Tibial hemimelia is a relatively rare congenital tibial longitudinal deficiency (approximately 1 per 1 million live births), unilateral or bilateral, with a relatively intact fibula. Hemimelia results from a disruption of the lower limb developmental field during embryogenesis due to slow or even abort of chondrification process, which results in leg length discrepancy. Affected leg commonly appears short and deformed with knee, ankle, and foot involvement. It may present with a variety of associated anomalies. Surgical treatment varies according to the type and degree of deformity, and reconstructive interventions are still limited. Reported cases of tibial hemimelia are very infrequent, especially tibial hemimelia in twins. Usually, the cases were in single embryo or less frequently in one of the monozygotic twins, but no reported cases regarding tibial hemimelia in one of the dizygotic twins as this article reports.
Malignant peripheral nerve sheath tumours (also called neurofibrosarcomas) are a rare, highly aggressive soft tissue sarcomas that arise from the peripheral nerves or cells associated with the nerve sheath, such as Schwann cells, peri-neural cells and fibroblasts. It is representing 10% of all soft tissue sarcomas in which it is considered as an extremely rare malignancy, especially in patients with neurofibromatosis type I. In the general population, it affects approximately 1 in 100,000 people. This article is reporting a 56-year-old Malay female patient who is a known case of neurofibromatosis type I for 20 years, presented with a lower back, pruritic, gradually increasing swelling during the last five months. Last month before the presentation, the lesion rapidly grows, reaching a size of (15×15 cm), accompanied by foul-smelling discharge and pain exacerbated with movement. Although no history of preceding trauma or accident, the mass bleeds within contact. In conclusion, only a few cases of giant malignant peripheral nerve sheath tumours reported in the literature describing its location and growth progression. We present a massive, extremely rapid growth of cutaneous exophytic malignant peripheral nerve sheath tumours over the lower back.
Extensive soft tissue injuries with exposed joints, bones, and tendons due to trauma of the hand -particularly fingers- necessitate soft tissue reconstruction and coverage. However, these injuries are challenging; therefore, various management options for traumatic skin loss over fingers are widely performed. These options comprise wound care permitting wound contracture without surgical intervention, full or split-thickness skin grafting, skeletal shortening and primary closure, and various types of flaps. We present a case of successfully placed Integra over the exposed phalangeal bones followed by split-thickness skin grafting a few weeks later, with a good outcome. We conclude that Integra is an alternative, safe and effective method for reconstructing severely traumatized fingers with exposed bone, tendon, or joint without sacrificing outcome.
Neurofibromatosis type 1 (NF1) is a complex autosomal dominant, multisystem genetic disease affecting about 1 in 3500 individuals. Plexiform neurofibromas represent a rare variant (30%) of NF1 in which the spread of tumor cells along nerve fascicles leads to a diffuse mass of thickened nerve fibers. Affected patients with NF1 have a greater chance of developing soft tissue sarcomas than the general population. Leiomyosarcoma is one of the most frequent soft tissue sarcomas, seldom observed in patients with NF1. Herein we report a rare concurrency of bone leiomyosarcoma in a patient with a plexiform neurofibroma, adding to the few reported cases of leiomyosarcomas in patients with NF1. Our case is a 14-year-old male who is a known case of NF1 and presented with a four-month history of pain and swelling on the medial side of the right knee. Imaging and biopsy confirmed the diagnosis of leiomyosarcoma. Based on the authors' knowledge and search, this is the first reported case of plexiform neurofibroma with a primary bone leiomyosarcoma, representing an extremely rare concurrency. Patients with such uncommon tumors should be assessed regularly, and continuous follow-up is essential.
Despite being the most common primary malignant bone tumor in children and adolescents, the presence of osteosarcoma at the wrist is infrequent; only less than 1% of osteosarcomas arise in the distal radius. The clinical presentation may mimic common musculoskeletal problems or benign lesions such as osteomyelitis, and a high index of suspicion is necessary so that the treating surgeon does not miss such lesions. We reported a case treated initially as osteomyelitis before being diagnosed as distal radius osteosarcoma. We conclude that an unusual location of osteosarcoma may be easily misdiagnosed, and therefore, osteosarcoma should be considered one of the main differential diagnosis in such cases until proven otherwise.
Introduction Modern treatments still aim to keep the impact of surgical intervention low and the outcome of surgeries as good as a surgeon can. Assessing the long-term nasolabial appearance of patients who underwent cleft lip (CL) repair surgery is one of the methods of evaluating the outcomes of cleft surgery. Methods This is a retrospective cross-sectional descriptive study of data records of unilateral CL patients. The data records of all patients who underwent unilateral CL repair by the reconstructive science unit at Hospital Universiti Sains Malaysia (HUSM) within the first two years of their lives and whose current age is 14 years or above were accessed and analyzed. Results The data records of 50 patients were analyzed, including 13 (26%) males and 37 (74%) females. The surgeons opined that 28% of the patients had an acceptable nasolabial appearance, while there were 10 (20%) patients whose nasolabial appearance was considered unacceptable by the reviewing surgeons. Fifteen (30%) patients were described as having an acceptable lip appearance with secondary nasal deformity, and 11 (22%) patients had an acceptable nasal appearance with secondary lip deformity. There were no surgical modifications or postoperative complications among the patients. None of our variables reported a significant association with long-term nasolabial appearance. Conclusion The long-term evaluation of the nasolabial appearance in individuals with CL following surgical correction significantly improves the service and care provided to patients to achieve optimum results. Although our results showed no relationship between gender, age at operation, type or diagnosis of cleft, and family history and long-term nasolabial appearance, frequent assessments will enhance surgical results.
Keloid scars are a relatively common condition but extremely rare in the penis. This case aims to be added to the previous 34 cases of penile keloid reported in the literature. We present the case of a 15-year-old patient with a high keloid-forming tendency who was successfully cured of penile keloid scarring with excision alone and without any adjuvant therapy. No recurrence was reported over eight years of follow-up. In contrast, at the same time, recurrence is frequently observed in other body parts after excision, making this particular part of the body an area of less keloid occurrence and recurrence.
Juvenile hyaline fibromatosis (JHF) is a rare, hereditary disease characterized by abnormal hyaline deposits within the skin, soft tissues, joints, and bones. The condition itself is often debilitating, with no curative treatment available. A definitive diagnosis is established by genetic testing. However, the hallmarks of gingival hypertrophy, subcutaneous scalp nodules, and joint contractures can be used as a clinical guide when genetic testing is unavailable. Here, we report an unusual case of a five-year-old child clinically diagnosed with juvenile hyaline fibromatosis with atypical nodules exclusively confined to the perioral region.
Breast implant surgery typically improves patient breast satisfaction and health-related quality of life. However, breast implants are also linked to long-term local problems like capsular contracture and breast discomfort. Chest pain is one of the reasons that patients with breast implants seek consultations, which is not typically attributable to cardiovascular reasons. The potential reasons for atypical chest pain are diverse. The absence of a precise diagnosis may also result in incorrect examinations and management, leading to further worry and wasted work time. A 55-year-old woman with a breast implant 10 years prior to the incident, presented with atypical chest pain on and off for a year and was treated as a case of unstable angina, costochondritis, and vasospastic spasm. Despite multiple visits, her symptoms did not resolve. Later, the patient presented with a lump over the left breast, associated with constitutional symptoms. Examination revealed a left breast implant with capsular contracture grade III, and ultrasonography showed signs of a ruptured implant. Symptoms eventually resolved after the removal of the breast implant.
Reconstruction of the scalp after acquired defects poses a great challenge to reconstructive surgeons. In oncologic resections, the defect must be covered with well-vascularized tissue to withstand radiotherapy post-surgery. However, due to the limited scalp tissue mobility, primary closure or loco-regional flaps are challenging and limited in choice. Fortunately, with the current understanding of the robust blood supply system to the scalp tissue, they can survive with the closure under tension. In this paper, we present a case of scalp reconstruction using a bi-pedicled visor flap to cover the two skull defects after ablative surgery. In addition, this article highlights the reason for the option, the surgical procedure, and the cosmetic outcome of the surgery.
Conjunctival melanoma is a rare and potentially deadly tumor. Therefore, adequate oncological resection is essential, commonly leading to total orbital exenteration, which causes patients' extensive functional and cosmetic impairment. As a result, it is essential to reconstruct the orbital region post-exenteration to obliterate the cavity, provide adequate and pliable cutaneous covering, and restore a stable vascularized tissue that can withstand adjuvant radiotherapy. In recent years, the techniques used for orbital reconstruction have included the transorbital temporoparietal fascial flap, the anterolateral thigh flap, and local flaps, such as the paramedian forehead flap. A free radial forearm flap is currently not commonly used for orbital reconstruction due to potential donor site morbidity and cosmetic issues. In our case, we report a free radial forearm fasciocutaneous flap that has been utilized with promising surgical outcomes to reconstruct the orbital region following orbital exenteration.