Thoracoscopic Repair of Congenital Diaphragmatic Hernia in Neonates: Lessons Learned

Abstract Purpose: We sought to characterize our recent experience with thoracoscopic congenital diaphragmatic hernia (CDH) repair and identify patient selection factors. Methods: We reviewed the medical records of full-term neonatal (<1 month of age) patients who underwent thoracoscopic CDH repair between 2004 and 2008 (n=15). We obtained data on prenatal diagnosis, characteristics of the CDH and repair, complications, and outcome. Results: All patients were stabilized preoperatively and underwent repair at an average of 5.7+/-1.3 days. Six patients were prenatally diagnosed, including the 5 inborn. Thirteen defects were left-sided. All were intubated shortly after birth and 2 required extracorporeal membrane oxygenation (ECMO). Twelve of 15 (80%) patients underwent successful thoracoscopic primary repair, including 1 of the patients who required ECMO prior to repair. Conversion to open repair occurred in 3 of 15 (20%) patients because of the need for patch closure or intraoperative instability. Among those converted to open, all had left-sided CDH defects and 3 had stomach herniation (of 5 such patients). Patients spent an average of 6.9+/-1.0 days on the ventilator following repair. The average time until full-enteral feeding was 16.7+/-2.25 days, and average length of hospital stay was 23.8+/-2.73 days. All patients survived to discharge, and average length of follow-up was 15.3+/-3.6 months. Conclusions: Thoracoscopic repair of CDH is a safe, effective strategy in patients who have undergone prior stabilization. Stomach herniation is associated with, but does not categorically predict, conversion to open repair. ECMO use prior to repair should not be an absolute contraindication to thoracoscopic repair.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/78119/1/lap.2009.0129.pd

Anomalous pancreatic ductal system allowing distal bowel gas with duodenal atresia

A twelve-day-old male presented with non-bilious, non-bloody emesis 2 hours after feeding since two-days-old, with flatus and light stools since birth. An upper gastrointestinal series (UGI) at an outside institution was reported as normal. Abdominal radiographs demonstrated distal bowel gas with proximal duodenal dilation. Abdominal ultrasound demonstrated gas in the main pancreatic duct and a dilated, fluid-filled duodenum. An UGI revealed duodenal atresia with enteric bypass by congenital anomaly of the pancreaticobiliary system. At thirteen days old the patient underwent uncomplicated duodenoduodenostomy. The post-operative course was uneventful and the patient subsequently tolerated oral feeds. Bypass of the atretic duodenal segment through an anomalous pancreatic ductal system is a rare anomaly described in the literature in only a handful of cases. This case report highlights the importance of considering duodenal atresia and pancreaticobiliary enteric bypass in the differential diagnosis of neonates presenting with partial duodenal obstruction. On ultrasound, the presence of gas in the biliary tree or pancreatic duct should alert the physician to the possibility of duodenal atresia with congenital pancreaticobiliary duct anomalies that allow for bypass of enteric contents, including air, into more distal bowel, thereby creating a gas pattern aptly described as double bubble with distal gas

Kaposiform hemangioendothelioma causing intestinal obstruction

A previously healthy toddler with bilious vomiting and erythematous gluteal rash over 2 weeks had intermittent pain, constipation and decreased appetite. All labs were negative with the exception of fecal occult blood. Abdominal x-ray and ultrasound revealed dilated air-filled loops of bowel and partial small bowel obstruction. After persistent worsening abdominal pain and vomiting a CT scan with IV contrast (Fig. 1) suggested small bowel obstruction. Emergent surgery was performed and diagnostic laparoscopy revealed about 61 cm of necrotic bowel causing stricture formation and mesenteric shortening in the distal small bowel. 56 cm of inflamed bowel was resected with end-to-end anastomosis. Final pathology report indicated diffuse intestinal angiomatosis with transmural involvement and focal erosion consistent with KHE (Fig. 2). Presentation is varied, consists of cutaneous lesion, retroperitoneal mass, intestinal obstruction, jaundice, intussusception, or multifocal neoplasms. Complete surgical resection with wide margins is the best therapeutic option and has achieved the best outcomes. If not treated in sufficient time, KHE has a relatively high mortality rate of 30%, with most deaths occurring due to its locally invasive effects [5]. There are limited reports of identifying features of KHE on imaging. Of 165 cases of KHE none were presented in the small bowel [5]. We report the unique case of KHE presenting as a hypervascular mass causing obstruction in the distal small bowel. Although extremely rare, KHE should be considered as a reason for severe GI stricture or obstruction in infants and children in obscure cases and included in the differential

Let them play: A prospective study of postoperative activity restrictions in children

Background: We aim to compare time to resume full activity, postoperative outcomes, and describe family preferences of self-directed activity restrictions (SDAR) and physician-directed activity restrictions (PDAR) following routine pediatric surgeries. Methods: We conducted a single institution, clinical trial (NCT04145895) from January 2020 to February 2022. Patients who presented for a routine inguinal hernia repair (ages 2-13) or laparoscopic appendectomy for uncomplicated appendicitis (aged ≤ 18) were eligible for participation. Participants chose to enroll in either the control (PDAR) or experimental (SDAR) groups. Control patients were instructed to restrict full activities for two weeks. For SDAR, patients were instructed to return to full activity when the patient and family felt comfortable doing so. Postoperatively, participants were asked to complete a survey to assess time to resume full activity, surgical complications, and patient and caregiver satisfaction. Results: 20 (24.4%) enrolled in PDAR and 62 patients (75.6%) enrolled in SDAR. The majority of patients underwent laparoscopic appendectomy (78%). Of patients who completed the postoperative survey, the average time to resume full activity was 11.91 days for PDAR and 9.51 days for SDAR (p = 0.328). There was no difference in parent satisfaction or postoperative complications (p >0.999). Conclusions: The time to resume full activity was two days sooner for SDAR without compromising post-operative outcomes. There are psychosocial benefits to children resuming normal activities, and SDAR may lessen a family's societal burden. Therefore, we propose that SDAR appears safe and should be utilized for routine surgery

A low mortality model of chronic pulmonary hypertension in sheep.

<p>BACKGROUND: Pulmonary hypertension and right ventricular failure are major contributors to morbidity and mortality in chronic lung disease. Therefore, large animal models of pulmonary hypertension and right ventricular hypertrophy are needed to study underlying disease mechanisms and test new treatment modalities. The objective of this study was to create a low-mortality model of chronic pulmonary hypertension and right ventricular hypertrophy in sheep.</p> <p>METHODS: The vena cavae of nine sheep weighing 62 ± 2 (SEM) kg were injected with 0.375 g of dextran beads (sephadex) every day for 60 d. Pulmonary hemodynamics were assessed via pulmonary artery catheterization prior to the first injection and again on d 14, 28, 35, 42, 49, and 56. At the end of the experiment, the heart was removed, dissected, and weighed to determine the ratio of right ventricular mass to left ventricle plus septal mass (RV:LV+S).</p> <p>RESULTS: All sheep survived to 60 d. The average pulmonary artery pressure rose from 17 ± 1 mmHg at baseline to 35 ± 3 mmHg on d 56 with no significant change in cardiac output (8.7 ± 0.7 to 9.8 ± 0.7 L/min, P = 0.89). The RV:LV+S was significantly higher (0.42 ± 0.01, P < 0.001) than a historic group of untreated normal animals (0.35 ± 0.01, n = 13).</p> <p>CONCLUSION: This study provides a low-mortality large animal model of moderate chronic pulmonary hypertension and right ventricular hypertrophy.</p

Hemodynamic design requirements for in-series thoracic artificial lung attachment in a model of pulmonary hypertension.

<p>Recent thoracic artificial lung (TAL) prototypes have impedances lower than the natural lung. With these devices, proximal pulmonary artery (PA) to distal PA TAL attachment may be possible in patients without right ventricular dysfunction. This study examined the relationship between pulmonary system impedance and cardiac output (CO) to create TAL design constraints. A circuit with adjustable resistance and compliance (C) was attached in a PA-PA fashion with the pulmonary circulation of seven sheep with chronic pulmonary hypertension. The pulmonary system zeroth harmonic impedance modulus (Z(0)) was increased by 1, 2.5, and 4 mmHg/(L/min) above baseline. At each Z(0), C was set to 0, 0.34, and 2.1 ml/mmHg. The change in pulmonary system zeroth and first harmonic impedance moduli (ΔZ(0) and ΔZ(1)), the percent change in CO (%ΔCO), and the inlet and outlet anastomoses resistances were calculated for each situation. Results indicate that ΔZ(0) (p < 0.001) but not ΔZ(1) (p = 0.5) had a significant effect on %ΔCO and that %ΔCO = -7.45*ΔZ(0) (R(2) = 0.57). Inlet and outlet anastomoses resistances averaged 0.77 ± 0.16 and 0.10 ± 0.19 mmHg/(L/min), respectively, and the relationship between %ΔCO and TAL resistance, R(T), in mmHg/(L/min) was determined to be %ΔCO = -(7.45f)×(R(T) + 0.87), in which f = the fraction of CO through the TAL. Thus, newer TAL designs can limit %ΔCO to less than 10% if f < 0.75.</p

Use of venovenous extracorporeal membrane oxygenation and an atrial septostomy for pulmonary and right ventricular failure.

BACKGROUND: Right ventricular failure is a major contributor to morbidity and mortality on the lung transplant waiting list. This study was designed to evaluate the effectiveness of an atrial septostomy with venovenous extracorporeal membrane oxygenation (VV-ECMO) as a novel potential bridge to transplantation. METHODS: Adult sheep (58±3 kg; n=12) underwent a clamshell thoracotomy and instrumentation to measure all relevant pressures and cardiac output (CO). Sheep with tricuspid insufficiency (TI [n=5]) and without tricuspid insufficiency (ØTI [n=7]) were examined. After creation of a 1-cm atrial septal defect and initiating VV-ECMO, the pulmonary artery (PA) was banded to allow progressive reduction of pulmonary blood flow, and data were collected. RESULTS: The CO in both groups remained unchanged from baseline at all pulmonary blood flow conditions. With TI, the CO was 5.1±1.2 L/min at baseline versus 5.1±1.2 L/min with a fully occluded PA (p=0.99). For ØTI, the CO was 4.5±1.4 L/min at baseline versus 4.5±1.2 L/min with no pulmonary blood flow (p=0.99). Furthermore, CO was not affected by the presence of TI (p=0.76). Mean right ventricular pressures were significantly lower in the TI group (TI=20.2±11 mm Hg versus ØTI=29.9±8.9 mm Hg; p0.5). Lastly, VV-ECMO maintained normal blood gases, with mean O2 saturations of 99% ± 4.1% in both groups. CONCLUSIONS: Right to left atrial shunting of oxygenated blood with VV-ECMO is capable of maintaining normal systemic hemodynamics and normal arterial blood gases during high right ventricular afterload dysfunction.</p

Veno-venous extracorporeal membrane oxygenation with interatrial shunting: a novel approach to lung transplantation for patients in right ventricular failure.

OBJECTIVE: This study evaluated the effectiveness of an atrial septostomy with veno-venous extracorporeal membrane oxygenation in alleviating high afterload right ventricular dysfunction while providing respiratory support. This technique could be applied as a bridge to lung transplantation. METHODS: Sheep (56±3 kg) underwent a clamshell thoracotomy and hemodynamic instrumentation, including right ventricular pressure and cardiac output. Sheep with and without tricuspid insufficiency (n=5 each) were examined. While sheep were on extracorporeal membrane oxygenation, right ventricular failure was established by banding the pulmonary artery until cardiac output was 40% to 60% of baseline. An extracardiac atrial shunt was created with modified vascular grafts to examine the effect of shunt flow on hemodynamics. Hemodynamic data were thus collected at baseline, during right ventricular failure, and for 1 hour at 100% (fully open), 70%, 50%, and 30% of baseline shunt flow. RESULTS: Cardiac output was returned to baseline values (tricuspid insufficiency: 5.2±0.2 L/min, without tricuspid insufficiency: 5.3±1.2 L/min) with 100% shunt flow (tricuspid insufficiency: 4.8±1.1 L/min, without tricuspid insufficiency: 4.8±1.0 L/min; P=.15) but remained significantly lower than baseline at 70% to 30% shunt flow. At 100% shunt flow, tricuspid insufficiency shunt flow was 1.4±0.8 L/min and without tricuspid insufficiency shunt flow was 1.7±0.2 L/min. Right ventricular pressure was significantly elevated over baseline values at all shunt flows (P CONCLUSIONS: An atrial septostomy accompanied by veno-venous extracorporeal membrane oxygenation is capable of eliminating right ventricular failure while maintaining normal arterial blood gases if sufficient shunt flows are achieved. The presence of tricuspid insufficiency improves the efficacy of the shunt.</p

In-parallel artificial lung attachment at high flows in normal and pulmonary hypertension models.

BACKGROUND: End-stage lung disease patients who require a thoracic artificial lung (TAL) must be extubated and rehabilitated prior to lung transplantation. The purpose of this study is to evaluate hemodynamics and TAL function under simulated rest and exercise conditions in normal and pulmonary hypertension sheep models. METHODS: The TAL, the MC3 Biolung (MC3, Inc, Ann Arbor, MI), was attached between the pulmonary artery and left atrium in nine normal sheep and eight sheep with chronic pulmonary hypertension. An adjustable band was placed around the distal pulmonary artery to control the percentage of cardiac output (CO) diverted to the TAL. Pulmonary system hemodynamics and TAL function were assessed at baseline (no flow to the TAL) and with approximately 60%, 75%, and 90% of CO diverted to the TAL. Intravenous dobutamine (0, 2, and 5 mcg . kg(-1). min(-1)) was used to simulate rest and exercise conditions. RESULTS: At 0 and 2 mcg . kg(-1). min(-1), CO did not change significantly with flow diversion to the TAL for both models. At 5 mcg . kg(-1). min(-1), CO decreased with increasing TAL flow up to 28% +/- 5% in normal sheep and 23% +/- 5% in pulmonary hypertension sheep at 90% flow diversion to the artificial lung. In normal sheep, the pulmonary system zeroth harmonic impedance modulus, Z(0), increased with increasing flow diversion. In hypertensive sheep, Z(0) decreased at 60% and 75% flow diversion and returned to baseline levels at 90%. The TAL outlet blood oxygen saturation was 95% or greater under all conditions. CONCLUSIONS: Pulmonary artery to left atrial TAL use will not decrease CO during rest or mild exercise but may not allow more vigorous exercise.</p