Use of Transesophageal Echocardiography for Enhanced Safety During Bar Removal Procedures After Minimally Invasive Repair of Pectus Excavatum.

Background: Minimally invasive repair of pectus excavatum involves placement of retrosternal support (Nuss) bars. Hardware removal has been rarely associated with life-threatening hemorrhage from the heart, aorta, internal mammary arteries, and/or lung. There is no accepted standard intraoperative monitoring technique used during removal. We hypothesized that the use of transesophageal echocardiography (TEE) during Nuss bar removal would enhance safety of the procedure and be cost-effective. Methods: IRB-approved retrospective review of patients who underwent Nuss bar removal with intraoperative TEE monitoring over a 4-year period, from March 2013 to May 2017, was completed. Bar removal procedures were performed supine, under general anesthesia. TEE images were monitored and any distortion of the cardiac silhouette, new pericardial effusion, and/or cardiac arrhythmias would be considered evidence of possible bar adherence, triggering possible conversion to sternotomy or thoracotomy. Results: In total, 87 consecutive patients, mean age of 20 years, were identified. Bars had been in place for a mean of 30 months. Average procedure time was 67 minutes. No patients experienced arrhythmias, cardiac injury, or significant hemorrhage during removal. TEE gave excellent visualization of the cardiac silhouette and pericardium in all cases. No patient required insertion of an arterial line, a postoperative chest X-ray, or overnight hospitalization. Patients were discharged from the recovery room an average of 89 minutes postprocedure. Conclusion: TEE offers a minimally invasive safe way to visualize the pericardium and its contents during Nuss bar removal. Significant cardiac/mediastinal injuries should be immediately visible. The use of TEE is cost-effective and allows safe discharge the day of surgery.

Use of Magnets as a Minimally Invasive Approach for Anastomosis in Esophageal Atresia: Long-Term Outcomes.

Introduction: The majority of esophageal atresia (EA) patients undergo surgical repair soon after birth. However, factors due to patient characteristics, esophageal length, or surgical complications can limit the ability to obtain esophageal continuity. A number of techniques have been described to treat these patients with "long-gap" EA. Magnets are a nonsurgical alternative for esophageal anastomosis. The purpose of this study was to report long-term outcomes for the use of magnets in EA. Materials and Methods: Between July 2001 and December 2017, 13 patients underwent placement of a magnetic catheter-based system under fluoroscopic guidance at six institutions. Daily chest radiographs were obtained until there was union of the magnets. Magnets were then removed and replaced with an oro- or nasogastric tube. Complications and outcomes were recorded. The average length of follow-up was 9.3 years (range 1.42-17.75). Results: A total of 85% of the patients had type A, pure EA, and 15% had type C with previous fistula ligation. The average length of time to achieve anastomosis was 6.3 days (range 3-13). No anastomotic leaks occurred, and all of the patients had an expected esophageal stenosis that required dilation given the 10F coupling surface of the magnets (average 9.8, range 3-22). Six patients (46%) had retrievable esophageal stents, and two underwent surgery; yet all maintained their native esophagus without interposition. A total of 92% were on full oral feeds at the time of follow-up. Conclusion: The use of magnets for treatment of long-gap EA is safe and feasible and accomplished good long-term outcomes. The main complication was esophageal stricture, although all patients maintained their native esophagus. A prospective observational study is currently enrolling patients to evaluate the safety and benefit of a catheter-based magnetic device for EA.

Homozygous XYLT2 variants as a cause of spondyloocular syndrome.

Spondyloocular syndrome (SOS) is a rare autosomal recessive, skeletal disorder. Two recent studies have shown that it is the result of biallelic sequence variants in the XYLT2 gene with pleiotropic effects in multiple organs, including retina, heart muscle, inner ear, cartilage, and bone. The XYLT2 gene encodes xylosyltransferase 2, which catalyzes the transfer of xylose (monosaccharide) to the core protein of proteoglycans (PGs) leading to initiating the process of PG assembly. SOS was originally characterized in 2 families A and B of Iraqi and Turkish origin, respectively. Using DNA from affected members of the same 2 families, we performed whole exome sequencing, which revealed 2 novel homozygous missense variants (c.1159C > T, p.Arg387Trp) and (c.2548G > C, p.Asp850His). Our findings extend the body of evidence that SOS is caused by homozygous variants in the XYLT2 gene. In addition, this report has extended the phenotypic description of SOS by adding follow-up data from 5 affected individuals in one of the two families, presented here.

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Morphine, opioids, and the feline pulmonary vascular bed.

BACKGROUND: Opioid-induced vasodepressor responses have been reported in a variety of species and laboratory models. The aim of this study was to ascertain the relative potencies of different clinically relevant opioids compared with traditional vasodepressor agents in the feline pulmonary vascular bed. A second aim was to study the effects of morphine and to identify the receptors involved in the mediation or the modulation of these effects. METHODS: This was a prospective vehicle-controlled study involving an intact chest preparation of adult mongrel cats. The effects of various opioids, morphine, fentanyl, remifentanil, sufentanil, and meperidine were compared with other vasodepressor agents. Additionally, the effects of L-N(5)-(1-iminoethyl) ornithine hydrochloride (L-NIO) (nitric oxide synthase inhibitor), nimesulide [selective cyclooxygenase (COX)-2 inhibitor], glibenclamide (ATP-sensitive K+ channel blocker), naloxone (non-selective opioid receptor antagonist), and diphenhydramine (histamine H(1)-receptor antagonist) were investigated on pulmonary arterial responses to morphine and other selected agonists in the feline pulmonary vascular bed. The systemic pressure and lobar arterial perfusion pressure were continuously monitored, electronically averaged, and recorded. RESULTS: In the cat pulmonary vascular bed of the isolated left lower lobe, morphine, remifentanil, fentanyl, sufentanil, and meperidine induced a dose-dependent moderate vasodepressor response and it appeared that sufentanil was the most potent on a nanomolar basis. The effects of morphine were not significantly altered after administration of L-NIO, nimesulide, and glibenclamide. However, the vascular responses to morphine were significantly attenuated following administration of naloxone and diphenhydramine. CONCLUSION: The results of the present study suggest that sufentanil appears to have slightly more potency and morphine the least of the five opioid agonists studied on a nanomolar basis. Morphine-induced vasodilatory responses appeared to be mediated or modulated by both opioid receptor and histamine-receptor-sensitive pathways.

Experimental infection of domestic cats (Felis domesticus) with Cytauxzoon manul from Pallas' cats (Otocolobus manul).

In a random, blind study, six domestic cats were assigned to two treatment groups that received either sterile water or dexamethasone by subcutaneous injection prior to intravenous inoculation with Pallas' cat (Otocolobus manul) blood infected with Cytauxzoon manul. A seventh domestic cat served as a control and was inoculated only with sterile water. Cats were monitored for clinical signs consistent with cytauxzoonosis, and periodically screened for hemoparasitemia. All domestic cats (6/6) that received Pallas' cat blood infected with C. manul developed a low but detectible parasitemia by 9 days post-inoculation, yet remained clinically healthy. All domestic cats (7/7) were subsequently challenged with Cytauxzoon felis and developed clinical signs typical of cytauxzoonosis within 5 days post-challenge. Affected animals were euthanized and cytauxzoonosis was confirmed by histopathology. While inoculation of domestic cats with Pallas' cat blood infected with C. manul induced a parasitemia, it did not cause disease or provide protection against challenge with C. felis. Further studies are warranted to determine the potential for interspecies transmission and disease with C. manul.

Closing arguments for gastroschisis: management with silo reduction.

BACKGROUND: There are two approaches to close gastroschisis. Primary closure (PC) is reduction and fascial closure; silo closure (SC) places viscera in a preformed-silo and reduces the contents over time. We have shifted from PC to SC. This study compared the outcomes of these two techniques. METHODS: Records of babies with gastroschisis from 1994-2004 were reviewed. Closure type, ventilator days, days to full-feeds, hospital days, complications, and mortality were recorded. RESULTS: Twenty-eight patients underwent PC; 20 patients had SC. Differences in ventilator days, days to full-feeds, and hospital days were not statistically significant. Nine PC patients developed closure-related complications vs. none in SC (P < 0.05). Eight PC vs. two SC patients had non-closure-related complications (P < 0.05). Four PC vs. zero SC patients developed necrotizing enterocolitis (P < 0.05). Five PC vs. one SC patients had ventral hernia (P < 0.05). No patient died. CONCLUSION: PC resulted in higher incidence of reclosure, non-closure-related complications, and necrotizing enterocolitis. Consequently, we recommend SC as the preferred treatment.

Chronic abdominal pain secondary to a mucous cystadenoma of the appendix in a 10-year-old boy.

Mucocele of the appendix is a thin-walled dilated appendix filled with mucus. It occurs secondary to chronic obstruction of the appendiceal lumen because of a range of pathologies. Cystadenomas in children are exceedingly rare and most frequently of ovarian origin. A mucous cystadenoma of the appendix in a 10-year-old boy with chronic abdominal pain is presented.

Herbal medication use in the pediatric surgical patient.

PURPOSE: The aim of this study was to determine the prevalence of herbal medication use in the pediatric surgical patient population, because herbal medications can cause major perioperative complications. METHODS: A questionnaire on all drug use before surgery was given to the parents of 1,100 consecutive pediatric surgical patients operated on at a metropolitan children's hospital between June 14, 2002 and August 14, 2002. RESULTS: Eighty-three percent of the surveys were returned. Twenty-one percent of the parents were herbal medication users, but only 4% of patients utilized herbal medications. An average of 2.4 different herbal medicines were in use by each child, and the most common herbal medications were echinacea, chamomile, and aloe. Forty-two percent of herbal medication-using patients were taking prescription medicines concurrently. Fifteen herbal medications-using patients underwent major surgery, and the average preoperative herbal cessation interval was 3.5 days. Children of parents who were herbal medication users and children reported to have chronic diseases were more likely to use herbal medicines (P <.05). Ten percent of parents reported that the surgeon inquired about patient herbal medication use. CONCLUSIONS: This is the first report on herbal medication use in the pediatric surgical patient. The prevalence of herbal medication use is significantly higher in children of parents who use herbal medications and children whose parents consider them to be chronically ill. Surgeons need to specifically inquire about the use of herbal medication in their patients to prevent possible harmful interactions and perioperative complications.

Treadmill and cycle ergometry testing in 5- to 6-year-old children.

The primary purpose of this study was to determine the maximal cardiorespiratory responses of 48, 5- to 6-year-old children (24 girls and 24 boys), who were tested on a treadmill (TM) and an electronically braked cycle ergometer (CE). We also examined the percentage of boys and girls who were able to achieve the criteria for reaching maximal oxygen consumption (VO2max) on each testing mode. After an orientation period, each child was tested twice (once on the TM and once on the CE), with an interval of 1 week between tests. VO2max was measured during progressive, all out, continuous TM and CE tests using standardized procedures. A 2 x 2 analysis of variance test (genderxexercise mode) with Bonferroni adjustment revealed the following: (1) there were no gender differences in any of the measured dependent variables, (2) the children produced a significantly higher relative VO2max (ml x kg(-1) x min(-1) and a higher absolute VO2max (l x min(-1)) on the TM than on the CE (P < 0.001), (3) the children produced a higher heart rate on the TM than on the CE (P < 0.001), (4) the CE generated significantly higher values for respiratory exchange ratio (P < 0.001), (5) the criteria necessary to establish a maximal exercise effort were achieved on both the TM and the CE, and (6) all of the children reached two of the criteria associated with a maximal effort, while only 46% of the children reached three criterion measures. There were no significant differences in the attainment of criterion measures between the TM and the CE. The results of this study indicate a lack of gender differences in maximal exercise testing in 5- to 6-year-old children, and that both the CE and the TM are effective modes of maximal cardiorespiratory testing in this age group.

Colorectal cancer screening: video-reviewed flexible sigmoidoscopy by nurse endoscopists--a Canadian community-based perspective.

BACKGROUND: Colorectal cancer (CRC) is the third most common incident cancer and the second most fatal cancer in Canada. Flexible sigmoidoscopy (FS) is one of the modalities under consideration for CRC screening. The present series reports on a screening program of FS performed by nonphysician endoscopists in a Canadian community setting, with video review of procedures by physicians and recommendation of follow-up colonoscopy where polyps are identified. RESULTS: Five hundred twenty-five, average-risk, asymptomatic patients were examined. After exclusion of inappropriate referrals, 488 remained for analysis. The duration and extent of examination were comparable with those of previous studies elsewhere. Compliance with suggested follow-up was 97.3%. Polyps were identified at FS in 15.4% of examinees. In 8.2% of patients, the polyps were neoplastic at subsequent histology. Four malignant lesions were detected, all at an early stage. There were no complications of FS. INTERPRETATION: This report shows that FS can be carried out safely and effectively by nonphysician personnel in a community setting in Canada. The manpower cost for nonphysician operators is considerably less than that for specialist physician endoscopists. This approach deserves consideration in cost effectiveness analyses of CRC screening.

Multifocal pyogranulomatous osteomyelitis resembling chronic recurrent multifocal osteomyelitis in a lemur.

Chronic recurrent multifocal osteomyelitis is a rare inflammatory bone disease of children and adolescents that is characterized by localized swelling and pain in the clavicles and long bones of the limbs. Diagnosis of chronic recurrent multifocal osteomyelitis is made from clinical signs, characteristic radiographic and histopathologic findings, and negative results of microbial cultures. Treatment of chronic recurrent multifocal osteomyelitis in humans includes administration of nonsteroidal anti-inflammatory drugs or immune modulators, which may be effective in lemurs.

CD40/CD40 ligand interactions are critical for elicitation of autoimmune-mediated fibrosis in the lung.

Pulmonary interstitial fibrosis (PIF), associated with persistent inflammation and increased collagen deposition in the interstitium, is often considered an autoimmune disease. Hapten immune PIF (HIPIF), a model for PIF, is elicited in the lung by a single intratracheal (i.t.) challenge in mice sensitized with hapten (2,4,6-trinitrobenzene sulfonic acid, TNBS). In this study, we characterized the role of CD40/CD40 ligand (CD40L) interactions in the elicitation of secondary cell-mediated immune responses that lead to development of fibrosis in the lung using an adoptive transfer model of HIPIF. The expression of CD40 was detected on bronchoalveolar lavage (BAL) cells 1-3 days after i.t. challenge with hapten in the HIPIF lung, but not lungs from the control mice. The CD40(bright) BAL cells morphologically resembled infiltrating monocytes. Furthermore, blocking CD40/CD40L interactions with blocking Ab decreased BAL production of Th1-mediators (IL-12 and TNF-alpha). Moreover, either blocking CD40/CD40L interactions with the Ab or using IL-12 knockout recipient mice prevented the increased collagen deposition (accumulation of hydroxyproline) in the lungs during HIPIF induction. We conclude that second signals (CD40/CD40L interactions) are required for elicitation of secondary immune responses that lead to PIF in vivo. The results support the notion that CD40/CD40L interactions are involved in the pathogenesis of an ongoing autoimmune disease.

A critical role for alveolar macrophages in elicitation of pulmonary immune fibrosis.

Hapten immune pulmonary interstitial fibrosis (HIPIF) is induced by a recall cell-mediated immune response against the hapten 2,4, 6-trinitrobenzene sulphonic acid (TNBS) in the lung. Studies here dissect the role of the cellular components of the bronchoalveolar lavage (BAL) cells (alveolar macrophages [AMs] versus monocytes and immature dendritic cells) in the fibrogenic inflammatory response. BAL cells from HIPIF mice were generally more activated and produced a greater amount of tumour necrosis factor-alpha (TNF-alpha) than controls. Liposome-encapsulated dichloromethylene diphosphonate (Cl(2)MDP) that was inoculated intranasally (i.n.) into mice selectively depleted AMs. Following AM depletion, the number of TNF-alpha-containing cells was reduced, and both the number of immune inflammatory cells recruited into the alveolar space and the subsequent collagen deposition (hydroxyproline) were decreased in the sensitized and intratracheally (i.t.) challenged mice. In conclusion, AMs are required, in part, for the development of pulmonary fibrosis in HIPIF because AM-derived factors such as TNF-alpha are needed for initiation of chemokine and cytokine pathways and accumulation of immune inflammatory cells.

Overlapping corticostriatal projections from the rodent vibrissal representations in primary and secondary somatosensory cortex.

To determine whether the neostriatum receives overlapping projections from two somatosensory cortical areas, the anterograde tracers biotinylated dextran amine (BDA) and fluoro-ruby (FR) were injected into the whisker representations of primary (SI) and secondary (SII) somatosensory cortex. Reconstructions of labeled terminals and their beaded varicosities in the neostriatum and thalamus were analyzed quantitatively to compare the extent of overlapping projections to both subcortical structures. Corticostriatal projections from focal sites in both somatosensory areas exhibited substantial amounts of divergence within the dorsolateral neostriatum. Most of the labeled terminals were concentrated in densely packed arborizations that occupied lamellar-shaped regions along the dorsolateral edge of the neostriatum. Tracer injections in both cortical areas also produced dense anterograde and retrograde labeling in the thalamus, especially in the ventrobasal complex (VB) and in the medial part of the posterior (POm) nucleus. Because these thalamic regions are topographically organized and have reciprocal connections with corresponding representations in both SI and SII, the amount of labeled overlap in the thalamus was used to indicate the degree of somatotopic correspondence at the SI and SII injection sites. We found that the proportion of overlapping projections to the neostriatum was moderately correlated with the amount of overlap observed in the thalamus. This result strongly indicates that specific sites in the dorsolateral neostriatum receive convergent projections from corresponding somatotopic representations in SI and SII, but also suggests that some of the corticostriatal divergence may reflect neostriatal integration of somatosensory information from noncorresponding representations in SI and SII.