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.

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.

Thymectomy for myasthenia gravis in children: a comparison of open and thoracoscopic approaches.

PURPOSE: Thymectomy is an accepted component of treatment for myasthenia gravis (MG), but optimal timing and surgical approach have not been determined. Though small series have reported the feasibility of thoracoscopic resection, some studies have suggested that minimally invasive methods are suboptimal compared to open sternotomy owing to incomplete clearance of thymic tissue. Here we report the largest series of thymectomies for pediatric myasthenia gravis in the literature to date. METHODS: A retrospective review of patients undergoing thymectomy for MG between 1990 and 2013 in a tertiary referral hospital was performed. Twelve patients who underwent thoracoscopic thymectomy were compared to 16 patients who underwent open thymectomy via median sternotomy. Postoperative outcomes were determined by electronic chart review in consultation with the treating pediatric neurologist. Disease severities were graded according to a modified Myasthenia Gravis Foundation of America (MGFA) Quantitative MG (QMG) score. RESULTS: Overall, thoracoscopic resections tended to be performed on patients with earlier and less severe disease than open surgeries. Inpatient length of stay was significantly shorter after thoracoscopic surgery (mean 1.8 vs 8.0 days, p=0.045). The preoperative and postoperative MGFA QMG scores were equivalent between the two groups. Both groups experienced a decrease in disease severity (p<0.001) after median follow-up time of 23 months in the thoracoscopic group and 44 months in the open group. CONCLUSIONS: Minimally invasive thymectomy for MG in children has increased in popularity as surgeons and neurologists compare the risks and benefits of surgery against other therapies. This analysis suggests that thoracoscopic thymectomy is not inferior to median sternotomy in terms of disease control in this small series, and that the morbidity of the thoracoscopic approach appears sufficiently low to be considered for early stage disease. Low perioperative morbidity and shortened hospital course make thoracoscopic thymectomy an attractive option in centers with sufficient medical and surgical experience.

In utero recombinant adeno-associated virus gene transfer in mice, rats, and primates.

BACKGROUND: Gene transfer into the amniotic fluid using recombinant adenovirus vectors was shown previously to result in high efficiency transfer of transgenes into the lungs and intestines. Adenovirus mediated in utero gene therapy, however, resulted in expression of the transgene for less than 30 days. Recombinant adenovirus associated viruses (rAAV) have the advantage of maintaining the viral genome in daughter cells thus providing for long-term expression of transgenes. METHODS: Recombinant AAV2 carrying green fluorescent protein (GFP) was introduced into the amniotic sac of fetal rodents and nonhuman primates. Transgene maintenance and expression was monitor. RESULTS: Gene transfer resulted in rapid uptake and long-term gene expression in mice, rats, and non-human primates. Expression and secretion of the reporter gene, GFP, was readily demonstrated within 72 hours post-therapy. In long-term studies in rats and nonhuman primates, maintenance of GFP DNA, protein expression, and reporter gene secretion was documented for over one year. CONCLUSIONS: Because only multipotential stem cells are present at the time of therapy, these data demonstrated that in utero gene transfer with AAV2 into stem cells resulted in long-term systemic expression of active transgene roducts. Thus, in utero gene transfer via the amniotic fluid may be useful in treatment of gene disorders.

Long term physiologic modification using rAAV in utero gene-therapy.

BACKGROUND: Transfer of genes in utero via the amniotic fluid was shown previously with recombinant adeno-associated viruses (rAAV) to be highly efficient. Expression for over one year was demonstrated using reporter genes. In addition, it was shown previously that transgenes delivered by this method release protein into the general circulation. Given these results experiments were designed to test the hypothesis that in utero rAAV gene therapy could result in long term physiologic modification. METHODS: A rAAV recombinant expressing ciliary neurotrophic factor (cntf) and green fluorescent (gfp) in a polycistronic messenger was used to treat rat fetuses in utero. CNTF causes weight loss and decreased water consumption as a measurable physiologic effect. GFP was used as a marker of gene expression. RESULTS: In utero gene transfer with rAAV carrying human cntf and gfp resulted in long-term gene expression in rat. CNTF-specific physiologic effects of a decrease in weight and water intake were obtained. Expression of the GFP was documented in the treated animals at one year of age. CONCLUSION: Given this data, in utero gene therapy with rAAV into multipotential stem cells resulted in long term systemic physiologic modification of the treated animals by the transgene product. In utero rAAV gene therapy potentially could be used for gene replacement therapy in metabolic disorders.

The effects of the deep-sea environment on transmembrane signaling.

Membrane-associated processes may be particularly susceptible to perturbation by the high hydrostatic pressures and low temperatures of the deep ocean. Transmembrane signaling by guanyl nucleotide binding protein (G protein) coupled receptors (GPCRs) is affected at a number of steps: (1) agonist activation of the GPCR; (2) the interaction of the receptor with the heterotrimeric G protein; (3) the G protein GTPase cycle; and (4) the activation and function of the effector element, adenylyl cyclase. The effects of low temperature and high hydrostatic pressures on the A(1) adenosine receptor-inhibitory G protein (G(i))-adenylyl cyclase signaling complex were examined in teleost fishes from three families, Scorpaenidae, Macrouridae and Moridae. In a comparison of teleost fishes, rat and chicken, species with body temperatures from 1 to 40 degrees C, at atmospheric pressure, A(1) adenosine receptor agonist binding is conserved at the body temperature of the species. In the marine teleost fishes examined, increased pressure decreases agonist efficacy. There are differences among species in the effects of increased hydrostatic pressure on G protein interactions with receptors, GTP binding to G protein alpha subunits and the intrinsic GTPase activity of alpha subunits. Adenylyl cyclase activity and modulation are affected by increased pressure in all the species examined, except Antimora rostrata which was unaffected by pressure changes. At pressures approximating those which the species experience in situ adenylyl cyclase activity retains its sensitivity to modulators. To understand the physiological consequences of impaired cell signaling several prototypical human diseases are discussed.

Single-incision laparoscopic cholecystectomy in children: a feasible alternative to the standard laparoscopic approach.

PURPOSE: Our aim was to evaluate the outcomes of the single-incision laparoscopic (SIL) cholecystectomy compared with the standard 4-incision laparoscopic (SL) cholecystectomy. METHODS: A retrospective chart review of consecutive patients undergoing cholecystectomy using the SIL approach from January 2008 to September 2010 was performed. These patients were compared with a cohort who underwent an SL cholecystectomy from January 2007 to June 2009. Demographics, operative times, length of stay, blood loss, and intravenous narcotic use was obtained from the charts. A nonpaired Student's t test was used to determine statistical significance. RESULTS: We identified 40 patients in the SIL group and 68 in the SL group. Main diagnosis was cholelithiasis followed by gallstone pancreatitis and cholecystitis. The mean operative time for SIL cholecystectomies was 79.2 minutes vs 63 minutes in the SL group (P < .006). The average length of stay was 1.9 days in the SIL group vs 2.3 days in the SL group (P < .24). The mean intravenous narcotic use was 1 dose in the SIL group vs 2.9 doses in the SL group (P < .007). There were no intraoperative complications. At 1-month postoperative follow-up, all patients had satisfactory recovery. CONCLUSION: Single-incision laparoscopic cholecystectomy is a safe and feasible alternative to the standard laparoscopic approach in children, even in the setting of acute disease.

Single-incision laparoscopic approach to management of splenic pathology in children: an early experience.

PURPOSE: Certain splenic conditions in children require surgical interventions, the majority of which are approached via standard laparoscopy with multiple incisions. The single-incision laparoscopic (SIL) technique is gaining popularity. The aim of this study is to review our institutional experience using the SIL technique to surgically manage different splenic pathology in the pediatric population. METHODS: A retrospective review was performed of the patients who underwent SIL splenic procedures at Miller Children's Hospital (Long Beach, CA) from January 2009 to December 2010. RESULTS: Seven patients underwent a SIL technique for different splenic diseases. Five patients underwent splenectomy, 1 patient underwent a splenic cystectomy and omental patching, and 1 patient underwent reduction of splenic torsion and splenopexy. There were no conversions to open. Six procedures were successfully performed without the need for an additional trocar. However, 1 patient required an additional grasper through a separate stab incision. There were no intraoperative complications. One patient had a superficial wound infection at 2-week postoperative follow-up, which resolved with local wound care. CONCLUSIONS: Our preliminary experience shows the SIL technique for the management of splenic pathology in children is safe and feasible.