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Early recurrence of congenital diaphragmatic hernia is higher after thoracoscopic than open repair: a single institutional study

Gander, Jeffrey W; Fisher, Jason C; Gross, Erica R; Reichstein, Ari R; Cowles, Robert A; Aspelund, Gudrun; Stolar, Charles J H; Kuenzler, Keith A
INTRODUCTION: Experience in thoracoscopic congenital diaphragmatic hernia (CDH) repair has expanded, yet efficacy equal to that of open repair has not been demonstrated. In spite of reports suggesting higher recurrent hernia rates after thoracoscopic repair, this approach has widely been adopted into practice. We report a large, single institutional experience with thoracoscopic CDH repair with special attention to recurrent hernia rates. METHODS: We reviewed the records of neonates with unilateral CDH repaired between January 2006 and February 2010 at Morgan Stanley Children's Hospital. Completely thoracoscopic repairs were compared to open repairs of the same period. In addition, successful thoracoscopic repairs were compared with thoracoscopic repairs that developed recurrence. Data were analyzed by Mann-Whitney U and Fisher exact tests. RESULTS: Thirty-five neonates underwent attempted thoracoscopic repair, with 26 completed. Concurrently, 19 initially open CDH repairs were performed. Preoperatively, patients in the open repair group required more ventilatory support than the thoracoscopic group. Recurrence was higher after thoracoscopic repair (23% vs 0%; P = .032). In comparing successful thoracoscopic repairs to those with recurrence, none of the factors analyzed were predictive of recurrence. CONCLUSIONS: Early recurrence of hernia is higher in thoracoscopic CDH repairs than in open repairs. Technical factors and a steep learning curve for thoracoscopy may account for the higher recurrence rates, but not patient severity of illness. In an already-tenuous patient population, performing the repair thoracoscopically with a higher risk of recurrence may not be advantageous.
PMCID:4297678
PMID: 21763826
ISSN: 0022-3468
CID: 170817

Thoracoscopic lobectomy in infants less than 10 kg with prenatally diagnosed cystic lung disease

Rothenberg, Steven S; Kuenzler, Keith A; Middlesworth, William; Kay, Saundra; Yoder, Suzanne; Shipman, Kristin; Rodriguez, Ruben; Stolar, Charles J
PURPOSE: Thoracoscopic lobectomy for congenital cystic lung lesions is an accepted technique in pediatric surgery. Since an increasing number of these lesions are detected prenatally, the safety and efficacy of infant resections have been questioned. We reviewed our experience over a 10-year period to evaluate early resection of these lesions. METHODS: From January 2001 to August 2009, 75 patients under 1 year of age and weighing <10 kg underwent thoracoscopic lobectomy at two institutions. Patients carried the following diagnoses: 52 had congenital cystic adenomatoid malformation, 20 had bronchopulmonary sequestration, and 3 had congenital lobar emphysema. All lesions were confirmed after birth by computed tomography scan. Patient age at operation ranged from 4 days to 11 months and patient weight from 3.1 to 10 kg. RESULTS: Seventy-four of 75 lobectomies were thoracoscopically completed. There were 16 upper lobectomies, 1 middle lobectomy, and 55 lower lobectomies. Operative time ranged from 45 to 225 minutes. Hospital length of stay ranged from 1 to 5 days. A subset of 26 patients had surgery younger than 3 months of age and <5 kg, despite being asymptomatic. Their operative time averaged 90 minutes, and mean length of hospital stay was 1.5 days. CONCLUSION: Thoracoscopic lobectomy is safe for infants <10 kg and avoids the morbidity associated with thoracotomy. Operating early on younger patients may avoid the inflammatory changes associated with both clinically apparent and subclinical infections, even in patients weighing <5 kg. This may make the procedures less technically challenging and may result in lower complication and conversion rates.
PMID: 21214429
ISSN: 1092-6429
CID: 914632

Thoracoscopic lobectomy for severe bronchiectasis in children

Rothenberg, Steven S; Kuenzler, Keith A; Middlesworth, William
BACKGROUND/PURPOSE: Lung resection should be considered for severe, localized bronchiectasis causing recurrent infections in patients with cystic fibrosis (CF) and other diseases. This series represents our experience and results with thoracoscopic lobectomy for the treatment of severe bronchiectasis confined to a single lobe. METHODS: Nineteen thoracoscopic anatomic lobectomies were performed between July 1994 and August 2008. Patient age at the time of surgery ranged from 14 months to 22 years. Left lower lobectomy was the most frequently performed procedure (n = 9). Eight patients suffered from CF, 5 had chronic pneumonia, 2 had chronic aspiration, and 3 had other diagnoses. RESULTS: Mean operative time was 162 minutes (range, 65-300), and no cases required a conversion to thoracotomy. The mean duration of postoperative chest tube drainage was 3.2 days (range, 1-9). The mean postoperative length of stay was 3.6 days (range, 1-12). One patient had a prolonged air leak lasting 9 days, which resolved with the placement of a second chest tube. Another had a small hydropneumothorax, which persisted after chest tube removal but resolved spontaneously. CONCLUSIONS: Thoracoscopic lobectomy for severe lobar bronchiectasis with recurrent infection is technically challenging, but appears to be safe and effective. Avoidance of a thoracotomy, in this group of patients, allows for earlier mobilization, less postoperative pain with cough and chest physiotherapy, and faster recovery. There appear to be significant benefits in these patients with chronic respiratory illnesses
PMID: 19575632
ISSN: 1092-6429
CID: 105609

Surgical correction of pectus excavatum

Kuenzler, Keith A; Stolar, Charles J H
Pectus deformities arise from overgrowth of the cartilagenous portion of the ribs. Traditional resective/open procedures have been almost entirely replaced by video assisted retrosternal bar placement with excellent outcomes. This review considers the comprehensive evaluation and care of a pectus patinet as well as technical details regarding conduct of the surgery
PMID: 19203738
ISSN: 1526-0550
CID: 105605

Challenges to cannulation for extracorporeal support in neonates with right-sided congenital diaphragmatic hernia [Case Report]

Fisher, Jason C; Jefferson, Rashida A; Kuenzler, Keith A; Stolar, Charles J H; Arkovitz, Marc S
Right-sided diaphragmatic defects represent less than 20% of all congenital diaphragmatic hernias (CDH). Recent data suggest that right CDH (R-CDH) may carry a disproportionately high morbidity as well as increased rates of extracorporeal support when compared with left CDH. Treatment of infants with R-CDH may be further complicated by anatomical distortion unique to right-sided defects. We report 2 cases of azygous vein cannulation in neonates with large isolated R-CDH. Both infants had postnatal deteriorations within 48 hours, met our criteria for extracorporeal membrane oxygenation (ECMO), and underwent venoarterial cannulations through the right neck. In each case, the venous cannula passed directly into the azygous vein and failed to provide adequate ECMO support. Echocardiography confirmed both cases of azygous cannulation. In one child, the right atrium was successfully cannulated after 90 minutes of extensive cannula manipulation. This child survived a 5-day ECMO course and is alive at 22-month follow-up. In the second child, despite prolonged efforts at cannula repositioning, cannulation of the right atrium was not achieved. We did not offer central cannulation because of a rapidly deteriorating clinical course, with expiration in several hours. At autopsy, a dilated azygous vein was evident as a result of inferior vena cava compression by a malpositioned liver. The possibility of azygous vein cannulation may be increased in neonates with R-CDH and has not been previously reported. When evaluating infants with R-CDH for ECMO, clinicians must recognize the possibility of azygous cannulation and its potentially lethal consequences, and should anticipate alternative venous cannulation
PMID: 18082723
ISSN: 1531-5037
CID: 105600

Central pancreatectomy with pancreaticogastrostomy in children [Case Report]

Fisher, Jason C; Kuenzler, Keith A; Bodenstein, Lawrence; Chabot, John A
Children requiring surgical intervention for pancreatic disease may be at risk long term for exocrine insufficiency and glucose intolerance. Pediatric surgeons must balance the need to perform adequate surgical resection while preserving as much normal pancreatic parenchyma as possible. Neoplasms of the middle pancreatic segment with low malignant potential and isolated trauma to the pancreatic body or neck represent 2 conditions where extensive pancreatic resection is unnecessary. Central pancreatectomy for such lesions is well described in adults. Reconstruction of the distal pancreatic remnant is traditionally performed via Roux-en-Y pancreaticojejunostomy. Pancreaticogastrostomy is an alternative approach that has been used to reconstruct the distal pancreas in the adults. Pancreaticogastrostomy offers several technical advantages over pancreaticojejunostomy. Because children may be uniquely susceptible to the long-term consequences of excessive pancreatic resection, 2 cases using this technique of central pancreatectomy with pancreaticogastrostomy are described
PMID: 17448780
ISSN: 1531-5037
CID: 105598

Glucagonlike peptide-2 analogue: a possible new approach in the management of inflammatory bowel disease

Arthur, Grier L; Schwartz, Marshall Z; Kuenzler, Keith A; Birbe, Ruth
BACKGROUND/PURPOSE: Glucagonlike peptide-2alpha (GLP-2alpha) has been shown to be a growth factor for the small intestine. This study investigated the benefits of intravenous and intraluminal administration of GLP-2alpha using a rat model of inflammatory bowel disease (IBD). METHODS: Normal Fisher rats and HLA-B27 (IBD) rats were treated for 14 days as follows: Fisher, intravenous saline (n = 6); HLA-B27, intravenous saline (n = 6); HLA-B27, intravenous GLP-2alpha (50 microg/kg/d; n = 5); Fisher, intraluminal saline (n = 5); HLA-B27, intraluminal saline (n = 5); or intraluminal GLP-2alpha (50 microg/kg/d; n = 5). Rats were evaluated for frequency of diarrhea, and the bowel was analyzed for gross and microscopic lesions. Statistical evaluations were determined using analysis of variance (ANOVA). A P value of.05 was significant. RESULTS: Intravenous GLP-2alpha decreased diarrhea and the number of bowel lesions (P <.05). Microscopic inflammation was reduced by 24% but was not statistically significant. Intraluminal GLP-2alpha decreased the number of small intestine lesions (P <.05) and the microscopic inflammation (P <.05) but did not significantly reduce diarrhea or the overall number of bowel lesions. CONCLUSIONS: GLP-2alpha ameliorates the signs of IBD in HLA-B27 rats. Intravenous GLP-2alpha reduces diarrhea more effectively than intraluminal administration, and both routes are equally effective in ameliorating inflammation. GLP-2alpha potentially provides a new modality for the treatment of IBD
PMID: 15017568
ISSN: 1531-5037
CID: 105587

Hepatocyte growth factor treatment ameliorates diarrhea and bowel inflammation in a rat model of inflammatory bowel disease

Arthur, L Grier; Schwartz, Marshall Z; Kuenzler, Keith A; Birbe, Ruth
BACKGROUND/PURPOSE: Transfection of the HLA-B27 gene into normal Fischer rats induces phenotypic changes similar to inflammatory bowel disease (IBD). This study investigated the benefits of 2 doses of hepatocyte growth factor (HGF) on the manifestations of IBD in this rat model. METHODS: Fischer rats and HLA-B27 rats were divided into 4 groups: Fischer rats treated with saline, HLA-B27 rats treated with saline, HGF at 150 microg/kg/d, and HGF at 300 microg/kg/d. HGF or saline was infused for 14 days via an osmotic pump attached to a catheter in the internal jugular vein. After treatment, rats were evaluated for diarrhea and reduction in gross and microscopic bowel inflammation. Statistics were determined using analysis of variance (ANOVA). A P value < or =.05 was considered significant. RESULTS: Administration of HGF at 150 microg/kg/d decreased diarrhea by 40%, gross inflammation by 41%, and microscopic inflammation by 72% (P < or =.05). At 300 microg/kg/d HGF decreased diarrhea by 46%, gross inflammation by 45%, and microscopic inflammation by 54% (P < or =.05). CONCLUSIONS: HGF administration reduces the clinical manifestations of IBD in this rat model. Similar effects were seen at both doses of HGF administration, implying that there is a plateau above which further increases in HGF levels provides no added benefit. HGF administration may be clinically useful in the management of IBD
PMID: 14966727
ISSN: 1531-5037
CID: 105586

Hepatocyte growth factor ameliorates inflammatory bowel disease in a rat model

Arthur, L Grier; Kuenzler, Keith A; Schwartz, Marshall Z
This study was designed to investigate the benefits of administration of hepatocyte growth factor in a rat model of inflammatory bowel disease. Transfection of the HLA-B27 gene into Fisher rats induces a phenotype similar to inflammatory bowel disease. Fisher rats and HLA-B27 rats were divided into six groups: (1) Fisher, intravenous saline; (2) HLA-B27, intravenous saline; (3) HLA-B27, intravenous hepatocyte growth factor; (4) Fisher, luminal saline; (5) HLA-B27, luminal saline; and (6) HLA-B27, luminal hepatocyte growth factor. Rats received a 14-day infusion through an osmotic pump attached to a catheter positioned in either the jugular vein or the terminal ileum. Rats were evaluated for stool character, and gross and microscopic bowel inflammation. Statistics were analyzed using analysis of variance or the Kruskal-Wallis nonparametric test. A value of P<0.05 was significant. Compared to untreated HLA-B27 rats, intravenous administration of hepatocyte growth factor decreased diarrhea by 41% and microscopic inflammation by 54% (P<0.05). Luminal hepatocyte growth factor exposure decreased total bowel lesions by 53% and microscopic inflammation by 40% compared to untreated HLA-B27 rats (P<0.05), but it did not have an effect on diarrhea. Administration of hepatocyte growth factor ameliorates many of the features of bowel disease in this rat model and theoretically could have therapeutic applications in the management of inflammatory bowel disease in humans
PMID: 14675716
ISSN: 1091-255x
CID: 105584

Gastric volvulus after laparoscopic Nissen fundoplication with gastrostomy [Case Report]

Kuenzler, Keith A; Wolfson, Philip J; Murphy, Stephen G
The authors present the case report of a 13-year-old boy in whom organoaxial gastric volvulus developed four months after a laparoscopic Nissen fundoplication and placement of gastrostomy. Intraoperative findings were significant for volvulus about an axis defined by 2 fixation points, one at the fundoplication site and the other in the area of the gastrostomy tube, positioned close to the pylorus. Of the 142 pediatric cases of gastric volvulus reported to date, only 7 describe it as a complication subsequent to gastric surgery, and just one case has been reported after laparoscopic Nissen fundoplication. This case shows that fundoplication and gastrostomy, a recommended treatment for gastric volvulus, does not always preclude its development. Increasing numbers of pediatric surgeons are performing laparoscopic Nissen fundoplication procedures. The authors advise surgeons to consider this potentially fatal complication, both intraoperatively when selecting a location for gastrostomy and postoperatively when evaluating complaints
PMID: 12891502
ISSN: 1531-5037
CID: 105581