Reconstruction of Congenital Arhinia With Stereolithographic Modeling: Case Correlate and Literature Review.

Complete congenital arhinia is a rare defect of embryogenesis leading to the absence of the external nose and airway. We report our novel multistaged reconstructive approach and literature review. Nasal methyl methacrylate prosthesis was created from a stereolithographic model for use as a temporary prosthesis and tissue expander. Lefort 1 with cannulization was utilized for midface advancement and airway formation. External framework was reconstructed with bilateral conchal bowl cartilage and rib osteocartilagenous grafts. Patient was pleased with the aesthetics and had safe decannulation with the ability to breathe through the nose and airway.

Blindness Secondary to Orbital Giant Cell Granuloma Mass-Effect in Noonan Syndrome With Return of Vision Following Surgical Decompression.

ABSTRACT: Noonan syndrome is a rare, autosomal dominant disorder encompassing multiple congenital defects, as well as association with solid tumor and lesion development. The authors present a 26-year-old female with known Noonan syndrome and ongoing complaint of worsening unilateral vision, progressing to vision loss due to lesion mass effect. Decompressive surgery was performed, restoring patient's vision to baseline immediately postoperative. The lesion was confirmed to be giant cell granuloma. In this paper we discuss the unique presentation of vision loss due to orbital giant cell granuloma in Noonan syndrome with postoperative return of vision; the importance of a multi-disciplinary team evaluation, thorough preoperative clinical and image-based work up, intraoperative findings, postoperative outcome, and complexity of definitive management.

Vector control in internal midface distraction using temporary anchorage devices.

Le Fort III and monobloc distraction osteogenesis serve as the primary surgical treatment for children with severe midface hypoplasia. The orbital retrusion and class III malocclusion of patients with midface hypoplasia is best addressed with bodily advancement of the midface segment parallel to the cephalometric Frankfort horizontal plane. Use of internal distraction devices allows for advancement of the midface without extensive external hardware but comes at the cost of less vectorial control, resulting in a distraction vector that can cause a clockwise rotation of the entire midface or frontofacial component creating hollow appearing orbits. To counteract this clockwise rotation, we have developed a technique using orthodontic microimplants to anchor interarch class III relationship elastics. We report our experiences with this technique on a cadaveric model and as a case series of 17 patients who underwent midface distraction. A Le Fort III distraction procedure was carried out on a cadaver, and the orbital height was measured at 0-, 10-, and 20-mm distraction advancement with and without elastics in a class III relationship. Improvement of both subjective hollow appearance of the orbits and objective measurement of the orbital height with class III relationship elastics demonstrated the efficacy of class III relationship elastics in counteracting the clockwise rotation of the midface segment. A review of 17 patients with midface or frontofacial hypoplasia treated with Le Fort III or monobloc distraction with simultaneous microimplant anchored class III relationship elastics revealed correction of malocclusion and improved midface projection without significant increase in vertical height of the orbits.

Gigli saw facilitates safe minimal access sagittal suturectomy in infants.

The authors describe the use of the Gigli saw for craniectomy in minimal access surgery to address sagittal craniosynostosis. This modification allows for supine positioning and avoidance of potential brain compression with endoscopic instruments, and provides visually clear, safe, and facile removal of the fused suture and surrounding calvaria. The video can be found here: https://vimeo.com/511568750.

Preserving the Knee in the Setting of High Segmental Tibial Bone and Massive Soft-Tissue Loss Using Vascularized Distal Tibial Bone Docking and a Foot Fillet Flap: A Case Report.

CASE: We discuss our reconstructive approach to avoid an above-knee amputation in a 33-year-old man presenting after lower extremity crush injury. We used a vascularized tibial bone flap and a foot fillet flap to restore length and joint functionality to the residual limb. The patient ambulates with good prosthetic fit on durable heel pad skin and 100° active knee motion. CONCLUSION: This pairing of intramedullary nail with vascularized bone flap and fillet flap to address soft-tissue coverage and retain limb length is a useful tool in traumatic lower extremity injury management, providing an alternative technique for tibial bone graft stabilization with robust, sensate tissue coverage.

A malignant omental extra-gastrointestinal stromal tumor on a young man: a case report and review of the literature.

BACKGROUND: Gastrointestinal stromal tumors (GIST) are uncommon intra-abdominal tumors. These tumors tend to present with higher frequency in the stomach and small bowel. In fewer than 5% of cases, they originate primarily from the mesentery, omentum, or peritoneum. Furthermore, these extra-gastrointestinal tumors (EGIST) tend to be more common in patients greater than 50 years of age. Rarely do EGIST tumors present in those younger than 40 years of age. CASE PRESENTATION: We report a case of a large EGIST in a 27-year-old male. An abdominal pelvic computerized tomography imaging demonstrated an intra-abdominal mass of 22 cm, without invasion of adjacent viscera or liver lesions. This mass was resected en bloc with its fused omentum and an adherent portion of sigmoid colon. Pathology results demonstrated a malignant gastrointestinal stromal tumor with positive CD117 (c-kit) staining, and negative margins of resection, and no continuity of tumor with the sigmoid colon. Due to the malignant and aggressive nature of this patient's tumor, he was started on STI-571 as adjuvant chemotherapy. CONCLUSION: Stromal tumors of an extra-gastrointestinal origin are rare. Of the reported omental and mesenteric EGISTs in four published series, a total of 99 tumors were studied. Of the 99 patients in these series only 8 were under 40 years of age, none were younger than 30 years old; and only 5 were younger than 35 years old. Our patient's age is at the lower end of the age spectrum for the reported EGISTs. Young patients who present with an extra-gastrointestinal stromal tumor (EGIST), who have complete resection with negative margins, have a good prognosis. There is little data to support the role of STI-571 in adjuvant or neoadjuvant therapy after curative resection. Given the lack of data, the use of STI-571 must be individualized.