Hamda Al Zarooni, MD, Shadi Al Bahri, MD, Guido Mannaerts, MD, PhD. Tawam Hospital Johns Hopkins International
Introduction: Kartagener syndrome is a congenital, autosomal recessive disorder that affects ciliary motility. It presents as a triad of situs inversus, bronchiectasis and sinusitis, with a prevalence of 1 in 12,500 to 1 in 50,000. Situs inversus, is an anatomical abnormality with preserved organs physiological function, where the visceral organs are mirrored from their normal position.
Case Presentation: 64 year old male known to have Kartagener syndrome with situs inversus totalis variant. His comorbidities include atrial fibrillation, dyslipidemia, hypertension, metallic aortic valve replacement and coronary artery stenting. He presented with rectal bleeding and was diagnosed with sigmoid colon adenocarcinoma. Preoperative planning included colonoscopy with tattooing of the tumor site, CT angiography of the abdomen and pelvis to delineate vascular and anatomical variations. Patient was classified as ASA 3 with moderate cardiac risk. Important operative steps included placing the patient in lithotomy position, with the surgeon standing to the left and the assistant to the right of the patient. Four trocars were used; supra-umbilical 12mm camera port, LLQ 12mm and left flank 5mm working ports, and right flank 5mm assistant port. Endoscopic devices including ultrasonic dissection, Endoclips and linear stapler used during procedure were introduced through the LLQ port. After mobilization and transection of the sigmoid colon, the specimen was extracted through a Pfannensteil incision. End to end colonic anastomosis done using endoscopic circular stapler with reinforced interrupted absorbable sutures. Postoperative course was unremarkable except for an abdominal wall hematoma due to anticoagulation. He was seen in clinic following discharge and pathology revealed negative lymph node involvement.
Discussion: Kartagener syndrome, includes the triad of situs inversus, bronchiectasis and sinusitis. According to our literature review, 13 cases were reported of laparoscopic colonic resection in patients with situs inversus alone. Taking into consideration that patients with Kartagener syndrome have associated comorbidities that can increase patient morbidity and mortality. Appropriate preoperative evaluation and multidisciplinary involvement, in addition to several technical intraoperative considerations can make laparoscopic sigmoid resection feasible. Points to consider include optimization of patient’s comorbidities, preoperative CT angiography and colonoscopy with tumor tattooing. In addition, intraoperative port placement, surgeon assistant position and the presence of an experienced laparoscopic surgeon allow for a smooth operative course.
Conclusion: Laparoscopic sigmoid colectomy can be challenging in patients with Kartagener syndrome due to mirror imaged anatomy and associated comorbidities. Advances in laparoscopic methods now allow patients with rare anatomic variations to benefit from laparoscopic surgery and its associated advantages.
Presented at the SAGES 2017 Annual Meeting in Houston, TX.
Abstract ID: 93647
Program Number: P318
Presentation Session: Poster Session (Non CME)
Presentation Type: Poster