Long-term follow-up and pregnancy after complete sacrectomy with lumbopelvic reconstruction: case report and literature review

Our patient was a 20-year-old woman with a history of a sacral mass diagnosed by CT-guided needle biopsy 7 years before as benign fibrohistiocytoma. A repeat biopsy of her lesion through an open approach revealed myofibroblastic sarcoma. Consideration was given for reduction of tumor burden and lumbopelvic reconstruction to facilitate long-term progression-free survival and to improve quality of life, respectively.

The extension of tumor into both sacroiliac joints prohibited sparing S1; thus, a two-staged total sacrectomy was planned, as previously described. Briefly, the first stage of the procedure included a midline laparotomy, mobilization of the visceral and neural structures, and ligation of the internal iliac vessels. A colostomy was performed, and a right vertical rectus abdominus myocutaneous flap based on the inferior epigastric vessels was mobilized, wrapped in a bowel bag, and placed in the pelvis. The second stage was performed the next day, and it included L5 and S1 laminectomies, bilateral osteotomies and disarticulation of the sacrum from the ilium at the sacroiliac joints, ligation of the thecal sac inferior to the takeoff of the L5 nerve roots, complete L5–S1 discectomy, and transection of the S1–S5 nerve roots. The entire sacrum, along with the tumor, was removed piecemeal. This was followed by lumbopelvic reconstruction with spinal instrumentation and bone graft.

At 5 years after surgery, the patient remains pain-free. She was able to ambulate independently with a right ankle-foot orthosis. There was no evidence of locally progressive or metastatic disease on follow-up imaging. Postoperative imaging showed settling and a stable fibrous pseudoarthrosis (Fig. 1a and b).

The patient’s first pregnancy was unbeknownst to us during a period of follow-up loss after sacrectomy. However, she gave birth to a healthy 5-lb, 2-oz baby girl through a cesarean delivery at 37 weeks’ gestation by a community obstetrician. There were no birth complications. The baby, now 3½ years old, is meeting developmental milestones.

The Baylor Maternal-Fetal Medicine Service at Texas Children’s Pavilion for Women followed her second pregnancy closely. Frequent visits every 2 to 3 weeks were scheduled to monitor maternal status (e.g., weight gain and blood pressure). The pregnancy was carefully documented with monthly fetal ultrasound and MRI (Figs. 2a, b, and 3). At each outpatient visit, catheterization was performed to send urine culture, and the patient was on Keflex suppression throughout the pregnancy. Routine prenatal labs were obtained, and routine vaccines were administered. A repeat elective cesarean was performed at 37 weeks of gestation. Great care was taken to identify the right inferior epigastric vessels, the pedicle of the rectus abdominis musculocutaneous flap used for closure of the sacral defect (Figs. 4 and 5). Plastic surgery was available at the time of delivery, but the right rectus abdominis muscle flap was not seen and was assumed to be posterior to the uterus. A healthy baby girl was delivered (Fig. 6). The birth weight was recorded as 6 lb and 9 oz. Apgar scores of 8 and 9 were assigned at 1 and 5 min, respectively.

Table 1 summarizes the patient history in timeline format.

Prior to the patient’s second pregnancy, we collected standardized and validated indices data to quantitate Health-Related Quality of Life (HRQoL) outcomes through the Oswestry Disability Index (ODI) and MOS Short Form 36 (SF-36).

Our patient’s ODI score was 30 %, representing mild disability.

SF-36 Physical Component Summary for our patient was calculated as 28.6 (national average, 53.5), and SF-36 Mental Component Summary was 52.9 (national average, 46.19). Our patient’s scores compared favorably in the domains of social functioning and mental health but were below the population norms across the remaining scales of physical functioning.

The treatment of primary sacral tumors represents a challenge because of their anatomical location and often large size at presentation. Instability at the lumbopelvic junction frequently accounts for the majority of the symptoms, such as axial back pain, that these patients, like ours, experience. Reconstruction of the lumbopelvic junction succeeds in stabilizing the spine from a biomechanical standpoint, which improves patient functionality. There have been no other reports archiving pregnancy after sacrectomy.

Women with neurological impairment may be insensate to the onset of labor. Instructions on techniques of self-uterine palpation to detect contractions at home and serial assessment of cervical length with ultrasonography and home uterine activity monitoring may constitute additional measures to prevent unexpected spontaneous prematurity or undiagnosed labor.

Postpartum depression has been reported to be higher in women with neurological deficits than in the general pregnant population. Correlates of depression include higher stress level, greater social isolation and lower satisfaction with social network, less mobility, unemployment, abuse, and poorer overall health.

We treated a 15-year-old female patient who underwent a total sacrectomy for gross total resection (GTR) of a sacral myofibroblastic sarcoma with extension into the iliac wings and, herein, have described long-term (5-year) functional follow-up. Pregnancy after sacrectomy has not been previously reported in the literature. During the follow-up period, our patient carried two babies to term and delivered via cesarean with no perinatal complications. Obstetric challenges and considerations for our patient with neurological deficits in lower extremity, impaired mobility, and bladder and bowel dysfunction include the following: prophylaxis for urinary tract infections, DVT prevention, precautions against pressure ulcers, maintenance of functional independence, and preservation of the blood supply (i.e., the inferior epigastric artery) to the myocutaneous flap used during the pelvic reconstruction when planning a cesarean. Long-term progression-free survival with satisfactory HRQoL outcomes seems to have been attained.

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review.