Overview
Definition:
Diaphragm resection and reconstruction is a complex surgical procedure involving the removal of all or part of the diaphragm, followed by its repair or replacement, primarily indicated for the management of malignant tumors, particularly sarcomas, that involve or invade the diaphragm.
Epidemiology:
Sarcomas arising from or invading the diaphragm are rare
They can be primary diaphragmatic sarcomas or secondary to invasion from adjacent structures like the pleura, chest wall, or abdominal organs
Incidence is low, often diagnosed at advanced stages.
Clinical Significance:
Diaphragm involvement by sarcoma significantly impacts resectability and prognosis
Successful en bloc resection with clear margins, often requiring diaphragmatic reconstruction, is crucial for achieving local control and long-term survival
This procedure tests the limits of surgical oncological principles and reconstructive techniques.
Indications
Surgical Indications:
Resection and reconstruction are indicated for localized sarcomas involving the diaphragm where complete tumor removal with negative margins is achievable
This includes primary diaphragmatic sarcomas and sarcomas invading the diaphragm from adjacent sites, provided there is no unresectable distant metastasis.
Contraindications:
Absolute contraindications include unresectable disease (e.g., extensive bilateral lung or liver metastases, encasement of major vascular structures not amenable to reconstruction)
Relative contraindications may include severe comorbidities, extensive diaphragm involvement precluding functional reconstruction, or patient refusal.
Preoperative Assessment:
Comprehensive assessment involves detailed history, physical examination, staging investigations (CT chest/abdomen/pelvis, MRI, PET-CT), pulmonary function tests, cardiac evaluation, and nutritional status assessment
Multidisciplinary team discussion is essential.
Preoperative Preparation
Imaging Review:
Detailed review of all imaging to precisely delineate tumor extent, relationship to vital structures (heart, great vessels, lungs, liver, spleen), and assess resectability.
Pulmonary Optimization:
Pulmonary rehabilitation, breathing exercises, and smoking cessation if applicable to improve lung function and tolerance to pneumonectomy or extended lung resection if needed.
Nutritional Support:
Optimizing nutritional status is vital for wound healing and recovery, especially in patients undergoing major resections and prolonged recovery periods.
Informed Consent:
Thorough discussion with the patient regarding the risks, benefits, alternatives, expected outcomes, and the potential need for complex reconstruction and prolonged recovery.
Surgical Management
Surgical Approach:
The approach can be thoracic (thoracotomy), abdominal (laparotomy), thoracoabdominal, or a combination, depending on the tumor's location and extent
Minimally invasive techniques are rarely feasible for extensive sarcomas.
Resection Techniques:
En bloc resection of the diaphragm along with involved adjacent structures is performed
Techniques include wedge resection for small peripheral lesions, segmental resection, or hemidiaÂphragÂmectomy or complete diaphragm resection for extensive involvement.
Reconstruction Methods:
Reconstruction may involve direct closure of small defects, autologous tissue grafts (e.g., rectus abdominis muscle flap, latissimus dorsi flap), or prosthetic materials (e.g., synthetic mesh like Gore-Tex, Marlex, or biologic meshes)
The choice depends on the defect size, location, and patient's condition.
Intraoperative Considerations:
Careful dissection to avoid tumor spillage
Control of hemorrage
Meticulous attention to vascular and airway integrity
Judicious use of positive pressure ventilation to manage paradoxical movement if the diaphragm is significantly compromised pre-reconstruction.
Postoperative Care
Monitoring:
Close monitoring of vital signs, respiratory status (oxygenation, ventilation), fluid balance, pain control, and wound healing in an intensive care setting.
Respiratory Support:
Mechanical ventilation may be required initially
Weaning from the ventilator is guided by respiratory mechanics and pulmonary function
Chest physiotherapy and incentive spirometry are crucial.
Pain Management:
Effective multimodal analgesia, including epidural anesthesia, patient-controlled analgesia (PCA), and adjuvant analgesics, to ensure comfort and facilitate early mobilization.
Ambulation And Rehabilitation:
Early mobilization is encouraged to prevent complications like deep vein thrombosis and pneumonia
Pulmonary rehabilitation may be initiated early.
Complications
Early Complications:
Respiratory failure, pneumonia, atelectasis, pleural effusion, chylothorax, wound infection, seroma, hemothorax, bleeding, diaphragmatic flap dehiscence, abdominal compartment syndrome.
Late Complications:
Chronic pain, incisional hernia, restrictive lung disease, mesh-related complications (infection, extrusion, migration), tumor recurrence, adhesions, bowel obstruction.
Prevention Strategies:
Meticulous surgical technique, appropriate reconstruction material selection, early mobilization, aggressive pulmonary toilet, judicious fluid management, and prophylactic antibiotics
Careful surgical planning to minimize tension on the reconstruction.
Prognosis
Factors Affecting Prognosis:
Histological subtype of sarcoma, tumor grade, stage at diagnosis, completeness of surgical resection (margin status), presence of metastasis, patient's performance status, and response to adjuvant therapies (chemotherapy/radiotherapy).
Outcomes:
Prognosis varies significantly based on the factors mentioned above
Complete resection with clear margins offers the best chance for long-term survival
Recurrence is common, especially with inadequate resection.
Follow Up:
Regular follow-up with clinical examination and imaging (CT scans) is essential to monitor for local recurrence or distant metastasis
The frequency and duration of follow-up are determined by the specific sarcoma characteristics and treatment response.
Key Points
Exam Focus:
Understand the indications for diaphragm resection, reconstructive options (mesh vs
autologous flaps), and common complications
Be aware of the role of multimodality treatment in sarcomas.
Clinical Pearls:
The success of diaphragm resection and reconstruction hinges on meticulous surgical planning and execution to achieve negative margins while preserving organ function
Consider the patient's pulmonary reserve carefully when planning significant diaphragmatic resection.
Common Mistakes:
Incomplete resection leading to local recurrence
Inadequate reconstruction causing respiratory compromise or hernia
Overly aggressive resection without considering functional outcomes or reconstructive feasibility
Underestimating the extent of tumor invasion in imaging.