Introduction

Retrocaval ureter, also known as circumcaval ureter, is an exceptionally rare congenital anomaly in which the ureter passes behind the inferior vena cava, creating a potential obstruction to urine flow. With a reported worldwide incidence of only 0.06-0.17% of the population, most medical professionals will never encounter this condition in their career. The anomaly is even more uncommon in children, as most cases are diagnosed in adults during the third or fourth decade of life.

This case study details the successful diagnosis and surgical management of retrocaval ureter in a 15-year-old patient at Burjeel Farha in Al Ain, United Arab Emirates. The case highlights the importance of appropriate diagnostic imaging in children with persistent, unexplained abdominal symptoms and demonstrates how targeted surgical intervention can dramatically improve quality of life for patients with this rare condition.

Understanding Retrocaval Ureter

Embryological Basis

Retrocaval ureter is fundamentally a venous anomaly rather than a ureteral one. During embryological development, the right subcardinal vein normally regresses, and the supracardinal vein forms the infrarenal portion of the inferior vena cava (IVC). In retrocaval ureter, the subcardinal vein persists and forms the infrarenal IVC, causing the ureter to course posterior to it. This creates an S-shaped or reverse J-shaped proximal ureter that winds around the IVC, potentially causing functional obstruction.

Classification

Retrocaval ureters are classified into two types based on radiographic appearance:

1. Type I (more common): Shows moderate to severe hydronephrosis with the ureter having an S-shaped or “fishhook” deformity
2. Type II (less common): Shows milder hydronephrosis with the ureter having a sickle-shaped curve

Clinical Presentation

The clinical manifestations of retrocaval ureter can vary widely:

• Some patients remain asymptomatic throughout life
• Others develop symptoms in adolescence or adulthood
• Common symptoms include flank pain, recurrent urinary tract infections, hematuria, and renal calculi
• Symptoms are typically related to the degree of ureteral obstruction and subsequent hydronephrosis

Diagnosis is frequently delayed due to the rarity of the condition and the nonspecific nature of symptoms, which may mimic more common disorders like urolithiasis or pyelonephritis.

Case Presentation

Patient History

A 15-year-old male patient presented to Burjeel Farha Hospital in Al Ain for the first time, accompanied by his parents. They reported that their son had been experiencing irregular abdominal pain and vomiting several times per week for several years. Despite consultations with numerous physicians who had suggested allergies and gastric disorders as potential causes, the prescribed medications had provided only temporary relief.

The parents noted a concerning progression in their son’s condition, with worsening pain and daily episodes of vomiting in recent months. This deterioration in his symptoms prompted them to seek further medical evaluation.

Initial Evaluation

The patient was initially assessed by a pediatrician at Burjeel Farha, who ordered an abdominal ultrasound—remarkably, the first ultrasound examination the child had ever undergone despite years of symptoms. The ultrasound revealed significant dilation of the right renal pelvis (hydronephrosis), prompting referral to Dr. Humam Qaraschouli, Consultant Urologist and Head of the Urology Department.

Dr. Qaraschouli performed a repeat ultrasound himself to further evaluate the findings. The examination confirmed hydronephrosis but also showed dilation of the proximal ureter, suggesting that the condition was not a typical ureteropelvic junction obstruction. To rule out urinary calculi as a cause of obstruction, a non-contrast CT scan of the abdomen was ordered. While this study confirmed the absence of stones, it revealed an unusual course of the right ureter.

Definitive Diagnosis

Based on the suspicious findings, a follow-up contrast-enhanced CT scan was performed, which clearly demonstrated the retrocaval course of the right ureter. The imaging showed the classic J-hook or reverse S appearance of the proximal ureter as it traveled behind the inferior vena cava before continuing its normal path to the bladder. The diagnosis of retrocaval ureter was established.

To complete the evaluation and better visualize the distal ureter (which was not clearly visible on the CT scan), a cystoscopy with retrograde ureterography was performed. This confirmed the diagnosis and provided additional anatomical details essential for surgical planning.

Surgical Management

Preoperative Planning

Given the patient’s young age, progressive symptoms, and confirmed anatomical abnormality, surgical correction was deemed necessary. The goals of surgery were to:

1. Relieve the obstruction
2. Restore normal urinary drainage
3. Preserve renal function
4. Alleviate the patient’s symptoms

The surgical approach chosen was an open flank incision rather than laparoscopic or robotic techniques, considering the specific anatomical details of this case.

Surgical Procedure

Under general anesthesia administered by Dr. Maitham Badawy, Specialist Anesthesia, Dr. Qaraschouli performed the following steps:

1. A right flank incision was made to access the retroperitoneal space
2. The right ureter was identified and carefully exposed
3. The ureter was divided at the point where it passed behind the inferior vena cava
4. The ureter was fully mobilized from its retrocaval position
5. The ureter was relocated to its normal anatomical position, anterior to the inferior vena cava
6. Spatulation of both cut ends of the ureter was performed to widen the anastomosis
7. A double-J ureteral stent was placed to maintain patency during healing
8. An end-to-end ureteral anastomosis was performed to reestablish urinary continuity
9. The incision was closed in layers

Postoperative Care

Four weeks after the initial procedure, the patient underwent a second brief procedure to remove the double-J stent. At this time, ureteroscopy with ureterography was performed to assess the healed anastomosis and confirm unobstructed urinary flow. The evaluation showed excellent results with a patent ureter and no evidence of stenosis at the anastomotic site.

Clinical Outcome

The results of this intervention were dramatic and immediate. Following surgery, the patient experienced complete resolution of his long-standing symptoms. The abdominal pain and vomiting that had plagued him for years disappeared entirely. At follow-up, he was described as being in “perfect condition,” with a remarkable improvement in his quality of life.

The parents reported their satisfaction with the care provided at Burjeel Farha and expressed gratitude that they did not need to travel abroad for this specialized procedure. This highlights the importance of advanced pediatric urological expertise being available within the region.

Discussion

Clinical Significance

This case underscores several important clinical lessons:

1. Importance of Imaging: Despite years of symptoms and multiple medical consultations, this was the first time the patient had undergone ultrasound imaging. Earlier imaging might have led to earlier diagnosis and intervention, potentially preventing years of suffering.
2. High Index of Suspicion: The diagnosis of retrocaval ureter requires awareness of this rare entity and appropriate diagnostic workup when hydronephrosis is identified without an obvious cause.
3. Value of Comprehensive Evaluation: The stepwise diagnostic approach—from ultrasound to non-contrast CT to contrast-enhanced CT and finally to retrograde ureterography—was essential for accurate diagnosis and surgical planning.
4. Effective Surgical Intervention: Despite the rarity of the condition, surgical correction with ureteral reanastomosis proved highly effective, with immediate and complete symptom resolution.

Epidemiological Context

The extreme rarity of this condition cannot be overstated. With a worldwide incidence between 0.06-0.17%, retrocaval ureter is one of the least common congenital urological anomalies. Most reported cases in the literature involve adults, making this pediatric case even more unusual.

The condition shows a male predominance with a male-to-female ratio of approximately 3:1, which aligns with this case of a male patient. This gender disparity is likely related to the embryological development of the inferior vena cava.

Comparison with Alternative Approaches

While this case was managed with traditional open surgery, it’s worth noting that minimally invasive approaches have been described in the literature:

1. Laparoscopic Repair: Can be performed with transperitoneal or retroperitoneal approaches, offering benefits of smaller incisions and faster recovery
2. Robotic-Assisted Repair: Provides enhanced visualization and precision for the ureteral anastomosis
3. Endourological Management: In select cases, ureteral stenting alone may provide symptom relief in patients who are poor surgical candidates

The choice of open surgery in this case was appropriate given the patient’s specific anatomy and the surgeon’s expertise. The excellent outcome validates this approach, though future similar cases might benefit from minimally invasive techniques as expertise with these approaches continues to evolve.

Conclusion

This case represents a successful diagnosis and surgical management of an exceptionally rare congenital anomaly in a pediatric patient. After years of misdiagnosis and ineffective treatments, the patient experienced complete symptom resolution following surgical correction of his retrocaval ureter.

The case highlights several important points for medical practitioners:

1. The critical role of appropriate imaging studies in evaluating persistent, unexplained abdominal symptoms in children
2. The need to consider rare anatomical anomalies when common diagnoses fail to explain a patient’s symptoms
3. The dramatic improvement in quality of life that can result from correct diagnosis and appropriate surgical intervention

For the medical community in the UAE, this case demonstrates the high level of specialized pediatric urological expertise available at Burjeel Farha in Al Ain, eliminating the need for families to travel internationally for complex urological care.

Most importantly, for the young patient in this case, a rare diagnosis followed by expert surgical correction has transformed his life, freeing him from years of unexplained pain and allowing him to enjoy a normal, symptom-free adolescence.

Experts

Introduction

Skull base tumors represent some of the most challenging conditions in neurosurgery. Their deep location, proximity to vital neurovascular structures, and complex surrounding anatomy make them particularly difficult to access and remove safely. Among these rare entities, clival chordomas stand out as especially challenging lesions that require multidisciplinary expertise and advanced technological approaches.

This case study explores the successful management of a clival chordoma in a young patient at Burjeel Medical City (BMC), highlighting the integration of cutting-edge minimally invasive techniques, intraoperative imaging, and comprehensive multidisciplinary care that resulted in an optimal outcome for a potentially devastating condition.

Understanding Clival Chordomas

What are Chordomas?

Chordomas are rare, slow-growing malignant tumors that develop from remnants of the notochord, an embryonic structure involved in the development of the spine. These tumors typically arise along the axial skeleton, with approximately 35% occurring at the skull base (clivus), 50% at the sacrum, and 15% in the mobile spine.

With an incidence of approximately 8 cases per 10 million people, clival chordomas are exceedingly rare. They typically present in adults between 40-60 years of age, though they can occur in any age group.

Clinical Presentation

The clinical manifestation of clival chordomas depends on their size, exact location, and the surrounding structures they compress or invade. Common presentations include:

1. Cranial nerve deficits: Due to compression of cranial nerves traversing the skull base
2. Headaches: Often due to increased intracranial pressure or direct pressure on pain-sensitive structures
3. Visual disturbances: Including double vision (diplopia), visual field defects, or decreased visual acuity
4. Brainstem compression symptoms: May include balance problems, motor or sensory deficits
5. Pituitary dysfunction: If the tumor extends into the sellar region

Diagnostic Challenges

Diagnosing clival chordomas presents several challenges:

1. Rarity: Many clinicians may never encounter a case during their entire career
2. Nonspecific symptoms: Initial symptoms like headache or double vision may be attributed to more common conditions
3. Deep location: The tumor’s position at the skull base makes clinical examination difficult
4. Variable radiographic appearance: Can mimic other skull base lesions

Case Presentation

Patient Profile

The patient in this case was a 35-year-old Pakistani gentleman who worked as a driver. His presenting complaint was double vision (diplopia) that had developed over a period of a few months, progressively worsening to the point where it significantly interfered with his daily activities and forced him to stop driving.

The patient initially sought medical attention at another hospital, where he was found to have paralysis of the left sixth cranial nerve (abducens nerve), which controls lateral eye movement. This explained his diplopia, as the abducens nerve palsy resulted in impaired ability to abduct the left eye.

Diagnostic Workup

The patient underwent magnetic resonance imaging (MRI) which revealed a concerning finding: an extra-axial abnormal signal intensity area in the prepontine cistern along the clivus. The lesion measured approximately 33 × 16 × 29 mm in craniocaudal, anteroposterior, and transverse dimensions.

The imaging characteristics included:

• Low signal intensity on T1-weighted images
• Heterogeneous signal on T2-weighted and FLAIR images
• Heterogeneous signal on diffusion-weighted imaging
• No significant enhancement on post-contrast images

The lesion demonstrated an infiltrative nature, invading and compressing the pons with a clear mass effect on the brainstem. Additionally, the vertebral artery was displaced toward the left. The tumor partially extended into the sella, compressing the pituitary gland, and laterally abutted the left temporal lobe and bilateral cavernous sinuses, with possible extension into the left cavernous sinus.

These radiological findings were highly suggestive of a clival chordoma, a rare skull base tumor.

Initial Diagnosis

Based on the clinical presentation and imaging findings, an initial diagnosis of a retroclival large mass with brain stem compression was made, with clival chordoma being the most likely etiology.

Comprehensive Treatment Approach

Multidisciplinary Assessment

Upon referral to Burjeel Medical City, the patient underwent a thorough evaluation in the neurosurgery clinic. The neurosurgical team explained his condition in detail and discussed various treatment options.

Additional investigations were conducted, including:

1. Endocrine workup: To assess any impact on pituitary function
2. Formal visual assessment: To document the extent of visual impairment

The case was then presented at the Oncology Tumor Board, headed by Prof. Humaid Obaid bin Harmal Al Shamsi, with participation from specialists in radiology, medical oncology, and radiation oncology. Following comprehensive discussion, the board recommended surgical intervention as the primary treatment modality, with subsequent adjuvant therapy to be determined based on histopathological findings.

Surgical Planning

The surgical team opted for a minimally invasive endoscopic endonasal trans-clival approach. This modern technique avoids the need for craniotomy and brain retraction by accessing the tumor through the natural corridor of the nasal cavity and sinuses. The surgical plan included:

1. Endoscopic Trans-Nasal Trans-Clival Microsurgical Resection: A minimally invasive approach to access and remove the tumor
2. Neuronavigation Guidance: Advanced computer-assisted technology to provide real-time surgical orientation
3. Electrophysiological Monitoring: Continuous monitoring of neural function during surgery to minimize the risk of neurological injury
4. Intraoperative Frozen Section: To confirm the diagnosis during surgery
5. Skull Base Reconstruction: To repair the surgical defect and prevent postoperative cerebrospinal fluid leak

Surgical Execution

The surgery proceeded as planned, with an endoscopic approach through the nasal cavity. During the procedure, several key steps were executed:

1. A biopsy with intraoperative frozen section analysis confirmed the diagnosis of clival chordoma
2. The surgical team performed a meticulous resection of the tumor through a narrow corridor between critical neurovascular structures
3. While the patient was still under anesthesia, an intraoperative MRI was performed, revealing a small residual portion of the tumor hidden behind the left carotid artery
4. Based on this crucial information, the patient was immediately returned to surgery for further resection to achieve gross total removal of the tumor

This case highlights the significant value of intraoperative MRI technology, which allowed the surgeons to identify residual tumor that might otherwise have been missed, enabling a more complete resection in a single surgical session.

A follow-up MRI performed 24 hours after surgery confirmed gross total resection of the tumor with no visible residual.

Multidisciplinary Post-Surgical Management

Following the final histopathological confirmation of clival chordoma, the case was again presented to the Tumor Board for discussion of adjuvant therapy. Based on the tumor characteristics and extent of resection, adjuvant radiotherapy was recommended to reduce the risk of recurrence.

This comprehensive, multi-stage approach to treatment planning exemplifies the value of multidisciplinary collaboration in managing complex oncological cases.

Challenges and Considerations

The management of this case involved several significant challenges:

1. Rarity of the Condition

With an estimated incidence of only 8 cases per 10 million people, clival chordomas are exceptionally rare. Consequently, few neurosurgeons gain substantial experience in managing these tumors, making each case a significant technical challenge requiring specialized expertise.

2. Critical Anatomical Location

The tumor’s position at the skull base presented formidable anatomical challenges:

• Located behind the clival bone and anterior to the brainstem
• Difficult to access transcranially without significant risk of neurological morbidity
• Situated in close proximity to critical vascular structures, including the carotid arteries anteriorly and the basilar artery posteriorly
• Adjacent to multiple cranial nerves that could be damaged during surgery

3. Technical Surgical Challenges

The endoscopic approach, while minimally invasive, presented its own technical demands:

• Operating through a narrow corridor with limited maneuverability
• Navigating between vital structures with minimal margin for error
• Working with a 4mm endoscope through the nasal passage to access a deep-seated tumor
• Creating a surgical window through the clival bone between the carotid arteries (approximately 1cm wide by 1.5cm high) to remove a 3cm tumor

4. Oncological Considerations

Chordomas present unique oncological challenges:

• Currently, the median survival for patients with clival chordomas is approximately 7 years
• The overall survival rates are reported as 68% at 5 years and 40% at 10 years
• Complete surgical resection offers the best chance for long-term survival, potentially reaching 67% at 10 years
• The tumor’s proximity to critical structures often makes complete resection difficult to achieve safely

Technological Advantages

This case demonstrates the impact of advanced technology in modern neurosurgery:

Endoscopic Skull Base Surgery

Endoscopic approaches represent the cutting edge of skull base surgery, offering:

• Minimally invasive access through natural corridors
• Excellent visualization with magnified, high-definition views
• Reduced collateral damage to surrounding structures
• Faster recovery compared to traditional open approaches

Intraoperative MRI

The availability of intraoperative MRI proved crucial in this case:

• Identified residual tumor that would otherwise have been missed
• Enabled immediate return to surgery for complete resection
• Eliminated the need for a second operation
• Potentially improved long-term oncological outcomes

Comprehensive Cancer Center Capabilities

Burjeel Cancer Institute at BMC offered several advantages:

• A highly trained, subspecialized team of physicians
• State-of-the-art equipment and facilities
• Up-to-date treatment protocols
• Complete continuum of care from diagnosis through surgery and adjuvant therapy

Conclusion

This case illustrates the successful management of a rare and challenging clival chordoma through a state-of-the-art minimally invasive approach. The integration of advanced surgical techniques, intraoperative imaging, and multidisciplinary collaboration exemplifies modern neuro-oncological care at its best.

The comprehensive capabilities at Burjeel Medical City—including specialized surgical expertise, advanced intraoperative imaging, and multidisciplinary tumor board—were instrumental in achieving an optimal outcome in this case. For rare and complex conditions like clival chordomas, such a comprehensive approach offers patients the best opportunity for successful treatment and improved quality of life.

This case serves as an excellent example of how technological advancement and multidisciplinary collaboration can overcome even the most challenging neurosurgical conditions, providing hope and healing for patients facing rare and difficult-to-treat disorders.

Experts

Introduction

Peritoneal carcinomatosis—the spread of cancer cells throughout the peritoneal cavity—represents one of the most challenging scenarios in oncology. Patients with malignant ascites (abnormal buildup of fluid in the abdomen containing cancer cells) often experience significant discomfort, reduced quality of life, and limited treatment options. Traditional intravenous chemotherapy frequently yields suboptimal results due to poor penetration into peritoneal tissues, while conventional intraperitoneal chemotherapy has been associated with significant complications and uneven drug distribution.

In this context, Pressurized Intraperitoneal Aerosolized Chemotherapy (PIPAC) emerges as an innovative approach, designed to overcome these limitations. This case study discusses the successful implementation of PIPAC therapy at Burjeel Holdings—a pioneering achievement as one of the earliest applications of this technique in the United Arab Emirates.

Understanding PIPAC Therapy

Mechanism of Action

PIPAC represents a significant advancement in the delivery of chemotherapy for peritoneal carcinomatosis. The procedure involves several key components that distinguish it from conventional treatments:

1. Aerosolization: Rather than using liquid chemotherapy, PIPAC converts the anticancer drugs into a fine aerosol spray consisting of micro-droplets.
2. Pressurization: The aerosol is delivered under pressure (approximately 12 mmHg), enhancing tissue penetration through physical principles.
3. Minimal Access Delivery: The entire procedure is performed laparoscopically through just two small incisions, minimizing surgical trauma.
4. Homogeneous Distribution: The aerosol format allows for more even distribution throughout the peritoneal cavity compared to liquid.
5. Enhanced Tissue Penetration: The combination of aerosolization and pressure results in deeper penetration of chemotherapy agents into tumor nodules.

Advantages Over Conventional Approaches

PIPAC offers several potential benefits compared to traditional intravenous or intraperitoneal chemotherapy:

• Improved Drug Delivery: Enhanced penetration into peritoneal tissues by a factor of 3-4 times compared to liquid solutions
• Reduced Systemic Exposure: Lower doses with minimal systemic absorption, resulting in fewer side effects
• Repeatable Procedure: Can be performed multiple times at intervals of 6-8 weeks
• Minimally Invasive: Outpatient procedure with rapid recovery
• Visual Assessment: Allows direct visualization and biopsy of peritoneal disease during each session
• Quality of Life: Typically associated with less toxicity and better preservation of function

Current Applications

PIPAC is primarily indicated for patients with peritoneal carcinomatosis arising from:

• Gastric cancer
• Colorectal cancer
• Ovarian cancer
• Peritoneal mesothelioma
• Other malignancies with peritoneal dissemination

It may be employed in various clinical scenarios:

• As palliative treatment for symptom control
• In combination with systemic chemotherapy
• As neoadjuvant therapy before definitive surgery
• For patients who have progressed on conventional treatments

Case Presentation

Patient Profile

A 72-year-old female patient presented to Burjeel Holdings on March 11, 2024, with a history of:

• Carcinoma of the stomach with malignant ascites for several months
• Previous emergency surgery (laparotomy with subtotal gastrectomy) for a perforated gastric ulcer due to malignancy
• Recurrent ascites requiring multiple drainage procedures (three paracentesis procedures within two months)

Disease History

The patient’s oncological journey began with an emergency presentation of a perforated gastric ulcer that was discovered to be malignant. The initial surgical intervention on March 22, 2024, in her home country included:

• Laparotomy
• Subtotal gastrectomy for perforation and peritonitis
• Roux-en-Y gastrojejunostomy performed with palliative intent

Intraoperative findings included:

• 1×1 cm perforation in the anterior wall near the gastric antrum
• Large neoplastic lesion along the lesser curvature
• Suspicious desmoplastic areas on the posterior wall of the stomach
• Enlarged lymph nodes
• Suspicious malignant-appearing deposits on the root of mesentery

Histopathology

Pathological examination of the surgical specimen revealed:

• Poorly differentiated adenocarcinoma of the stomach with perforation
• Positive lymph node involvement (1 out of 10 nodes)
• Positive immunohistochemistry markers: CK20, CDX2, CK7
• Negative synaptophysin

Previous Treatment

Following surgery, the patient received systemic chemotherapy:

• 12 cycles of CAPEOX regimen (capecitabine and oxaliplatin)
• Completed the full course

Despite this comprehensive treatment approach, the patient developed progressive abdominal distension due to recurrent ascites. This necessitated therapeutic paracentesis (fluid drainage) on three separate occasions within a two-month period, with approximately 3.5 liters of fluid removed during each procedure. The frequent need for these interventions significantly impacted her quality of life and functional status.

Clinical Assessment

At presentation, the patient reported:

• Progressive abdominal distension
• Discomfort and limited mobility
• Decreased quality of life due to frequent paracentesis requirements

Diagnostic Evaluation

Recent investigations included:

MRI Abdomen (February 23, 2024):

• Persistent abdominopelvic ascites
• Omental-peritoneal nodularity and strand changes
• Clumped small bowel loops without frank obstruction
• Normal-appearing liver

PET-CT Scan:

• Moderate ascites
• Mildly thickened peritoneal reflections
• Stable non-avid right adrenal adenoma
• Stable non-avid pancreatic cyst

Tumor Markers:

• CA 125: 7.2 U/ml
• CA 19-9: 30.75 U/ml
• CEA: 2.39 ng/ml

Treatment Decision and Implementation

Multidisciplinary Assessment

The patient’s case was evaluated by a multidisciplinary team including:

• Dr. Mohammed Basheeruddin Inamdar (Specialist Surgical Oncologist and Robotic Surgeon)
• Dr. Prasanta Kumar Dash (Specialist Medical Oncology)
• Dr. Mehdi (additional team member)

After comprehensive review of her clinical history, imaging studies, and consideration of available treatment options, the team proposed PIPAC as an innovative approach to address her recurrent malignant ascites.

Rationale for PIPAC Selection

Several factors influenced the decision to proceed with PIPAC:

1. Failure of Conventional Therapy: Despite completing 12 cycles of systemic chemotherapy, the patient had developed progressive peritoneal disease with recurrent malignant ascites.
2. Symptom Burden: The need for repeated paracentesis procedures (three times in two months) indicated significant symptom burden and quality-of-life impact.
3. Localized Disease Control: The patient’s disease appeared predominantly confined to the peritoneal cavity, making a local-regional approach appropriate.
4. Age and Performance Status: At 72 years, the patient would benefit from a less toxic, minimally invasive approach rather than aggressive systemic therapy.
5. Therapeutic Goal: Palliative management focused on symptom control and quality-of-life improvement aligned well with PIPAC’s capabilities.

Procedure Details

The PIPAC procedure was performed under general anesthesia using minimally invasive techniques:

1. Access: Two small keyhole incisions (5mm and 12mm) were created for laparoscopic access
2. Assessment: Diagnostic laparoscopy was performed to visualize the extent of peritoneal disease
3. Preparation: After evacuation of ascitic fluid, the abdomen was inflated with carbon dioxide to create a pneumoperitoneum
4. Aerosolization: Chemotherapy drugs were aerosolized using a specialized nebulizer (CapnoPen) connected to a high-pressure injector and delivered into the peritoneal cavity
5. Exposure: The aerosol was maintained under pressure within the peritoneal cavity for a predetermined period to allow optimal tissue penetration
6. Evacuation: Following the treatment phase, the aerosol was safely evacuated through a closed filtration system
7. Closure: The small incisions were closed, completing the minimally invasive procedure

The entire procedure was completed as a day case surgery, with the patient discharged the same day after recovery from anesthesia.

Significance and Innovation

Regional Context

The implementation of PIPAC therapy at Burjeel Holdings represents a significant milestone in advanced cancer care in the region:

• Believed to be only the second case of PIPAC therapy performed in the United Arab Emirates
• Demonstrates the institution’s commitment to adopting innovative approaches for challenging oncological scenarios
• Expands the therapeutic options available to patients with peritoneal carcinomatosis in the region

Institutional Capabilities

The successful implementation of PIPAC therapy highlights several key institutional strengths:

1. Advanced Facilities: The procedure requires specialized equipment and technology available only at comprehensive cancer centers
2. Specialized Training: Surgical oncologists performing PIPAC require specific training and expertise in the technique
3. Multidisciplinary Approach: Collaboration between surgical oncology, medical oncology, and supporting specialties is essential for patient selection and management
4. Comprehensive Care: Ability to offer advanced palliative interventions represents an important component of complete cancer care

Clinical Implications

Patient Selection

PIPAC therapy is particularly beneficial for:

• Patients with peritoneal carcinomatosis from gastric, ovarian, colorectal, or other GI malignancies
• Those with recurrent malignant ascites requiring frequent paracentesis
• Individuals who have progressed on systemic therapy
• Patients seeking minimally invasive palliative options with low morbidity

Integration with Standard Care

PIPAC can be integrated into comprehensive cancer care in several ways:

• Palliative Setting: For symptom control and quality-of-life improvement
• Combination Approach: Alongside systemic chemotherapy for enhanced disease control
• Sequential Therapy: As part of a multimodal treatment strategy
• Clinical Trials: Within research protocols to establish optimal protocols and outcomes

Practical Advantages

From a practical perspective, PIPAC offers several important advantages:

• Day Case Procedure: Minimal hospitalization requirement
• Minimal Side Effects: Reduced systemic exposure compared to IV chemotherapy
• Repeatable: Can be performed multiple times as needed (typically every 6-8 weeks)
• Assessment Opportunity: Allows repeated visual assessment of treatment response
• Tissue Acquisition: Facilitates biopsy for molecular and histological evaluation

Future Directions

Ongoing Research

The field of PIPAC therapy continues to evolve, with several areas of active investigation:

• Optimization of drug selection and dosing
• Development of novel aerosolization devices
• Combination approaches with immunotherapy
• Personalization based on molecular profiling
• Expansion to additional tumor types

Institutional Development

Burjeel Holdings’ successful implementation of PIPAC therapy represents an opportunity for:

• Development of a specialized peritoneal malignancy program
• Participation in international collaborative research
• Training and education of additional specialists
• Expansion of treatment access to more patients in the region

Conclusion

The successful implementation of Pressurized Intraperitoneal Aerosolized Chemotherapy (PIPAC) at Burjeel Holdings demonstrates the institution’s commitment to advancing cancer care through innovative approaches. As one of the earliest applications of this technique in the UAE, it represents an important addition to the therapeutic armamentarium for patients with peritoneal carcinomatosis and malignant ascites.

PIPAC offers a minimally invasive, repeatable approach with enhanced drug delivery to peritoneal tissues while minimizing systemic exposure and side effects. For selected patients, particularly those with recurrent malignant ascites requiring frequent paracentesis procedures, PIPAC may provide improved symptom control and quality of life.

The multidisciplinary approach employed in this case, combining surgical oncology and medical oncology expertise, highlights the comprehensive cancer care available at Burjeel Holdings. As experience with the technique grows and research continues to refine protocols and applications, PIPAC therapy is likely to play an increasingly important role in the management of peritoneal malignancies.

Experts

Introduction

Kienbock’s disease, a relatively rare condition affecting the lunate bone in the wrist, presents significant challenges for both patients and surgeons. This progressive disorder is characterized by avascular necrosis (death of bone tissue due to insufficient blood supply) of the lunate, one of the eight small carpal bones that make up the wrist. Left untreated, the condition leads to wrist pain, diminished grip strength, limited motion, and eventually, debilitating arthritis.

This case study explores an advanced surgical approach – the vascularized bone graft – for managing a case of advanced Kienbock’s disease in a young patient. The procedure, performed at Burjeel Hospital in Abu Dhabi, demonstrates how microsurgical techniques can offer hope for patients with conditions previously considered difficult to treat effectively.

Understanding Kienbock’s Disease

Pathophysiology

Kienbock’s disease involves the progressive collapse of the lunate bone in the wrist due to compromised blood supply. This vascular disruption leads to bone necrosis, fragmentation, and eventual collapse, resulting in altered wrist mechanics, pain, and functional limitations.

The condition typically progresses through several stages:

• Stage I: Ischemia (reduced blood supply) without radiographic changes
• Stage II: Sclerosis (hardening) of the lunate visible on X-rays
• Stage III: Collapse and fragmentation of the lunate
  • Stage IIIA: Lunate collapse without scaphoid rotation
  • Stage IIIB: Lunate collapse with fixed scaphoid rotation
• Stage IV: Advanced collapse with degenerative changes throughout the wrist

Risk Factors

Several factors may contribute to the development of Kienbock’s disease:

• Anatomical variations: A shorter ulna relative to the radius (negative ulnar variance) may increase mechanical stress on the lunate
• Vascular anatomy: Variations in blood supply to the lunate can predispose to ischemia
• Trauma: History of wrist injury
• Systemic factors: Conditions affecting blood flow or coagulation
• Occupational factors: Activities involving repetitive wrist loading or vibration

Clinical Presentation

Patients with Kienbock’s disease typically present with:

• Wrist pain, particularly during activities
• Swelling and tenderness over the lunate area
• Limited range of motion
• Decreased grip strength
• In advanced stages, wrist instability and crepitus

Case Presentation

Patient Profile

A 31-year-old female presented with persistent pain in her left wrist of approximately one year’s duration. Her medical history included a traumatic injury to the left wrist one year prior, which was conservatively managed at another facility. Despite this management, she continued to experience persistent wrist pain, leading to her referral to Burjeel Hospital with a diagnosis of Kienbock’s disease.

Clinical Findings

On examination, the patient exhibited:

• Diffuse swelling around the left wrist
• Tenderness throughout the wrist, particularly over the lunate area
• Painful and restricted range of motion

Diagnostic Imaging

The radiographic assessment revealed:

• X-rays showing sclerosis of the lunate
• Loss of height of the lunate bone
• Fragmentation of the lunate structure

Magnetic Resonance Imaging (MRI) confirmed these findings and supported the diagnosis of advanced Kienbock’s disease, classified as Stage 3A according to Lichtman’s classification system. At this stage, the lunate had undergone collapse but without scaphoid rotation or carpal instability.

Treatment Approach

Surgical Options Assessment

For Stage 3A Kienbock’s disease, several surgical options exist:

1. Proximal Row Carpectomy: Removal of the scaphoid, lunate, and triquetrum bones
2. Limited Wrist Fusion: Joining certain carpal bones to redistribute forces
3. Revascularization Procedures: Techniques to restore blood supply to the lunate
4. Total Wrist Arthrodesis: Complete fusion of the wrist in severe cases
5. Denervation Procedures: To reduce pain while maintaining some motion

After thorough discussion with the patient regarding the risks, benefits, and long-term outcomes of each approach, a revascularization procedure was selected. This decision was based on the patient’s young age, the stage of disease, and the desire to preserve wrist function.

Surgical Technique

The patient underwent a specialized procedure combining several techniques:

1. 4+5 Extensor Compartmental Artery-Based Vascularized Bone Graft: This microsurgical procedure involves harvesting bone with its blood supply from the dorsal distal radius and transferring it to the lunate to provide both structural support and blood supply.
2. Posterior Interosseous Nerve (PIN) Neurectomy: Division of a nerve branch to reduce pain signals from the affected area.
3. Scapho-capitate Pinning: Temporary stabilization of these carpal bones with Kirschner wires (K-wires) to maintain proper alignment during healing.

Technical Challenges

The surgical team faced several challenges during this procedure:

1. Microsurgical Complexity: The harvesting of bone with its minute blood vessel supply requires advanced microsurgical expertise and specialized equipment.
2. Anatomical Variations: The 4+5 extensor compartmental artery is small and can have variable anatomy between patients.
3. Vascular Pedicle Preservation: Maintaining the viability of the blood vessels during transfer is critical and technically demanding.
4. Recipient Site Preparation: Creating an appropriate cavity in the necrotic lunate while preserving its outer shell.

Surgical Implementation

The procedure was performed under general anesthesia with a tourniquet applied to the upper arm. A detailed step-by-step approach was followed:

1. A dorsal approach to the wrist was utilized to expose both the 4th and 5th extensor compartments and the lunate.
2. The Posterior Interosseous Nerve was identified and neurectomy performed to provide pain relief.
3. The lunate was accessed and a cavity created after curettage of the necrotic bone while preserving its outer shell.
4. A vascularized bone graft based on the 4+5 extensor compartmental artery was harvested from the distal radius, with careful preservation of its vascular pedicle.
5. The vascularized bone graft was transferred and inserted into the prepared cavity in the lunate.
6. Temporary scapho-capitate pinning was performed with K-wires to provide stability during the healing process.
7. Meticulous closure was performed in layers.

Intraoperative photographs documented key steps of the procedure, including:

• PIN nerve identification before neurectomy
• Preparation of the cavity in the lunate after curettage
• Harvesting of the bone graft with its vascular pedicle
• Insertion of the vascularized bone graft into the lunate cavity

Post-Operative Course

The perioperative period was uneventful, with the patient discharged on the same day following appropriate pain management and wound care instructions. A below-elbow splint was applied to protect the wrist during the initial healing phase.

Post-operative radiographs demonstrated:

• Restoration of lunate height
• Proper positioning of the scapho-capitate K-wires
• Maintained carpal alignment

Clinical Outcome

At the six-month follow-up assessment, the patient reported significant improvement:

• Pain-free wrist
• Ability to perform daily activities without discomfort
• Progressive improvement in range of motion
• Return to normal hand function for most activities

Radiographic evaluation at six months showed signs of lunate revascularization with maintenance of carpal height and alignment.

Discussion

Significance of Vascularized Bone Grafting

Vascularized bone grafting represents an important advancement in the management of Kienbock’s disease, particularly for younger patients in whom preservation of wrist mechanics is crucial. The technique offers several advantages over conventional approaches:

1. Biological Reconstruction: Provides both structural support and blood supply to the necrotic lunate.
2. Potential Disease Modification: May halt the progression of avascular necrosis and prevent further collapse.
3. Preservation of Natural Anatomy: Maintains normal carpal relationships and biomechanics.
4. Avoidance of Salvage Procedures: May prevent the need for more drastic surgeries like proximal row carpectomy or wrist fusion.

Contraindications and Limitations

Despite its benefits, vascularized bone grafting is not suitable for all patients with Kienbock’s disease:

• Advanced Stages (Stage IV): When significant degenerative changes are present throughout the wrist
• Technical Demands: Requires microsurgical expertise and equipment
• Recovery Period: Longer rehabilitation compared to some alternative procedures
• Limited Evidence Base: Long-term outcomes still being established through research

Comparison with Alternative Treatments

For Stage 3A Kienbock’s disease, the main treatment alternatives include:

1. Proximal Row Carpectomy (PRC):
   • Advantages: Reliable pain relief, maintained motion
   • Disadvantages: Loss of carpal height, potential grip weakness, non-reversible
2. Limited Wrist Fusion:
   • Advantages: Stable construct, pain relief
   • Disadvantages: Reduced wrist motion, potential non-union
3. Vascularized Bone Graft:
   • Advantages: Preserves native anatomy, biological solution, reversible
   • Disadvantages: Technical complexity, variable outcomes

The selection of vascularized bone grafting in this case reflects a treatment philosophy prioritizing anatomical preservation and function, particularly appropriate for a young patient.

Clinical Implications

This case demonstrates several important points for practitioners:

1. Early Diagnosis: Recognition of Kienbock’s disease before advanced collapse is crucial for preserving treatment options.
2. Stage-Appropriate Treatment: Surgical approach should be tailored to the disease stage, patient age, and functional demands.
3. Microsurgical Advances: Techniques previously considered experimental are increasingly becoming viable options with experienced surgical teams.
4. Team Approach: Collaboration between hand surgeons, radiologists, and rehabilitation specialists optimizes outcomes.

Conclusion

Vascularized bone grafting represents an advanced approach for managing Kienbock’s disease that aims to restore both structure and blood supply to the affected lunate. This case illustrates the successful application of a 4+5 extensor compartmental artery-based vascularized bone graft in a young patient with Stage 3A disease, resulting in pain relief and return to function without resorting to more radical salvage procedures.

The complexity of the procedure necessitates specialized training and expertise, but in appropriate cases and experienced hands, it offers the potential to halt disease progression while preserving wrist anatomy and function. As microsurgical techniques continue to evolve, such reconstructive approaches may become increasingly important in the treatment algorithm for Kienbock’s disease and other conditions characterized by avascular necrosis.

This case adds to the growing body of evidence supporting the role of vascularized bone grafting in the management of selected cases of advanced Kienbock’s disease, offering hope for improved outcomes in this challenging condition.

Experts

Introduction

Complex Regional Pain Syndrome (CRPS) represents one of the most challenging pain conditions to manage effectively. Characterized by intense pain disproportionate to the inciting event, along with autonomic, sensory, and motor abnormalities, CRPS can be severely debilitating for patients. This case study explores the successful implementation of lumbar sympathetic block as an interventional pain management technique for CRPS following knee surgery, highlighting its efficacy in providing relief when conservative treatments fail.

Understanding Lumbar Sympathetic Block

A lumbar sympathetic block is a minimally invasive pain management procedure designed to disrupt the nerve supply from the sympathetic chain to the lower extremities. This intervention targets the sympathetic nervous system, which plays a critical role in pain perception and transmission, particularly in conditions with sympathetically maintained pain components.

Anatomical Considerations

The lumbar sympathetic ganglia form a chain of nerve cell bodies that run parallel to the vertebral column in the retroperitoneal space. The densest portion of these ganglia is typically found at the L2 and L3 vertebral levels, making these the optimal targets for intervention. The procedure is most commonly performed at the L3 level, providing effective disruption of sympathetic innervation to the lower limbs.

Procedural Approach

The lumbar sympathetic block procedure typically follows a two-step approach:

1. Diagnostic Block: Initially, a diagnostic block using only local anesthetic is administered to assess the patient’s response. This serves as a predictor of success for more definitive interventions.
2. Therapeutic Intervention: If optimal relief is achieved with the diagnostic block, radiofrequency ablation is subsequently performed at the L2, L3, and L4 levels to provide longer-lasting relief.

Primary Indications

Lumbar sympathetic blocks are primarily indicated for several painful conditions affecting the lower extremities:

• Complex Regional Pain Syndrome (CRPS)
• Phantom limb pain
• Hyperhidrosis (excessive sweating)
• Vascular insufficiencies
• Herpes zoster affecting the lower limbs

Understanding Complex Regional Pain Syndrome

CRPS is a disabling pain condition that typically affects limbs following an injury or surgery. The condition manifests with pain that is disproportionate to the inciting event and is associated with abnormal sensory, motor, sudomotor, vasomotor, and trophic findings.

Classification

CRPS is classified into two types:

1. CRPS Type 1 (Reflex Sympathetic Dystrophy): Occurs when there is no obvious nerve injury. This type primarily involves nociceptive pain mechanisms.
2. CRPS Type 2 (Causalgia): Occurs when there is an obvious nerve injury. This type involves significant neurogenic components of pain.

In clinical practice, these conditions often present with overlapping symptoms, making clear distinction challenging in some cases.

Clinical Presentation

Patients with CRPS typically present with a constellation of symptoms:

• Severe, burning pain
• Paresthesia (abnormal sensations)
• Edema (swelling)
• Limited range of motion
• Temperature changes in the affected limb
• Skin changes, including color and texture alterations
• Hyperalgesia (increased sensitivity to pain)
• Allodynia (pain from stimuli that are not normally painful)

Pathophysiology

While the definitive pathophysiology of CRPS remains incompletely understood, current evidence suggests it is a disorder of the immune system that affects the nervous system. The condition involves complex interactions between:

• Peripheral and central sensitization
• Neurogenic inflammation
• Autonomic dysregulation
• Immune system activation
• Psychological factors

Diagnosis

Diagnosis of CRPS is primarily clinical, based on established criteria such as the Budapest Criteria, which include:

1. Continuing pain disproportionate to any inciting event
2. At least one symptom in each of the four categories: sensory, vasomotor, sudomotor/edema, and motor/trophic
3. At least one sign at time of evaluation in two or more categories
4. No other diagnosis better explaining the signs and symptoms

Case Study: CRPS Following Knee Surgery

Patient Profile

A young male patient was referred to the pain management service from the orthopedic department at Burjeel Day Surgery Center, Al Reem Island. The patient had undergone right knee surgery three months prior and subsequently developed symptoms consistent with CRPS.

Clinical Presentation

Upon evaluation, the patient presented with:

• High skin temperature in the affected limb
• Severe pain (VAS 7/10)
• Mild swelling
• Hypoesthesia (decreased sensation) along the lateral limb
• Allodynia (pain from non-painful stimuli) along the anterior aspect of the limb
• Limited range of motion

Previous Management

The patient had previously received conservative management, including:

• Analgesic medications
• Physical therapy
• Activity modification

Unfortunately, these conventional approaches proved ineffective in providing adequate pain relief or functional improvement.

Intervention Plan

After comprehensive evaluation and proper counseling regarding expectations and potential outcomes, a management plan was formulated:

1. Perform a diagnostic lumbar sympathetic block at the right L3 level to assess response
2. If favorable response achieved, proceed with radiofrequency ablation for longer-term relief
3. Continue multimodal pain management alongside interventional approaches

Procedure Details

Under appropriate monitoring and sterile conditions, a lumbar sympathetic diagnostic block was performed at the right L3 level. Local anesthetic was precisely delivered to the sympathetic chain using fluoroscopic guidance to ensure accurate needle placement.

Outcomes

The results of the intervention were impressive:

• The patient reported being pain-free immediately following the procedure
• Significant improvement in range of motion was observed
• Reduced allodynia in the affected limb
• Normalization of skin temperature

Based on this positive response to the diagnostic block, the patient was scheduled for radiofrequency ablation to provide more sustained relief. The treatment plan continues at Burjeel Day Surgery Center, Al Reem Island.

Discussion

Efficacy of Lumbar Sympathetic Block in CRPS

This case illustrates the potential efficacy of lumbar sympathetic blockade in managing CRPS of the lower extremity. While CRPS is notoriously difficult to treat and often has no definitive cure, timely interventional procedures can:

1. Slow disease progression
2. Provide crucial pain relief
3. Improve function and quality of life
4. Help patients cope with daily activities
5. Potentially prevent long-term disability

Timing of Intervention

Early intervention is critical in CRPS management. Research suggests that outcomes are generally better when appropriate treatments are initiated within 3-6 months of symptom onset. In this case, the patient was referred approximately three months after surgery, allowing for timely intervention before the condition became more refractory to treatment.

Limitations of Treatment

It’s important to acknowledge certain limitations in CRPS management:

• CRPS typically has no definitive cure
• Treatment responses can be variable
• Multiple interventions may be necessary
• A multimodal approach is often required

Advantages of Lumbar Sympathetic Block

As demonstrated in this case, lumbar sympathetic blockade offers several advantages:

1. Minimally Invasive: The procedure requires only needle insertion rather than open surgery
2. Diagnostic and Therapeutic: Can serve both to confirm sympathetically maintained pain and provide relief
3. Targeted Approach: Directly addresses the sympathetic component of pain
4. Low Complication Rate: When performed by experienced practitioners
5. Option for Progression: Can progress from diagnostic blocks to more permanent interventions like radiofrequency ablation

Clinical Implications

This case highlights several important clinical implications for pain practitioners:

1. Early Recognition: Identifying CRPS early improves outcomes. Healthcare providers should maintain a high index of suspicion for CRPS in patients with disproportionate pain following surgery or injury.
2. Prompt Referral: Timely referral to pain specialists when conservative measures fail can prevent progression to chronic, refractory pain states.
3. Stepped Approach: A graduated approach from diagnostic blocks to more definitive interventions allows for personalized treatment planning.
4. Multimodal Strategy: Even with successful interventional procedures, a comprehensive approach including physical therapy, psychological support, and appropriate medications optimizes outcomes.

Conclusion

Lumbar sympathetic block represents a safe and effective procedure for alleviating pain in patients with CRPS who are refractory to medical management. This case demonstrates the potential for significant improvement in pain and function following appropriate interventional management.

While CRPS remains a challenging condition to treat, a timely, multimodal approach that includes targeted interventions like lumbar sympathetic blockade can significantly improve patient outcomes and quality of life. This case emphasizes the importance of early diagnosis, prompt referral to pain specialists, and individualized treatment planning in the management of complex pain conditions.

For patients suffering from CRPS of the lower extremities who have not responded to conservative management, lumbar sympathetic block should be considered as part of a comprehensive pain management strategy.

Experts

Introduction

Infective endocarditis is a serious infection of the endocardial surface of the heart, often affecting one or more heart valves. When complicated by septic shock, it presents a significant clinical challenge with high mortality rates, requiring prompt medical intervention and potentially emergent surgical management. This case study details the successful management of a complex case of septic shock and endocarditis in a 42-year-old male patient, marking a significant milestone as the first cardiac surgery performed at Burjeel Specialty Hospital, Sharjah.

Patient Presentation

A 42-year-old Filipino male presented to a non-Burjeel healthcare facility on April 4, 2024, with complaints of sudden-onset breathing difficulty that began at approximately 10:30 PM that same evening. Upon arrival at the emergency department, the patient was in a gasping state, unresponsive, and exhibiting concerning symptoms of desaturation and hypotension.

Initial Emergency Management

The severity of the patient’s condition necessitated immediate intervention. The emergency team promptly:

• Initiated endotracheal intubation to secure the airway
• Established inotropic support to stabilize blood pressure
• Began diagnostic workup to identify the underlying cause

Diagnostic Findings

The initial diagnostic evaluation resulted in a diagnosis of:

• Septic shock
• Cardiogenic shock
• Multi-organ failure

Further history revealed that the patient had been experiencing:

• Intermittent fever for three months
• Significant weight loss
• Known thyroid disease with no surgical history

Due to bed unavailability at the initial facility, the patient was transferred to Medeor Hospital the following day. There, additional diagnostic tests were performed.

Key Laboratory Findings

Laboratory tests demonstrated:

• Elevated white blood cell count
• Markedly elevated C-reactive protein (CRP)
• High procalcitonin levels, strongly suggestive of bacterial infection

Imaging and Microbiological Results

• 2D Echocardiography: Revealed large vegetation on the mitral anterior leaflet with severe mitral regurgitation
• Blood Cultures: Identified Streptococcus species from samples taken at the non-Burjeel facility

Comprehensive Diagnosis

Based on the clinical presentation and diagnostic findings, the patient was diagnosed with:

1. Rheumatic Heart Disease
2. Severe Mitral Regurgitation
3. Infective Endocarditis
4. Severe Pulmonary Arterial Hypertension (PAH)
5. Acute Kidney Injury (AKI)

Treatment Course

Initial Medical Management

The patient showed a positive response to antimicrobial therapy with:

• Vancomycin
• Linezolid

Within two weeks of initiating antibiotic therapy, the patient’s CRP levels normalized, indicating effective control of the infection. He was subsequently discharged home on oral Linezolid tablets.

Surgical Planning

Following stabilization and initial treatment of the acute infection, the patient was scheduled for mitral valve replacement at Burjeel Specialty Hospital, Sharjah. This procedure would represent a significant milestone as the first cardiac surgery to be performed at this facility.

Surgical Intervention

Procedure Details

The surgical team performed:

1. Mitral valve replacement using a 31 mm Carbomedics mechanical valve
2. Excision of vegetation from the aortic valve
3. Repair of the aortic valve, where small vegetation was identified on the non-coronary cusps

Surgical Team

The landmark procedure was performed by an expert team comprising:

• Dr. Dinesh Sadasival, Consultant Cardiothoracic Surgeon
• Dr. Tarig Ali, HOD, Cardiothoracic Surgeon
• Dr. Y. A. Nazer, Consultant Cardiac Surgery
• Dr. Arun Vijaykumar, Cardiac Anesthesia
• Dr. Sival Pillai, HOD, Cardiac Anesthesia

Discussion

This case highlights several important clinical considerations in the management of infective endocarditis complicated by septic shock:

Diagnostic Challenges

Infective endocarditis can present with varied and nonspecific symptoms, making early diagnosis challenging. In this case, the patient had experienced prolonged fever for three months before presenting in extremis with respiratory distress. This underscores the importance of considering endocarditis in patients with:

• Unexplained, persistent fever
• Significant weight loss
• New cardiac murmurs
• Predisposing factors such as rheumatic heart disease

Timing of Surgical Intervention

The timing of surgical intervention in infective endocarditis requires careful consideration. Key factors include:

1. Hemodynamic stability: In this case, initial stabilization with medical therapy was necessary before proceeding to surgery.
2. Control of infection: The two-week course of antibiotics resulting in normalization of inflammatory markers indicated adequate control of the acute infection.
3. Prevention of complications: Surgery was planned to prevent further complications such as embolization of vegetation, progressive valve destruction, and persistent heart failure.

Choice of Prosthetic Valve

The decision to use a mechanical valve (31 mm Carbomedics) in this 42-year-old patient aligns with current guidelines that often favor mechanical valves in younger patients who can tolerate long-term anticoagulation. Factors influencing this decision typically include:

• Patient age
• Anticoagulation compliance
• Risk of reinfection
• Hemodynamic performance of the valve

Management of Multi-Valve Involvement

This case also demonstrates the surgical approach to multi-valve involvement in endocarditis. While the mitral valve required replacement due to severe damage, the aortic valve had only small vegetation on the non-coronary cusps, allowing for successful repair rather than replacement. This valve-sparing approach offers several advantages:

• Avoids the need for a second prosthetic valve
• Reduces the risk of prosthetic valve endocarditis
• Preserves native valve hemodynamics
• Potentially reduces the intensity of required anticoagulation

Clinical Significance

This case holds particular significance as the first cardiac surgery performed at Burjeel Specialty Hospital, Sharjah. It demonstrates:

1. Successful multidisciplinary collaboration between emergency medicine, infectious disease, cardiology, and cardiac surgery.
2. Effective management of a life-threatening condition through appropriate medical stabilization followed by definitive surgical intervention.
3. Expansion of advanced cardiac surgical capabilities to Burjeel Specialty Hospital, Sharjah, enhancing access to specialized cardiac care in the region.

Conclusion

This landmark case of successful management of septic shock and infective endocarditis demonstrates the importance of prompt diagnosis, appropriate antimicrobial therapy, and timely surgical intervention. The successful outcome underscores the value of a multidisciplinary approach to complex cardiac conditions and highlights the expanding capabilities of cardiac surgical centers in the region.

The case serves as an important reminder of the need for high clinical suspicion for infective endocarditis in patients with prolonged fever and weight loss, particularly in those with underlying structural heart disease. Early diagnosis and appropriate management remain critical in reducing morbidity and mortality in this challenging clinical entity.

Experts

Introduction

Pancreatic surgery has historically been associated with significant morbidity and mortality. However, advancements in robotic surgical technology have revolutionized the approach to complex pancreatic procedures. This case study highlights the successful implementation of a totally robotic spleen-sparing distal pancreatectomy for a patient with multiple pancreatic neuroendocrine tumors, demonstrating the precision and advantages of robotic-assisted surgery in preserving critical organs while effectively treating pathology.

Patient Presentation and History

A 58-year-old male with multiple comorbidities including type 2 diabetes, hypertension, and hyperlipidemia initially presented with back pain in 2019. Despite not exhibiting classic symptoms typically associated with pancreatic tumors (such as jaundice, weight loss, or digestive difficulties), diagnostic imaging revealed concerning findings that warranted further investigation.

The patient’s initial workup included:

• MRI and MRCP (Magnetic Resonance Cholangiopancreatography) scans that detected a non-functioning pancreatic tumor
• Identification of a large cystic lesion in the liver
• Institution of regular imaging surveillance due to the asymptomatic nature of the findings

Comprehensive Diagnostic Evaluation

In March 2024, following a period of watchful waiting with regular imaging follow-ups, the patient underwent a comprehensive diagnostic evaluation to better characterize the lesions and determine appropriate management.

Advanced Imaging and Tissue Sampling

1. Endoscopic Ultrasound (EUS)
   • Revealed a 3.4 cm cystic lesion in the pancreatic body and tail region
   • Identified a concurrent liver lesion in the right lobe
   • Facilitated fine needle aspiration (FNA) biopsies of both lesions
2. Histopathological Analysis
   • Pancreatic lesion: Confirmed a well-differentiated Grade 1 neuroendocrine tumor
   • Liver lesion: Showed evidence of steatohepatitis without malignancy
3. CT Scan of Abdomen and Pelvis
   • Documented progression with multiple pancreatic lesions:
     • 3.3 × 3.3 cm lesion in the mid-body
     • 10 mm lesion in the tail
     • 9 mm lesion in the proximal body
   • Revealed a 4 × 3 cm lobulated cystic lesion in segment 6 of the liver
4. DOTA PET Scan
   • Demonstrated somatostatin receptor-expressing lesions in the pancreas
   • Identified additional satellite lesions
   • Provided functional characterization of the tumors

Multidisciplinary Approach to Treatment Planning

Despite negative tumor markers and normal serum Chromogranin-A levels (a biomarker typically elevated in neuroendocrine tumors), a multidisciplinary team (MDT) recommended surgical intervention based on the imaging findings and histological confirmation. The patient was specifically referred to the Department of Gastrointestinal Surgery for consideration of robotic surgery—an approach that would allow for precise tumor removal while potentially preserving the spleen.

Surgical Approach and Technical Considerations

Following thorough preoperative counseling and evaluation, the surgical team proceeded with a totally robotic spleen-sparing distal pancreatectomy. This approach offered several key advantages:

1. Enhanced Visualization: The robotic system provided magnified, high-definition 3D visualization of the surgical field, essential for identifying and preserving the splenic vessels.
2. Intraoperative Ultrasonography: Used to precisely locate the tumors and guide the extent of resection, ensuring complete removal of all lesions while preserving maximum healthy pancreatic tissue.
3. Precision Dissection: The robotic instruments allowed for meticulous dissection around critical vascular structures, particularly important in separating the pancreas from the splenic vessels.
4. Minimal Blood Loss: The procedure was completed with less than 100 mL of blood loss, significantly lower than traditional open approaches.
5. Efficient Operative Time: Despite the complexity of the procedure, the operation was completed in 5 hours.

Surgical Technique: Preserving the Spleen While Removing Diseased Pancreas

The spleen-sparing technique employed in this case deserves special attention. In traditional distal pancreatectomy, the spleen is often removed along with the distal pancreas because of their shared blood supply. However, preserving the spleen offers significant immunological benefits, particularly important for long-term health and prevention of post-splenectomy infections.

The robotic approach facilitated:

1. Warshaw Technique Modification: Preservation of the splenic vessels while completely mobilizing the pancreas from surrounding structures.
2. Selective Vascular Control: Precise ligation of small pancreatic vessels without compromising the main splenic vasculature.
3. Real-time Assessment: Continuous evaluation of splenic perfusion throughout the procedure.

Postoperative Course and Outcomes

The patient experienced an exceptionally smooth recovery:

• Minimal postoperative pain, effectively managed with standard analgesics
• Resumed oral intake on the first postoperative day
• Normalized bowel function by the second postoperative day
• Discharged home on the third postoperative day
• No evidence of pancreatic fistula or other complications
• Maintained excellent follow-up status with preserved glycemic control

Technical Challenges and Considerations

The case presented several technical challenges that highlight why robotic approaches offer advantages in complex pancreatic surgery:

1. Patient Factors: The patient’s obesity represented a technical challenge that was mitigated by the enhanced dexterity and visualization of the robotic system.
2. Multiple Pancreatic Neoplasms: The presence of several tumors throughout the distal pancreas required precise localization and comprehensive resection.
3. Splenic Preservation: Maintaining adequate blood supply to the spleen while completely removing the distal pancreas was technically demanding, particularly due to the intricate vascular anatomy behind the pancreas.

Discussion: Advantages of Robotic Approach in Pancreatic Surgery

This case exemplifies the unique advantages of robotic-assisted surgery for complex pancreatic procedures:

Enhanced Technical Capabilities

The da Vinci Robotic Surgical System offers:

• Superior Visualization: High-definition 3D imaging allows for better appreciation of fine tissue planes.
• Increased Dexterity: The robotic “wrists” enable 540-degree rotation and movement in tight spaces, critical when working around major vessels.
• Tremor Filtration: Elimination of natural hand tremor enhances precision during delicate dissection.
• Improved Ergonomics: The surgeon’s improved positioning reduces fatigue during lengthy procedures.

Clinical Benefits

Patients undergoing robotic pancreatic surgery often experience:

• Reduced blood loss compared to open procedures
• Lower conversion rates to open surgery compared to conventional laparoscopy
• Shorter hospital stays
• Faster return to normal activities
• Improved cosmetic outcomes due to smaller incisions

Organ Preservation Benefits

The spleen plays critical roles in:

• Immune function, particularly against encapsulated bacteria
• Blood filtration and recycling of red blood cells
• Serving as a blood reservoir

Preserving this organ provides long-term benefits to the patient, including reduced risk of overwhelming post-splenectomy infection (OPSI) and maintained hematological function.

Conclusion

This case demonstrates the successful application of robotic technology in performing a complex spleen-sparing distal pancreatectomy. The robotic approach facilitated precise tumor removal while preserving the immunologically important spleen, despite the technical challenges posed by the patient’s body habitus and the presence of multiple neoplasms.

The excellent postoperative outcome, characterized by minimal blood loss, short hospital stay, and rapid recovery, underscores the potential benefits of robotic surgery in selected patients with pancreatic pathology. As this technology continues to evolve and surgical teams gain more experience, robotic approaches may become increasingly important in the management of complex pancreatic diseases.

This case, managed by Dr. Ali Iyoob Valiyaveettil, Consultant & Head of Gastrointestinal Surgery, serves as an excellent example of how cutting-edge robotic technology can be leveraged to achieve optimal surgical outcomes while prioritizing organ preservation and minimizing surgical trauma.

Experts

Introduction

Intussusception, a condition where one segment of the intestine telescopes into an adjacent segment, is predominantly seen in children but rarely occurs in adults. When it does appear in adults, it presents unique diagnostic and treatment challenges that require careful consideration. This case study examines an unusual presentation of ileocolic intussusception in a middle-aged patient and highlights the importance of prompt surgical intervention.

Patient Presentation

A previously healthy 56-year-old woman presented to the emergency department with a two-day history of concerning gastrointestinal symptoms. Her chief complaints included:

• Lower abdominal pain that had progressively worsened
• Multiple episodes of vomiting
• Constipation with no bowel movements for two days

These symptoms had developed suddenly with no apparent trigger, leading to her seeking medical attention after conservative measures at home failed to provide relief.

Diagnostic Journey

Initial Assessment

Upon presentation, the patient appeared uncomfortable but had stable vital signs. Physical examination revealed tenderness in the lower right quadrant of the abdomen with mild distension. The initial clinical impression suggested a possible bowel obstruction, necessitating further investigation.

Laboratory Findings

Blood work demonstrated a mild elevation of inflammatory markers:

• Slightly elevated white blood cell count
• Moderately increased C-reactive protein levels
• Other laboratory parameters remained within normal ranges

Imaging Studies

A computed tomography (CT) scan of the abdomen and pelvis proved crucial for diagnosis, revealing:

• Classic “target sign” indicating ileocecal intussusception
• Proximally dilated small bowel loops suggestive of partial obstruction
• No evidence of perforation or ischemia

Based on these findings, a diagnosis of ileocolic intussusception was established, and the patient was prepared for urgent surgical intervention.

Surgical Management

Given the patient’s age and the rarity of intussusception in adults, the surgical team approached the case with careful consideration that the condition likely had a pathological lead point.

Operative Approach

The patient underwent laparotomy, which confirmed the preoperative diagnosis of ileocolic intussusception. The surgical procedure involved:

• Careful manual reduction of the intussusception
• Identification of a large, smooth-surfaced, rubbery ileal polyp as the lead point
• Ileocecal resection with primary ileocolic anastomosis

The decision for bowel resection rather than simple reduction was based on the understanding that adult intussusception typically has a pathological lead point that requires removal.

Histopathological Findings

Examination of the resected specimen confirmed:

• A benign ileal polyp measuring approximately 3.5 cm in diameter
• No evidence of dysplasia or malignancy
• Normal surrounding mucosa

These findings validated the surgical approach and provided reassurance regarding the patient’s prognosis.

Post-Operative Course

The patient’s recovery proceeded smoothly with:

• Gradual return of bowel function on the second postoperative day
• Successful transition from intravenous to oral nutrition
• Adequate pain control with standard analgesics
• No postoperative complications

She was discharged home on the fourth postoperative day with appropriate follow-up arrangements and dietary guidance.

Discussion

Uniqueness of Adult Intussusception

This case highlights several important aspects of adult intussusception:

1. Epidemiological rarity: While common in children, intussusception accounts for only 1-5% of all cases of intestinal obstruction in adults.
2. Etiology differences: Unlike pediatric cases that are usually idiopathic, adult intussusception nearly always has a pathological lead point, which in this case was a benign ileal polyp.
3. Surgical management: The standard approach in adults is resection rather than reduction alone due to the high likelihood of pathological lead points and potential for malignancy.

Diagnostic Challenges

Diagnosing adult intussusception can be challenging due to:

• Non-specific symptoms that can mimic other abdominal conditions
• Chronic, intermittent presentations in some cases
• Varying clinical presentations depending on location and duration

In this case, the acute presentation and comprehensive imaging studies facilitated timely diagnosis and intervention.

Surgical Considerations

The surgical management of this case illustrates important decision-making factors:

• Assessment of the lead point’s nature during surgery
• Determining appropriate extent of resection
• Balancing oncological principles with preservation of bowel length

The confirmation of a benign pathology justified the limited resection performed.

Conclusion

This case demonstrates the successful management of adult ileocolic intussusception caused by a benign ileal polyp. Despite being uncommon in adults, intussusception should remain in the differential diagnosis for patients presenting with bowel obstruction symptoms. The case underscores the importance of prompt surgical intervention and careful pathological assessment in adult intussusception cases.

The patient’s smooth recovery and uncomplicated postoperative course highlight the effectiveness of timely surgical management. This case, managed by Dr. Talat Shaban Abdullah Al-Shaban, Consultant General Surgeon, represents a textbook example of the proper approach to adult intussusception.

Key Learning Points

• Adult intussusception is rare but should be considered in the differential diagnosis for bowel obstruction.
• CT scanning is the imaging modality of choice for diagnosis.
• Unlike in children, adult intussusception typically requires surgical resection rather than simple reduction.
• Identifying the nature of the lead point is crucial for determining the extent of surgical resection.
• Most adult intussusceptions have an identifiable lead point, which can range from benign polyps to malignant lesions.

Experts

A novel technique combining laparoscopic surgery with on-table flexible ureteroscopy has been successfully implemented at Lifecare Hospital, a Burjeel Holdings facility, offering new hope for patients with complex ureteric strictures.

Complex Case Presentation

A 41-year-old male presented with persistent left flank pain and recurrent urinary tract infections over a one-year period. His medical history revealed a previous ureteroscopic intervention for a left mid-ureteric stricture that had developed secondary to an impacted ureteric stone. The initial management included balloon dilation and stenting, but despite these standard interventions, the patient experienced symptom recurrence indicative of stricture reformation.

Diagnostic ultrasound demonstrated left-sided hydronephrosis, while previous intraoperative retrograde ureterogram had confirmed a mid-ureteric stricture approximately 2 cm in length with proximal ureteral dilation. Laboratory tests showed normal renal function with no active infection at the time of presentation.

Innovative Surgical Approach

The urology team at Lifecare Hospital, led by Dr. Althaf Hussain and Dr. Anand Srivastava, implemented a novel combined approach that integrated both laparoscopic and endourological techniques in the same procedure.

Traditional management of recurrent ureteric strictures typically involves either:

1. Endoscopic management (balloon dilation, endoureterotomy)
2. Open surgical reconstruction (ureteroureterostomy, ureteral reimplantation)
3. Standard laparoscopic repair

The innovative aspect of this case was the simultaneous use of flexible ureteroscopy during the laparoscopic procedure, providing enhanced visualization and precision that neither technique alone could achieve.

Technical Advantages

This combined approach offers several significant benefits over conventional methods:

Enhanced Visualization: The flexible ureteroscope provides real-time endoluminal imaging during laparoscopic dissection, allowing for precise identification of the stricture’s proximal and distal extent. This dual visualization prevents inadvertent injury to healthy segments of the ureter.

Minimally Invasive: The laparoscopic approach minimizes surgical trauma compared to open surgery, while the addition of flexible ureteroscopy reduces the need for extensive ureteral mobilization and dissection.

Improved Technical Precision: The combined technique enables more accurate placement of sutures during reconstruction and immediate confirmation of ureteral patency following repair.

Reduced Morbidity: By minimizing dissection and handling of the ureter, this approach potentially reduces the risk of devascularization, which can lead to recurrent stricture formation.

Advancing Urological Care

“Recurrent ureteric strictures, particularly in the mid-ureter, present significant therapeutic challenges,” notes Dr. Hussain. “This novel approach represents a promising advancement in the management of complex strictures that have failed conventional treatment.”

The technique demonstrated at Lifecare Hospital merits further investigation and could potentially become a standard approach for managing similar cases, offering patients the benefits of minimally invasive surgery with enhanced precision and potentially improved long-term outcomes.

This case exemplifies the commitment to surgical innovation within the Burjeel Holdings network, where specialists continuously explore new approaches to address challenging clinical scenarios and improve patient outcomes.