Charles J. Smithers

Direct tracheobronchopexy to correct airway collapse due to severe tracheobronchomalacia: Short-term outcomes in a series of 20 patients

PURPOSE Tracheobronchomalacia (TBM) is associated with esophageal atresia, tracheoesophageal fistulas, and congenital heart disease. TBM results in chronic cough, poor mucous clearance, and recurrent pneumonias. Apparent life-threatening events or recurrent pneumonias may require surgery. TBM is commonly treated with an aortopexy, which indirectly elevates tracheas anterior wall. However, malformed tracheal cartilage and posterior tracheal membrane intrusion may limit its effectiveness. This study describes patient outcomes undergoing direct tracheobronchopexy for TBM. METHODS The records of patients that underwent direct tracheobronchopexy at our institution from January 2011 to April 2014 were retrospectively reviewed. Primary outcomes included TBM recurrence and resolution of the primary symptoms. Data were analyzed by McNemars test for matched binary pairs and logistic regression modeling to account for the endoscopic presence of luminal narrowing over multiple time points per patient. RESULTS Twenty patients were identified. Preoperative evaluation guided the type of tracheobronchopexy. 30% had isolated anterior and 50% isolated posterior tracheobronchopexies, while 20% had both. Follow-up was 5 months (range, 0.5-38). No patients had postoperative ALTEs, and pneumonias were significantly decreased (p=0.0005). Fewer patients had tracheobronchial collapse at postoperative endoscopic exam in these anatomical regions: middle trachea (p=0.01), lower trachea (p<0.001), and right bronchus (p=0.04). CONCLUSION The use of direct tracheobronchopexy resulted in ALTE resolution and reduction of recurrent pneumonias in our patients. TBM was also reduced in the middle and lower trachea and right mainstem bronchus. Given the heterogeneity of our population, further studies are needed to ascertain longer-term outcomes and a grading scale for TBM severity.

Foker process for the correction of long gap esophageal atresia: Primary treatment versus secondary treatment after prior esophageal surgery

PURPOSE The Foker process (FP) uses tension-induced growth for primary esophageal reconstruction in patients with long gap esophageal atresia (LGEA). It has been less well described in LGEA patients who have undergone prior esophageal reconstruction attempts. METHODS All cases of LGEA treated at our institution from January 2005 to April 2014 were retrospectively reviewed. Patients who initially had esophageal surgery elsewhere were considered secondary FP cases. Demographics, esophageal evaluations, and complications were collected. Median time to esophageal anastomosis and full oral nutrition was estimated using the Kaplan-Meier method. Multivariate Cox-proportional hazards regression identified potential risk factors. RESULTS Fifty-two patients were identified, including 27 primary versus 25 secondary FP patients. Median time to anastomosis was 14 days for primary and 35 days for secondary cases (p<0.001). Secondary cases (p=0.013) and number of thoracotomies (p<0.001) were identified as significant predictors for achieving anastomosis and the development of a leak. Predictors of progression to full oral feeding were primary FP cases (O.R.=17.0, 95% CI: 2.8-102, p<0.001) and patients with longer follow-up (O.R.=1.06/month, 95% CI: 1.01-1.11, p=0.005). CONCLUSIONS The FP has been successful in repairing infants with primary LGEA, but the secondary LGEA patients proved to be more challenging to achieve a primary esophageal anastomosis. Early referral to a multidisciplinary esophageal center and a flexible approach to establish continuity in secondary patients is recommended. Given their complexity, larger studies are needed to evaluate long-term outcomes and discern optimal strategies for reconstruction.

Clinical outcomes of splenectomy in children: Report of the splenectomy in congenital hemolytic anemia registry

The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. Results were summarized as hematologic outcomes, short‐term adverse events (AEs) (≤30 days after surgery), and long‐term AEs (31–365 days after surgery). For children with hereditary spherocytosis, after surgery there was an increase in hemoglobin (baseline 10.1 ± 1.8 g/dl, 52 week 12.8 ± 1.6 g/dl; mean ± SD), decrease in reticulocyte and bilirubin as well as control of symptoms. Children with sickle cell disease had control of clinical symptoms after surgery, but had no change in hematologic parameters. There was an 11% rate of short‐term AEs and 11% rate of long‐term AEs. As we accumulate more subjects and longer follow‐up, use of a patient registry should enhance our capacity for clinical trials and engage all stakeholders in the decision‐making process. Am. J. Hematol. 90:187–192, 2015.

Clinical outcomes of splenectomy in children

The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. Results were summarized as hematologic outcomes, short‐term adverse events (AEs) (≤30 days after surgery), and long‐term AEs (31–365 days after surgery). For children with hereditary spherocytosis, after surgery there was an increase in hemoglobin (baseline 10.1 ± 1.8 g/dl, 52 week 12.8 ± 1.6 g/dl; mean ± SD), decrease in reticulocyte and bilirubin as well as control of symptoms. Children with sickle cell disease had control of clinical symptoms after surgery, but had no change in hematologic parameters. There was an 11% rate of short‐term AEs and 11% rate of long‐term AEs. As we accumulate more subjects and longer follow‐up, use of a patient registry should enhance our capacity for clinical trials and engage all stakeholders in the decision‐making process. Am. J. Hematol. 90:187–192, 2015.

Global Surgery Fellowship: A model for surgical care and education in resource-poor countries.

BACKGROUND/PURPOSE Surgical diseases have recently been shown to be a major cause of global morbidity and mortality. Effective methods to decrease the burden of surgical disease and provide care in resource-poor settings are unknown. An opportunity to meet this need exists through collaborative efforts to train local surgeons in specialty care, such as pediatric general surgery. METHODS We present a novel model for the provision of surgical care and education in a resource-poor setting via a collaborative Global Surgery Fellowship program. Through Partners in Health in Haiti, this program placed a fully trained pediatric surgeon at an established rural hospital, both to temporarily serve that community and to teach local surgeons pediatric surgical care. RESULTS The Global Surgery Fellow performed the cases presented here during his term, between July 2009 and June 2010. A total of 147 operative procedures were performed on 131 patients over the course of 12 weeks in Haiti. A total of 134 of the 147 total cases performed (91.2%) were educational cases, in which the Fellow operated with and trained one or more of the following: American medical students, American residents, Haitian residents, or Haitian staff surgeons. CONCLUSION The Global Surgery Fellowship model overcomes many of the traditional challenges to providing adequate surgical care in resource-poor countries. Specifically, it meets the challenge of providing a broad educational experience for many levels of local and foreign physicians, while working within an established locally run health care system. We believe that this model is generalizable to many resource-poor hospitals with permanent local staff that are open to collaboration.

Laparoscopic Gastrojejunostomy Tube Placement in Infants with Congenital Cardiac Disease

INTRODUCTION Gastrojejunostomy (GJ) tubes are an option for durable enteral access for critically ill infants with congenital cardiac disease who struggle with obtaining adequate nutrition. MATERIALS AND METHODS Infants weighing less than 10 kg with cardiac disease who received placement of a laparoscopic GJ tube from November 2011 to January 2015 were reviewed. The operative technique used an umbilical port for the camera and a single stab incision for the gastric access site. After insufflation to 5-8 mm Hg, the stomach was suspended to the abdominal wall, after which a dilator was maneuvered into a postpyloric position using laparoscopic visualization and fluoroscopy, and a glidewire was passed into the duodenum. The GJ tube was then fluoroscopically threaded over the glidewire; final position was confirmed by contrast injection. RESULTS There were 32 laparoscopic GJ tube placement operations performed; 7 (21.9%) of these tubes were standard single-unit GJ tubes, and 25 (78.1%) were low-profile gastrostomy tubes modified with a nasojejunal feeding tube threaded through the feeding port. Median patient age was 3.5 months (range, 0.75-11 months), with a median weight of 4.2 kg (range, 2.4-7.4 kg). Congenital defects were varied, including hypoplastic left heart syndrome and pulmonary vein stenosis. Median operative time was 62 minutes for isolated GJ placement (range, 35-114 minutes). There were three postoperative complications, resulting in a 30-day complication rate of 9.4%. Thirty-day mortality was 9.4% with no mortality related to the operation. CONCLUSIONS Laparoscopic GJ tube placement may be performed safely in infants with cardiac disease and allows these patients to receive adequate nutrition despite intolerance of gastric feeding.

Combined en bloc liver–double kidney transplantation in an infant with IVC thrombosis

McGuire MM, Jones BA, Hull MA, Misra MV, Smithers CJ, Feins NR, Jenkins RL, Lillehei CW, Harmon WE, Jonas MM, Kim HB. Combined en bloc liver–double kidney transplantation in an infant with IVC thrombosis.
Pediatr Transplantation 2011: 15: E142–E144.

Restoring esophageal continuity following a failed colonic interposition for long-gap esophageal atresia.

The Foker process is a method of esophageal lengthening through axial tension-induced growth, allowing for subsequent primary reconstruction of the esophagus in esophageal atresia (EA). In this unique case, the Foker process was used to grow the remaining esophageal segment long enough to attain esophageal continuity following failed colonic interpositions for long-gap esophageal atresia (LGEA). Initially developed for the treatment of LGEA in neonates, this case demonstrates that (i) an active esophageal lengthening response may still be present beyond the neonate time-period; and, (ii) the Foker process can be used to restore esophageal continuity following a failed colonic interposition if the lower esophageal segment is still present.

Reduced size liver transplantation from a donor supported by a Berlin Heart.

Patients on cardiac assist devices are often considered to be high‐risk solid organ donors. We report the first case of a reduced size liver transplant performed using the left lateral segment of a pediatric donor whose cardiac function was supported by a Berlin Heart. The recipient was a 22‐day‐old boy with neonatal hemochromatosis who developed fulminant liver failure shortly after birth. The transplant was complicated by mild delayed graft function, which required delayed biliary reconstruction and abdominal wall closure, as well as a bile leak. However, the graft function improved quickly over the first week and the patient was discharged home with normal liver function 8 weeks after transplant. The presence of a cardiac assist device should not be considered an absolute contraindication for abdominal organ donation. Normal organ procurement procedures may require alteration due to the unusual technical obstacles that are encountered when the donor has a cardiac assist device.

Direct Tracheobronchopexy and Posterior Descending Aortopexy for Severe Left Mainstem Bronchomalacia Associated With Congenital Pulmonary Airway Malformation and Left Circumflex Aortic Arch

Complex tracheobronchial obstruction and malacia can be associated with conotruncal and aortic arch anomalies. A circumflex aortic arch composed of a left aortic arch and right descending aorta is an extremely rare anomaly that can severely affect the distal trachea and mainstem bronchi, resulting in severe respiratory symptoms. We report the case of a patient with circumflex aortic arch and severe left mainstem bronchial obstruction and malacia in which the external aortic compression and residual bronchomalacia were addressed with direct bronchial and tracheal intervention.