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Alveolar soft part sarcoma of the lateral rectus muscle: Suture technique to prevent postoperative strabismus
Faris M Al Ghulaiga1, Miyoung Kwon2, Ho-Seok Sa2
1College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Insights
Alveolar soft part sarcoma (ASPS) in the eye muscle is rare. A novel hang-back suture technique successfully resected the tumor and maintained eye alignment, preventing strabismus.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Surgical Oncology
Background:
- Alveolar soft part sarcoma (ASPS) is a rare, aggressive soft tissue sarcoma.
- Intramuscular ASPS of the extraocular muscles is exceptionally uncommon, particularly in pediatric patients.
- This case highlights the challenges in managing orbital tumors in children, including resistance to chemotherapy.
Observation:
- A 5-year-old boy presented with proptosis due to a rapidly growing intramuscular ASPS of the left lateral rectus (LR) muscle, unresponsive to chemotherapy.
- Advanced imaging confirmed a large, contrast-enhancing mass within the LR muscle without evidence of metastasis.
- The surgical approach involved en bloc resection of the tumor with the affected LR muscle segment.
Findings:
- A specialized surgical technique utilizing a 4-0 Prolene® hang-back suture was employed to preserve ocular alignment post-resection.
- Botulinum toxin injection into the antagonist medial rectus muscle aided in maintaining orthotropia.
- The patient achieved excellent functional and cosmetic outcomes with no recurrence or metastasis at 51 months follow-up.
Implications:
- Complete surgical resection of intramuscular ASPS is crucial for favorable outcomes.
- The described hang-back suture technique offers a viable reconstructive solution for maintaining ocular alignment after LR muscle tumor resection.
- This approach minimizes the risk of significant sequelae like strabismus and the need for more radical orbital surgery.
Purpose:
We present a rare case of intramuscular alveolar soft part sarcoma (ASPS) of the lateral rectus (LR) muscle and the surgical technique used to maintain orthotropia after complete resection of the mass.
Observations:
A 5-year-old boy presented with progressive proptosis of the left eye due to an orbital tumor. The patient was previously diagnosed with ASPS of the left LR muscle from an incisional biopsy, and the tumor size increased despite 5 cycles of chemotherapy prior to presenting to our center. Magnetic resonance imaging showed a 28x19x15mm-sized contrast-enhancing intramuscular mass of the left LR muscle, and there was no evidence of nodal or distant metastasis. The mass was excised en bloc, along with the insertion and the posterior normal part of LR muscle. To maintain proper eye alignment after resecting LR muscle, a 4-0 Prolene® hang-back suture was placed between the scleral insertion and the periorbita of the posterior orbit and the left medial rectus muscle was injected with botulinum toxin. During the follow-up of 51 months after surgery, the patient had no evidence of recurrence or metastasis and remained orthotropic in primary gaze, with a good cosmetic result.
Conclusions And Importance:
ASPS of extraocular muscles is a rare tumor occurring mainly in children and young adults, and treatment may cause significant sequelae such as orbital exenteration, radiation-induced complications, and large-angle strabismus. Complete resection of tumor including the extraocular muscle is essential for treatment, and a subsequent reconstruction using a hang-back suture technique is useful to achieve proper eye alignment as well as a good cosmetic outcome.
