Mark L. Wulkan

Antireflux Surgery Outcomes in Pediatric Gastroesophageal Reflux Disease

OBJECTIVES:Antireflux surgery is performed frequently in children with gastroesophageal reflux disease (GERD). Few comparative studies exist which assess the indications for and short- or long-term outcome of open Nissen fundoplication (ONF) and laparoscopic Nissen fundoplication (LNF) for pediatric GERD. We investigated the frequency of reoperation and factors that might influence its occurrence.METHODS:We performed a retrospective, follow up cohort study of all children ≤5 years, who underwent LNF or ONF at our institution from January 1, 1997 to December 31, 2002, where five pediatric surgeons perform fundoplication. Mean follow up time was 36.2 months. The following information was obtained: surgical indications, hospital course data, and long-term surgical outcomes. Data were analyzed using univariate and multiple logistic regressions.RESULTS:Overall, 456 (150 [32.9%] ONF vs. 306 [67.1%] LNF) cases were analyzed. Reoperation was performed in 55 (12.06%), LNF 43 (14.05%), and ONF 12 (8%). The mean interim to reoperation for LNF was 11 months compared to 17 months for ONF (P = 0.007). The reoperation rate at 12 and 24 months were 10.5%, 13.4% and 4%, 6.7% respectively, when LNF was compared to ONF (P = 0.01). The multivariate analysis showed that initial LNF and prematurity were the main predictors for reoperation.CONCLUSIONS:The majority of reoperations for both LNF and ONF occurred in the first year after initial operation; LNF had a significantly higher reoperation rate than ONF. The probability of reoperation for LNF and ONF increases with the presence of comorbidities, especially prematurity and chronic respiratory conditions.

Gastric Volvulus in Infants and Children

INTRODUCTION. Gastric volvulus is an important cause of nonbilious emesis that must be recognized early to ensure a good outcome. We reviewed 7 cases from our institution, Childrens Healthcare of Atlanta (Egleston campus). In addition, we reviewed all gastric volvulus cases in children published to date in the English literature to draw general conclusions about the presentation and treatment of this unusual disease. METHODS. An electronic literature search was performed to find all published cases of pediatric gastric volvulus. The care of all children from January 2002 to December 2007 who were treated for gastric volvulus was also reviewed. RESULTS. There have been 581 cases of gastric volvulus in children published in English between 1929 and 2007. Of these, 252 were acute and 329 were chronic cases. The most common presentation of acute gastric volvulus is in a child <5 years old with nonbilious emesis, epigastric distention, and abdominal pain. Acute gastric volvulus is often associated with deformities of adjacent organs. Definitive diagnosis is made with upper gastrointestinal studies, and definitive therapy requires repair of associated defects and anterior fixation of the stomach to the abdominal wall. The most common presentation of chronic volvulus is in an infant <1 year old with emesis, epigastric distention, feeding difficulties, and growth failure. Treatment may be medical or surgical depending on the underlying etiology of the volvulus. CONCLUSIONS. Acute gastric volvulus is a potentially life-threatening occurrence with a good outcome when treated in a timely fashion. Chronic volvulus may be more difficult to recognize. The common features of acute and chronic gastric volvulus described in this review should assist pediatric health care providers in promptly diagnosing and treating this disease.

Extracorporeal life support for support of children with malignancy and respiratory or cardiac failure : The extracorporeal life support experience

Background: Extracorporeal life support (ECLS) is a means of respiratory and hemodynamic support for patients failing conventional therapies. Children with cancer who develop complications during therapy may require ECLS. Methods: The extracorporeal life support organization (ELSO) registry was queried for all patients younger than 21 years with an International Classification of Diseases, Ninth Revision diagnosis of neoplasm. ELSO centers were also asked whether patients with neoplasms should be offered ECLS. Results: From 1994 to 2007, 107 children met inclusion criteria: 73 with hematologic malignancies and 34 with solid tumors. The median age was 3.71 years. Patients had a total of 112 ECLS runs (five patients had two runs). Patients required ECLS primarily for pulmonary support (n = 86). Median duration of ECLS was 6.1 days. Overall, 62 of 107 patients (58%) died while on ECLS because of irreversible organ failure (n = 37), diagnosis incompatible with life (n = 11), hemorrhage (n = 6), and family request (n = 8). Forty-five of 107 patients (42%) survived ECLS. During the remaining hospitalization, eight patients died resulting in 37 of 107 surviving to hospital discharge (35%). Risk factors for death included lower Po2 before ECLS, higher oxygen index, higher positive end-expiratory pressure, and development of renal or cardiopulmonary complications (p < 0.05). No differences in outcomes were noted in patients with either hematologic malignancies or solid tumors. One hundred eighteen of 133 eligible ECLS centers responded to the questionnaire for an 89% response rate. Among those who responded, 112 of 133 would consider placing a child (95%) with a neoplasm on ECLS. Conclusion: Children with cancer and respiratory failure can be offered ECLS with a reasonable expectation for survival. The opinions of the ELSO center suggest that decisions to offer ECLS to a child with malignancy should be made on a case by case basis, with prognosis of the malignancy being an important factor.

The effect of insufflation pressure on pulmonary mechanics in infants during laparoscopic surgical procedures.

Background Few studies have reported objective measurements of pulmonary changes under controlled conditions in infants undergoing laparoscopic procedures. We objectively measured the pulmonary effects of laparoscopically‐induced pneumoperitoneum in infants less than 1 year of age undergoing surgical procedures under general anaesthesia.

Extracorporeal Life Support for Posttraumatic Acute Respiratory Distress Syndrome at a Children's Medical Center

BACKGROUND Primary traumatic injury was considered previously a contraindication for institution of extracorporeal life support because of high risk for persistent or new bleeding. Published experience in adults suggests that extracorporeal membrane oxygenation (ECMO) can successfully support trauma victims with pulmonary failure. The authors reviewed their experience with the use of ECMO in pediatric and adult trauma patients with acute respiratory distress syndrome (ARDS) at a childrens medical center. METHODS ECMO Center records from 1991 through 2001 (76 children, 8 adults) were reviewed to identify all patients with a primary or secondary ICD-9 diagnostic code of posttraumatic ARDS in addition to documented trauma. RESULTS Five children and 3 adults with traumatic injury and ARDS received ECMO support. Seven patients were injured in motor vehicle collisions; one patient suffered a gunshot wound to the chest. Patient ages ranged from 21 months to 29 years (pediatric median, 4 years; range, 21 months to 18 years). Four patients had pre-ECMO laparotomies, including 3 who required splenectomy. Four patients had liver lacerations, 3 had pulmonary contusions, and 1 had a renal contusion. Median ventilation before ECMO was 6 days (range, 2 to 10). Seven of 8 patients were placed on venovenous (VV) ECMO. Seven patients had significant bleeding on ECMO. Patients were treated with blood product replacement, epsilon-aminocaproic acid (EACA), and aprotinin infusions. Surgical intervention was not required for bleeding. Six patients received hemofiltration. Median time on ECMO was 653 hours (range, 190 to 921 hours). Six of 8 patients overall survived (75%). Four of 5 pediatric patients survived. CONCLUSIONS Children and adults with severe posttraumatic ARDS can be treated successfully on VV extracorporeal support. Hemorrhage occurs frequently but is manageable.

Gastric fundoplication is effective in promoting weight gain in children with severe congenital heart defects.

AIM The aim of this study was to determine outcomes, including weight gain, morbidity, and mortality, of children with severe congenital heart disease who underwent fundoplication (FP) for gastroesophageal reflux disease. METHODS An institutional review board-approved retrospective review was conducted on all children with congenital heart disease who underwent FP from 1999 to 2005. Preoperative age, weight, cardiac procedures, postoperative weight, and mortality were extracted from medical records. The Wilcoxon signed rank, Wilcoxon rank sum, and log-rank tests were used; P value less than .05 was significant. All procedures were performed with dedicated cardiac anesthesia personnel with recovery in a cardiac intensive care unit. RESULTS Of 112 subjects identified, 37 (33%) had single ventricle (SV) physiology. The most frequent cardiac procedures performed were Norwood (33), pulmonary artery band (11), and systemic pulmonary artery shunt (11). A total of 104 laparoscopic FPs (with 2 conversions to open) and 8 open FPs were performed. The median preoperative age was 3 months, and weight percentile was 1.5%. From baseline, postoperative median weight percentiles increased to 4% at 3 months (P < .001) and to 20% at 5 years postoperatively (P = .004). Single ventricle physiology had no significant effect on outcomes. Postoperative mortality (< or =30 days) was 4.5% (5/112); 5-year survival was 74% (83/112). Five-year survival of SV subjects (59%) was significantly lower (P = .03) than that of the other subjects (81%). No significant difference in survival was seen between SV subjects with FP and all SV patients seen at our center during the study period. Only one death was directly related to antireflux surgery (SV subject). There were 8 patients who had recurrent gastroesophageal reflux disease: 4 were treated with reoperation, and 4 were treated medically. CONCLUSION Weight gain in this high-risk population can be expected after antireflux surgery. Mortality is high because of intrinsic disease, especially in the SV population. Fundoplications performed with the assistance of dedicated pediatric cardiac anesthesia personnel followed by recovery in a cardiac intensive care unit is possible with acceptable postoperative morbidity and mortality.

Operative vs nonoperative management for blunt pancreatic transection in children: multi-institutional outcomes.

BACKGROUND The management of traumatic pancreatic transection remains controversial. STUDY DESIGN A multi-institutional review from 1995 to 2012 was conducted comparing operative with nonoperative management for grades II and III blunt pancreatic injuries in patients younger than 18 years. RESULTS Fourteen pediatric trauma centers participated, yielding 167 patients; 57 underwent distal pancreatectomy and 95 were managed nonoperatively. Fifteen patients treated with operative drain placement only were studied separately. Patients undergoing resection had a shorter time to goal oral feeds (7.8 ± 0.7 days vs 15.1 ± 2.5 days; p = 0.007) and a lower rate of pseudocyst formation (0% vs 18%; p = 0.001). Pseudocyst formation resulted in a greater need for endoscopic and interventional radiologic procedures (26% vs 2%; p = 0.002) in the nonoperative group, as well as a longer time to complete resolution (38.6 ± 6.4 days vs 22.6 ± 5.0 days; p = 0.05) compared with resection. When looking at those patients with clear evidence of main duct injury at presentation, those undergoing resection also had fewer complications (33% vs 61%; p = 0.05) and fewer total days in-hospital (12.6 ± 8.4 days vs 17.5 ± 9.7 days; p = 0.04) compared with nonoperative management. CONCLUSIONS In children with blunt pancreatic injury, distal pancreatectomy is superior to nonoperative management with more rapid resumption of diet, fewer repeat interventions, and a shorter period to complete resolution. When the main duct is involved, the benefits to operative resection also include lower morbidity and fewer days of hospitalization. Therefore, assessing the status of the pancreatic duct is paramount in determining management.

Undifferentiated Embryonal Sarcoma of the Liver is Associated with Mesenchymal Hamartoma and Multiple Chromosomal Abnormalities: A Review of Eleven Cases

Undifferentiated embryonal sarcoma (UES) of the liver is a primitive mesenchymal, malignant neoplasm occurring in children. The link between UES and mesenchymal hamartoma (MH) is controversial. Whether they share the same histiogenesis, representing 2 ends of a spectrum, or are distinct entities is unclear. The genetic aberrations of these neoplasms are not well understood, although a common breakpoint (19q13.4) was recently identified. The purpose of this study was to elucidate immunohistochemical markers that may establish a link between the 2 tumors by reviewing cases of UES and MH. Cases of UES from 1990 to 2008 were identified. Clinical demographics were reviewed. Hematoxylin and eosin staining and immunohistochemical staining for vimentin, alpha-1 antitrypsin, and alpha-fetoprotein were performed. Eleven children were diagnosed with UES. Five cases were seen arising in association with MH, and transitional zones were evident. The mean age at presentation was 10 years. To our knowledge, the 11-month-old patient is the youngest reported case of UES in concurrence with MH. All UES tumor cells were positive for vimentin, diastase-resistant periodic acid–Schiff stain, and alpha-1 antitrypsin. Chromosomal analysis of 3 UES cases, 2 arising with MH, showed complex karyotypes with no involvement of 19q13.4. We suggest a continuum between UES and MH. Although a chromosomal anomaly of 19q13.4 was not identified, a submicroscopic involvement of this locus cannot be excluded. Additionally, our analyses suggest that multiple chromosomal aberrations may be associated with the MH/UES spectrum.

Laparoscopic Adrenalectomy in Children: A Multicenter Experience

INTRODUCTION Laparoscopic adrenalectomy is now being recognized as the standard approach for adrenalectomy for benign lesions in adults. The published experience in children and adolescents has been limited to sporadic small case series. Therefore, we conducted a large multicenter review of children who have undergone laparoscopic adrenalectomy. METHODS After Institutional Review Boards approval, a retrospective review was conducted on all patients who have undergone laparoscopic adrenalectomy at 12 institutions over the past 10 years. Operative times included unilateral adrenalectomy without concomitant procedures. RESULTS About 140 patients were identified (70 males [50%]). Laterality included 76 (54.3%) left-sided lesions, 59 (42.1%) right, and 5 (3.6%) bilateral. Mean operative time was 130.2 ± 63.5 minutes (range 43-406 minutes). The most common pathology was neuroblastoma in 39 cases (27.9%), of which 23 (59.0%) had undergone preoperative chemotherapy. Other common pathology included 30 pheochromocytomas (21.4%), 22 ganglioneuromas (15.7%), and 20 adenomas (14.3%). There were 13 conversions to an open operation (9.9%). Most conversions were because of tumor adherence to surrounding organs, and tumor size was not different in converted cases (P=.97). A blood transfusion was required in 2 cases. The only postoperative complication was renal infarction after resection of a large neuroblastoma that required skeletonization of the renal vessels. At a median follow-up of 18 months, there was only one local recurrence, which was in a patient with a pheochromocytoma. CONCLUSIONS The laparoscopic approach can be applied for adrenalectomy in children for a wide variety of conditions regardless of age with a 90% chance of completing the operation without conversion. The risk for significant blood loss or complications is low, and it should be considered the preferred approach for the majority of adrenal lesions in children.

Association of feeding modality with interstage mortality after single-ventricle palliation.

OBJECTIVE Interstage mortality has been reported in 10% to 25% of hospital survivors after single-ventricle palliation. The purpose of this study was to examine the impact of feeding modality at discharge after single-ventricle palliation on interstage mortality. METHODS We conducted a retrospective review of all neonates undergoing single-ventricle palliation from January 2003 to January 2010. A total of 334 patients (90%) survived to hospital discharge, comprising the study group. Preoperative, operative, and postoperative variables were examined, including feeding method at discharge. Multivariate Poisson regression models were constructed to estimate the relative risk of interstage mortality. RESULTS Of 334 patients, 56 (17%) underwent gastrostomy tube ± Nissen. There was a statistically significant increase in interstage mortality for patients who underwent gastrostomy tube ± Nissen compared with patients who did not (relative risk, 2.38; 95% confidence interval, 1.05-5.40; P = .04]). Of the 278 patients who were not fed via a gastrostomy tube ± Nissen, 190 (68%) were fed with nasogastric feedings and 88 (32%) were fed entirely by mouth. There was no difference in interstage mortality between these 2 groups (relative risk, 0.92; 95% confidence interval, 0.31-2.73; P = .89). CONCLUSIONS Neonates undergoing single-ventricle palliation who require gastrostomy tube ± Nissen are at an increased risk of interstage mortality. The need for gastrostomy tube ± Nissen in this population may be a marker for other unmeasured comorbidities that place them at an increased risk of interstage mortality. Discharge with nasogastric feeds does not increase the risk of interstage mortality.