, fundoplication, pulmonary hypertension, stroke or intracranial hemorrhage rate, CDH recurrence, and reoperation. No survivor in our cohort was neurologically devastated. All had mild motor and/or speech delay, which improved in most. CONCLUSIONS In this pilot series of severe CDH survivors, EXIT-to-ECMO confers neither significant survival nor long-term morbidity benefit. LEVEL OF EVIDENCE Level III treatment study.

Posterior Tracheopexy for Severe Tracheomalacia Associated with Esophageal Atresia (EA): Primary Treatment at the Time of Initial EA Repair versus Secondary Treatment

Purpose We review outcomes of posterior tracheopexy for tracheomalacia in esophageal atresia (EA) patients, comparing primary treatment at the time of initial EA repair versus secondary treatment. Methods All EA patients who underwent posterior tracheopexy from October 2012 to September 2016 were retrospectively reviewed. Clinical symptoms, tracheomalacia scores, and persistent airway intrusion were collected. Indication for posterior tracheopexy was the presence of clinical symptoms, in combination with severe tracheomalacia as identified on bronchoscopic evaluation, typically defined as coaptation in one or more regions of the trachea. Secondary cases were usually those with chronic respiratory symptoms who underwent bronchoscopic evaluation, whereas primary cases were those found to have severe tracheomalacia on routine preoperative dynamic tracheobronchoscopy at the time of initial EA repair. Results A total of 118 patients underwent posterior tracheopexy: 18 (15%) primary versus 100 (85%) secondary cases. Median (interquartile range) age was 2 months (1–4 months) for primary (22% type C) and 18 months (8–40 months) for secondary (87% type C) cases (p < 0.001). There were statistically significant improvements in most clinical symptoms postoperatively for primary and secondary cases, with no significant differences in any postoperative symptoms between the two groups (p > 0.1). Total tracheomalacia scores improved significantly in primary (p = 0.013) and secondary (p < 0.001) cases. Multivariable Cox regression analysis indicated no differences in persistent airway intrusion requiring reoperation between primary and secondary tracheopexy adjusting for imbalances in age and EA type (p = 0.67). Conclusion Posterior tracheopexy is effective in treating severe tracheomalacia with significant improvements in clinical symptoms and degree of airway collapse on bronchoscopy. With no significant differences in outcomes between primary and secondary treatment, posterior tracheopexy should be selectively considered at the time of initial EA repair.