Diaphragmatic hernias represent an extremely rare entity in the adult patient population. This is even more so for non-traumatic presentations, representing a likely congenital aetiology. We present a unique case of a symptomatic massive congenital posterior diaphragmatic hernia, containing a combination of intraperitoneal and extraperitoneal structures - stomach, pancreaticoduodenal complex, spleen, small bowel, colon and left kidney - and our associated management algorithm.
Our patient is a previously well 32-year-old male. His pathology was identified during work up for immigration purposes. A screening chest x-ray revealed “thick-walled cystic spaces”. On further questioning it was discovered that he had been suffering with several years of progressively worsening exertional dyspnoea.
A subsequent CT scan revealed the large posterior diaphragmatic hernia containing the aforementioned structures.
All operative options were discussed and considered, including both open and minimally invasive approaches, as well as abdominal versus thoracic approaches. Case reports in the literature were reviewed. Thus far there is no best practice consensus internationally, and each case report appears to have been managed individualised to the patient’s involved. The anticipated challenges in our case were the size of the spleen relative to the defect itself, and the mobility and management of the retroperitoneal structures. Our goal was to reduce the contents without requiring resection if possible, achieve a tension free repair of the defect and ideally perform the entire procedure with minimally invasive techniques. Functionally we aimed to not only prevent future complications of such a hernia, but importantly hoped to achieve improved lung and exercise function if the left lung could be re-recruited in ventilation.
On discussion with the patient, it was agreed upon to trial a laparoscopic transabdominal approach, with a consideration for possible thoracoscopic assistance, and if this was unsuccessful then a thoracoabdominal open approach would be employed.
In preparation for surgery the patient was counselled and consented appropriately and was preoperatively optimised as per the national splenectomy vaccination protocol and administered bowel prep to assist with handling of colon.
The patient was intubated with a dual lumen endotracheal tube. He was then positioned in right semi-lateral decubitus in a bean bag, to allow for a thoracoabdominal approach if required. Laparoscopic entry was achieved with an optical 12mm trocar, and 3 additional 5mm working trocars were inserted under vision.
The intraperitoneal structures were reduced as best as possible, but it was clear that there were diaphragmatic attachments that precluded complete reduction and the spleen was adhered to the parietal pleura. It was also clear that there was no peritoneal sac whatsoever superiorly, which is consistent with the majority of case reports in the literature.
With extremely careful dissection the intraperitoneal structures were reduced including the spleen, however the retroperitoneal structures were relatively fixed despite extensive careful dissection.
At this stage, a “sarcoma-style” approach was considered in mobilising the entire retroperitoneal complex including all retroperitoneal perinephric fat. The key principle was to stay on the muscle of quadratus lumborum and psoas major, to ensure the entire retroperitoneum was mobilised. In doing so, we managed to successfully reduce the entire retroperitoneal complex safely through the diaphragmatic defect.
With the contents reduced completely the left lung was reviewed, and it was clear that the entire left lower lobe was non-functional and shunted. Administration of positive pressure allowed us to determine if this lobe would have potential to recover. A cardiothoracics opinion was also sought intraoperatively. Under vision, the entire left lower lobe was reinflated with the assistance positive pressure from of our anaesthetic colleagues. Interestingly as the lobe slowly inflated under vision, the compliance in the lung improved.
The diaphragmatic defect was closed primarily with interrupted 0 ethibon, however there was a region laterally that was unable to be approximated due to loss of domain. We placed a large composite mesh (Symbotex by Covidien) which covered the region of primary closure but also bridged the region that was unable to be approximated. The mesh was secured with permanent tacs (Capsure by Bard).
A port was placed thoracoscopically to review the repair from the intrathoracic perspective, and subsequently place a large 28Fr intercostal catheter. Final check was clear, closure was performed and the patient was extubated.
The patient has recovered well with no evidence of complications or recurrence.
Massive diaphragmatic hernias represent a challenging clinical entity. There are multiple challenges that can be anticipated depending on the individual’s pathology. Despite extensive planning we were prepared to convert to a hybrid laparoscopic thoracoscopic approach and potentially convert to open. Key to our success was the extensive retroperitoneal dissection in order to reduce the contents completely but also allow for wide mesh placement and fixation.