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Thoracoscopic approach to congenital diaphragmatic hernias in adults: Southampton approach and review of the literature.

ABSTRACT: Congenital diaphragmatic hernias (CDH) are likely to present in early postnatal or infancy and are associated with significant morbidity and mortality due to associated pulmonary hypoplasia, pulmonary hypertension and heart failure. Symptomatic adult congenital Bochdalek hernia, on the other hand, is extremely rare with a prevalence of 0.17-6% of all diaphragmatic hernias. They present with recurrent abdominal pain and shortness of breath. Acute presentations could be life threatening especially if there is incarcerated or threatened bowel in the chest. Repair of symptomatic Bochdalek hernia is recommended in the adult population. We present two cases of right and left symptomatic Bochdalek hernias in adults, encountered over 15 years of practice in a tertiary referral centre in the UK. We discuss their presentation and surgical management and review the literature of similar cases treated by video-assisted thoracoscopic surgery (VATS). Thoracic surgeons are increasingly becoming involved in these cases, which used to be the domain of upper gastrointestinal surgeons. A synthetic patch may be required to close the defect, therefore; the thoracic surgeon must be familiar with such techniques. The recent expansion in video format publishing in the internet and social media has revolutionized the way knowledge and how-to-do-it expertise is distributed around the world. It has the advantage of reaching far more viewer than subscription paper-printed format journals and has a rising significance in encouraging thoracic surgeons to do things they were not used to do. Finally; Symptomatic Bochdalek hernia, and possibly Morgagni hernia in the adult population could be safely repaired by VATS with good and lasting results.

SUBMITTER: Amer K

PROVIDER: S-EPMC5730526 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature

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Thoracoscopic approach to congenital diaphragmatic hernias in adults: Southampton approach and review of the literature.

Amer Khalid K

Journal of visualized surgery 20171130

Congenital diaphragmatic hernias (CDH) are likely to present in early postnatal or infancy and are associated with significant morbidity and mortality due to associated pulmonary hypoplasia, pulmonary hypertension and heart failure. Symptomatic adult congenital Bochdalek hernia, on the other hand, is extremely rare with a prevalence of 0.17-6% of all diaphragmatic hernias. They present with recurrent abdominal pain and shortness of breath. Acute presentations could be life threatening especially ...[more]

PMID: 29302452

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Project description:IntroductionMassive thymic hyperplasia (MTH) is a very rare entity, with fewer than 20 cases reported in the literature in infancy. Most patients have respiratory symptoms and the enlarged thymus gland occupies one side of the thoracic cavity. Posterolateral thoracotomy or median sternotomy is the main treatment for MTH in infants. We report a case of an infant with MTH in which the enlarged thymus occupied his bilateral thoracic cavity and he underwent video-assisted thoracoscopic surgery (VATS). In addition, we reviewed and summarized the relevant literature.Case reportA 4-month-old boy was admitted to the hospital with no apparent cause of dyspnea for 18 days, with cough and sputum. On examination, the patient was found to have cyanotic lips, diminished breath sounds in both lungs, and a positive three concave sign. There was no fever or ptosis. Preoperative imaging showed large soft tissue shadows in the bilateral thoracic cavity, with basic symmetry between the right and left sides. Tumor markers were within the normal range. Ultrasound-guided fine needle biopsy showed normal thymic structures with no evidence of malignancy. As his symptoms worsened, he eventually underwent unilateral thoracic approach video-assisted thoracoscopic exploratory surgery, during which a large mass occupying the bilateral thoracic cavity was removed in a separate block and part of the thymus in the left lobe was preserved. Pathological examination confirmed true thymic hyperplasia (TTH). No relevant complications occurred at the 2-month postoperative follow-up.ConclusionIn infants, MTH occupying the bilateral thoracic cavity can produce severe respiratory and circulatory symptoms due to occupying effects. Although a definitive preoperative diagnosis is sometimes difficult, after combining computed tomography (CT) and fine needle biopsy to exclude evidence of other malignancies, the enlarged thymus occupying the bilateral thoracic cavity can be resected via VATS. Whether the enlarged thymus occupies the bilateral thoracic cavity and the size of the thymus are not absolute contraindications to thoracoscopic surgery. The method is safe, feasible, and minimally invasive to the patient.

Project description:Background:Congenital diaphragmatic hernia (CDH) is commonly associated with pulmonary hypoplasia and pulmonary hypertension (PH). PH associated with CDH (CDH-PH) is frequently resistant to conventional pulmonary vasodilator therapy including inhaled nitric oxide (iNO) possibly due to right and left ventricular dysfunction. Milrinone is an intravenous inotrope and lusitrope with pulmonary vasodilator properties and has been shown anecdotally to improve oxygenation in PH. We developed this pilot study to determine if milrinone infusion would improve oxygenation in neonates ?36 weeks postmenstrual age (PMA) with CDH. Methods/design:Data on pulmonary vasodilator management and outcome of CDH patients was collected from 18 university NICUs affiliated with the Neonatal Research Network (NRN) from 2011 to 2012. The proposed pilot will be a masked, placebo-controlled, multicenter, randomized trial of 66 infants with CDH with an oxygenation index (OI) ?10 or oxygen saturation index (OSI) ?5. The primary outcome is the oxygenation response, as determined by change in OI at 24 h after initiation of study drug. As secondary outcomes, we will determine oxygenation at 48 h and 72 h post-infusion, right ventricular pressures on echocardiogram and the incidence of systemic hypotension, arrhythmias, intracranial hemorrhage, survival without extracorporeal membrane oxygenation, and chronic lung disease (oxygen need at 28 days postnatal age). Finally, we will evaluate the pulmonary and nutritional status at 4, 8 and 12 months of age using a phone questionnaire. Results:Three hundred thirty-seven infants with CDH were admitted to NRN NICUs in 2011 and 2012 of which 275 were ?36 weeks PMA and were exposed to the following pulmonary vasodilators: iNO (39%), sildenafil (17%), milrinone (17%), inhaled epoprostenol (6%), intravenous epoprostenol (3%), and intravenous PGE1 (1%). ECMO was required in 36% of patients. Survival to discharge was 71%. Discussion:CDH is an orphan disease with high mortality with few randomized trials evaluating postnatal management. Intravenous milrinone is a commonly used medication in neonatal/pediatric intensive care units and is currently used in 17% of patients with CDH within the NRN. This pilot study will provide data and enable further studies evaluating pulmonary vasodilator therapy in CDH. Trial registration:ClinicalTrials.gov; NCT02951130; registered 14 October 2016.

Dataset Information

Thoracoscopic approach to congenital diaphragmatic hernias in adults: Southampton approach and review of the literature.

Publications

Thoracoscopic approach to congenital diaphragmatic hernias in adults: Southampton approach and review of the literature.

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