By Omar Rashid, MD, JD VCU/MCV Department of Surgery CASE PRESENTATION By Omar Rashid, MD, JD VCU/MCV Department of Surgery

42yr female h/o HTN, protein S def, TIAs x 5, on the OBGYN service w Abd/Pelvis pain for which she underwent an MRI. 4.7cm x 4cm x 3.5cm complex collection

INTERVAL Hx EUA Path REPEAT IMAGING RESECTION

Cephalad

Cephalad

Cephalad RECTUM Coccyx

Anatomy

Epidemiology Rare 1 – 6 annually diagnosed at major referral centers Dunn, 2010 Incidence estimated at 1:40,000 patients in major referral center. Whittaker and Pemberton, 1938 50 cases at Cleveland clinic over 50 year period. Grundfest-Broniatowski et al., 1990 39 malignant tumors seen at Memorial-Sloan Kettering over 28 years. Cody et al., 1981

Presentation Usually incidental finding on pelvic/rectal examination in asymptomatic patients. Pain: more common in malignant lesions Jao et al., 1985 Recurrent infection; repeated operations for “perirectal abscess/fistula in ano”. Singer et al., 2003 Rectal complaints: sensation of rectal fullness, incomplete evacuation, constipation. Headache with straining and during intercourse in women with meningocele. Urinary dysfunction

Diagnosis Physical Exam: Inspection: Postanal dimple suggestive of developmental cyst communicating with the skin. Laxity of sphincter indicates sacral nerve involvement. Digital rectal examination: mass displacing rectum anteriorly; smooth intact mucosa. Sigmoidoscopy: Typically normal, edema suggestive of infected cyst.

Imaging Plain film: “scimitar” sacrum indicates presence of anterior meningocele; bony destruction indicates malignant process.

Imaging CT scan: Useful in identifying small tumors, distinguishing solid and cystic lesions, and assessing for invasion of adjacent structures.

Imaging MRI: More detailed than CT, useful in delineating nerve/bone involvement in pre-operative planning.

Imaging Endorectal ultrasound: May demonstrate rectal wall invasion and differentiate solid from cystic lesions.

To Biopsy or Not…??? Biopsy of presacral lesions controversial: Advantages Tissue diagnosis Disadvantages Fistula, Abscess, Tumor Seeding, Meningitis Always posterior (not transrectal)

Surgical Indication Resection recommended for all retrorectal tumors: Symptoms worsen with increasing size of lesion. Cystic lesions may become infected, making surgical management difficult. Solid lesions have high malignant potential. Untreated meningocele has high mortality secondary to infection.

Operative Strategies Operative approach dictated by size and location of lesion: Abdominal Posterior Anteroposterior

If proximal extent of tumor can be felt on digital exam, posterior approach feasible. If ½ of tumor can be palpated, posterior approach may be possible. If <1/2 of tumor palpated, anterior or combined approach recommended.

Anterior Approach Abdomen entered through midline incision. Sigmoid colon mobilized and retrorectal space entered anterior to parasympathetics. Ligation of middle sacral vessels. Tumor dissected free of surrounding structures. Rectal involvement may necessitate proctectomy.

Sacral involvement may necessitate sacrectomy through combined anterior/posterior approach. Posterior approach allows for better visualization of sacral nerve roots. Distal sacral vertebrae and nerve roots can be sacrificed bilaterally without significant functional impairment. S3 nerve root must be preserved unilaterally to preserve continence.

Posterior Approach Incision may be paracooccygeal, midline, or transverse depending on surgeon preference. Anococcygeal ligament and pubococcygeus divided and coccyx disarticulated. Cyst dissected free of surrounding structures. En bloc resection with coccyx reduces recurrence rate.

Questions???