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Desmoid tumors of the trunk and extremity.

BACKGROUND: Twenty-six patients with desmoid tumors of the extremities (n = 20) or trunk (n = 6) have been treated since 1977. Nine of these were referred with primary tumors and 17 with recurrent tumors. METHODS: The tumor was removed in all patients, and 10 received adjuvant radiation. Five patients (19%) developed local recurrence; one of them was lost to follow-up, and the remaining four patients had the recurrence controlled with surgical resection or resection plus adjuvant radiation. RESULTS: All 25 patients who have been followed are alive and disease-free at a mean follow-up of 84 months (median, 81 months). None with extremity tumors (n = 20) required an amputation. The estimated survival rate for the entire group is 95% at 42 months. CONCLUSIONS: Desmoid tumor of the trunk or extremities treated with surgical resection, supplemented selectively by adjuvant radiation, can be controlled locally in the majority (96%) of patients.

Abdominal Neoplasms↗

Preoperative regional therapy for extremity sarcoma. A tricenter update.

BACKGROUND: Preoperative radiation with sensitizing doxorubicin has been popularized in the treatment of high grade sarcoma of the extremity. A multiinstitutional experience with this protocol that was initiated by the Southeastern Cancer Study Group in 1984 and maintained in three institutions was updated by the authors. METHODS: Patients with biopsied sarcoma had intraarterial infusion with doxorubicin hydrochloride (Adriamycin) 30 mg/24 hrs x 3 days and were allocated by the institution to receive radiation 30-35 Gy in 10 fractions or 46 Gy in 23-25 fractions followed by resection. Surgery was performed within 10 days or 30 days depending on the radiation dose. Postoperative chemotherapy was administered to 31 patients. RESULTS: Of 66 patients, 32 were female and 34 were male. The median age was 46 years (range, 14-77 years); 73% had lower and 27% had upper extremity tumors. There were 55 soft-tissue and 9 malignant bone tumors. Common types were malignant fibrous histiocytoma 20%; liposarcoma, 17%; synovial sarcoma 18%; and 14% were osteosarcoma. American Joint Committee on Cancer stages were: I, IIB (17%), IIIA/B (59%), and IIIC/4A (24%). Limb salvage surgery was performed on 60 patients including radical resection in 21 with extensive tumors, wide local excision in 30, and limited excision in 2 patients. Primary amputation was performed on four patients and delayed amputation in two because of wound complications. Three patients had pulmonary metastasectomy in conjunction with primary surgery (2 are long term survivors > 5 years). There were no postoperative deaths, but wound complications occurred in 41% of the patients. Overall survival and disease free survival at 5 years was 59 and 49%. One patient (1.5%) had local recurrence 9 years after resection of an extensive synovial sarcoma of the shoulder. This was resected with limb salvage techniques. Among 23 patients who failed primarily in the lung, the median survival was 7 months with 4 surviving more than 5 years, after demonstration of the pulmonary metastases. Multivariant analysis of prognostic factors showed that extent of disease and stage correlated with disease free survival, whereas only extent of surgical resection correlated with overall surgical survival. CONCLUSION: Combined therapy for extremity sarcoma in a multicenter setting using preoperative radiation with sensitizing chemotherapy and adequate resection was associated with an excellent local control rate (98.5%) and reasonable long term tumor control, although distant metastases continued to be a major challenge.

Adolescent↗

Ergonomic and physiotherapeutic interventions for treating upper extremity work related disorders in adults.

BACKGROUND: Conservative interventions such as physiotherapy and ergonomic adjustments play a major part in the treatment of most work-related musculoskeletal disorders (WRMD). OBJECTIVES: The objective of this systematic review is to determine whether conservative interventions have a significant impact on short and long-term outcomes for upper extremity WRMD in adults. SEARCH STRATEGY: We searched the Cochrane Musculoskeletal Injuries Group specialised register (January 2002) and Cochrane Rehabilitation and Related Therapies Field specialised register (January 2002), the Cochrane Controlled Trials Register (The Cochrane Library Issue 3, 2001), PubMed (1966 to November 2001), EMBASE (1988 to November 2001), and CINAHL (1982 to November 2001). We also searched the Physiotherapy Index (1988 to November 2001) and reference lists of articles. No language restrictions were applied. SELECTION CRITERIA: Only randomised controlled trials and concurrent controlled trials studying conservative interventions for adults suffering from upper extremity WRMD were included. Conservative interventions may include exercises, relaxation, physical applications, biofeedback, myofeedback and work place adjustments. DATA COLLECTION AND ANALYSIS: Two reviewers independently selected the trials from the search yield and assessed the clinical relevance and methodological quality using the Delphi list. In the event of clinical heterogeneity or lack of data we used a rating system to assess levels of evidence. MAIN RESULTS: We included 15 trials involving 925 people. Twelve trials included people with chronic non-specific neck or shoulder complaints, or non-specific upper extremity disorders. Over 20 interventions were evaluated; seven main subgroups of interventions could be determined: exercises, manual therapy, massage, ergonomics, multidisciplinary treatment, energised splint and individual treatment versus group therapy. Overall, the quality of the studies appeared to be poor. In 10 studies a form of exercise was evaluated, and there is limited evidence about the effectiveness of exercises only when compared to no treatment. Concerning manual therapy (1 study), massage (4 studies), multidisciplinary treatment (1 study) and energised splint (1 study) no conclusions can be drawn. Limited evidence is found concerning the effectiveness of specific keyboards for patients with carpal tunnel syndrome. REVIEWER'S CONCLUSIONS: This review shows limited evidence for the effectiveness of keyboards with an alternative force-displacement of the keys or an alternative geometry, and limited evidence for the effectiveness of individual exercises. The benefit of expensive ergonomic interventions (such as new chairs, new desks etc) in the workplace is not clearly demonstrated.

Adult↗

Localized relapse in bone marrow of extremities after allogeneic stem cell transplantation for acute lymphoblastic leukemia.

We report a patient with a relapsed in bone marrow of extremities after allogeneic peripheral blood stem cell transplantation for acute lymphoblastic leukemia (ALL). The patient complained of pain in the right upper arm and left leg 15 months after transplantation. Magnetic resonance imaging (MRI) and fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET) showed abnormal findings in bone marrow of upper and lower extremities. There were no findings of relapse in aspirates from the sternum and iliac bone marrow. Biopsy specimen from the iliac bone marrow showed normocellular marrow without leukemic cells. Biopsy specimen from the right humerus revealed marked leukemic cell infiltration in the bone marrow. This is apparently the first case of localized relapse of ALL in bone marrow of extremities. Physicians should be aware of unusual relapse sites of leukemia after allogeneic stem cell transplantation. MRI and FDG-PET may be of value in detecting this type of relapse.

Adult↗

Upper extremity pain and computer use among engineering graduate students.

BACKGROUND: The objective of this study was to investigate risk factors associated with persistent or recurrent upper extremity and neck pain among engineering graduate students. METHODS: A random sample of 206 Electrical Engineering and Computer Science (EECS) graduate students at a large public university completed an online questionnaire. RESULTS: Approximately 60% of respondents reported upper extremity or neck pain attributed to computer use and reported a mean pain severity score of 4.5 (+/-2.2; scale 0-10). In a final logistic regression model, female gender, years of computer use, and hours of computer use per week were significantly associated with pain. CONCLUSIONS: The high prevalence of upper extremity pain reported by graduate students suggests a public health need to identify interventions that will reduce symptom severity and prevent impairment.

Adult↗

Mechanical contribution of the fibula to torsion stiffness in the lower extremity.

The role of the fibula in rotational stability of the lower extremity, taking into account the intact knee joint and tibia, to the best of our knowledge, has not been investigated. A cadaver study was designed to determine the torque transfer down the lower extremity, with and without the fibula. Six fresh, frozen human cadaver legs were used. The knee joint was left intact and the foot disarticulated. An external rotation up to 5 degrees , coupled with axial compression, was applied to the femur passing through the knee joint in extension and down the lower extremity to the fixed distal end, where torque (N m) was measured via a load cell. Each specimen was further tested with the fibula, cut 4 cm from the distal end, and finally with the entire fibula disarticulated at the proximal end and removed. To achieve 5 degrees rotation, torques reached 1.82 N m (SD, 0.66 N m). When the fibula was cut, the torque reduced a small yet significant amount of 5%. With the entire fibula removed, the torque reduction was significant and more pronounced at 11%. The fibula provided a small yet significant role in torsion stability. From this study, where the biomechanical characteristics of the fibula is explicated, it is hoped that this information will have use in further understanding the biomechanical role of the fibula, especially, in relation to the altered mechanics associated with lower limb pathology involving a deficient fibula.

Aged↗

Soft tissue sarcoma of the upper extremity: a 5-year experience at two institutions emphasizing the role of soft tissue flap reconstruction.

BACKGROUND: The objective of this study was to define the impact of soft tissue flap reconstruction on multimodality therapy for patients with soft tissue sarcomas of the upper extremity. Treatment standards continue to evolve for these patients, and, with multimodality therapy, most of them are candidates for limb-preserving surgery. Consequently, the role of soft tissue flap reconstruction is expanding. METHODS: A review was conducted of 100 consecutive patients with soft tissue sarcomas of the upper extremity who underwent surgery at several institutions between 1992 and 1997. RESULTS: Seventy-one patients underwent direct closure of defects after tumor resection, and 29 patients required soft tissue reconstruction with flaps. These groups were similar in most respects, except that 52% of the patients who required soft tissue reconstruction presented with recurrent disease (P = 0.0004), and 79% of them had tumors measuring > 5 cm in greatest dimension (P = 0.0003). The patients who required flap reconstruction had larger skin deficits after undergoing tumor resection (140 cm2) compared with the patients who had wounds that were managed by direct closure (40 cm2; P < 0.00001). Margins around the resected tumors were larger (1.62 cm) when flaps were employed compared with margins when defects were closed directly (0.87 cm; P = 0.0005). However, the number of patients with intralesional, marginal, wide, and radical resections was the same regardless of wound management. Major complications occurred in 14% of patients, but none led to death or amputation. The median follow-up was 31 months, and 66% of patients had no evidence of disease at that time. Rates of local recurrence and survival were similar for patients who underwent flap reconstruction compared with patients who underwent direct closure. CONCLUSIONS: Soft tissue flap reconstruction facilitates therapy for patients with soft tissue sarcomas of the upper extremity, so that patients with larger tumors can undergo resection, limiting complications and limb sacrifice.

Adolescent↗

Rhabdomyosarcoma of the extremities and trunk: analysis of 150 patients treated by surgical resection.

This report details the clinical experience of 150 patients with rhabdomyosarcomas treated by one group of surgeons. Of the 150 patients, 103 suffered from pleomorphic rhabdomyosarcomas, 40 patients had embryonal rhabdomyosarcomas, and 7 had alveolar cell type of rhabdomyosarcoma. The males suffered almost twice as much as the females from this form of cancer (96 males to 54 females). The fifth decade had the highest incidence of all rhabdomyosarcomas. The younger patients generally suffered from embryonal rhabdomyosarcomas and the older ones from pleomorphic rhabdomyosarcomas, although age was no barrier to the occurrence of embryonal rhabdomyosarcomas in some of the older patients and pleomorphic rhabdomyosarcomas in some of the younger patients. Caucasians were almost exclusively afflicted by this form of cancer (93% of this series). The lower extremity was the most frequent site of the lesion, occurring in 50.7% of the 150 cases. The upper extremity was the second most frequently involved site (28%), and the trunk was the least involved site (21.3%). Of the 150 patients studied, 40 were considered indeterminate. Thirteen patients are alive and free of cancer, but were treated less than 5 years ago; 12 patients reported for consultation only. There were 13 who were free of sarcomas at periods in excess of 2 years but were lost to follow-up. Two patients in this series died before the 5-year period, and autopsy revealed the death to be due to causes other than the rhabdomyosarcoma. Of the 110 determinate patients, 75 (68%) died as a result of the cancer. The 5-year survival rate equals 32%. The fact of the 30% 5-year survival after treatment for recurrences should help dismiss the pessimism which prevails once a recurrence is noted subsequent to major therapy. Age per se had no great effect on prognosis. The poorest results occurred in the 11-20 age range, with a 5-year survival of 15%. This value reflects on increased incidence of embryonal rhabdomyosarcomas which tend to be more malignant and metastasize earlier than do the pleomorphic type. The effect of location upon survival revealed that of 76 patients with involvement of the lower extremity, 34% survived 5 years or longer, and those with sarcomas of the trunk had the poorest survival (26%). The fact that the trunk, by virtue of its anatomy, limits the degree of surgery that can be perfomred, probably helps to explain this difference. The role of radiation therapy and chemotherapy awaits elucidation. Reports of combined surgery, radiation therapy, and chemotherapy are encouraging.

Adolescent↗

Leiomyosarcomas of the extremities: angiography as a diagnostic aid.

Thirteen cases of leiomyosarcomas of the extremities are reported. Most of the cases were operated on without information concerning benignancy or malignancy. Plain radiographs showed amorphous calcification in two cases. Three cases had angiographic study and all revealed malignant neovascularity. In no literature that we reviewed was angiography mentioned in the cases of leiomyosarcomas of the extremities. Venography alone has been done in many reported cases. The leiomyosarcoma of the extremities looks often benign but is notorious for local recurrences and distant metastases.

Adult↗

[Stroma-free hemoglobin solution and perfusion of isolated extremities].

Pyridoxylated stroma-free hemoglobin solution was compared to hemodiluted blood in extremity perfusion of mongrel dogs. pH-values in tissue and in perfusate as well as oxygen tension showed no significant difference, and oxygen supply to the periphery was sufficient. Measurement of lactate, pyruvate, ATP and CP demonstrated domination of aerobic metabolism. We proved that stroma-free hemoglobin solution ( SFHb -PLP) can substitute hemodiluted blood (Hk 18) in isolated extremity perfusion. Metabolic results were confirmed by histological examination where no tissue alterations could be found in the perfused extremity or the whole body system.

Adenosine Triphosphate↗

Magnetic resonance venography of congenital vascular malformations of the extremities.

Contrast angiography can demonstrate the vascular components of a vascular malformation, but can be technically challenging in small patients with complex venous anomalies. We reviewed the role of magnetic resonance venography (MRV) in the evaluation of children with predominantly low-flow, vascular malformations of the extremities. MRV (2D time-of-flight technique) and magnetic resonance (MR) imaging examinations were performed in ten young patients with congenital predominantly low-flow vascular malformations of the extremities. MR imaging was used to characterize and determine the extent of the malformations, and MRV to evaluate the deep and superficial venous channels. In all patients, MRV studies were reviewed in conjunction with contrast angiograms, considered the gold standard, to confirm the findings. All significant channel anomalies seen with contrast angiography were identified with MRV. In addition, MRV demonstrated some veins that were not intentionally opacified during contrast studies. MRV demonstrates both the superficial and deep conducting veins, whereas contrast angiography is a more directed study, evaluating only those channels intentionally opacified. Together, MR imaging and MRV data can non-invasively form the basis for determining the prognosis and choosing the individual treatment of congenital vascular malformations of the extremities.

Adolescent↗

[A modified dye dilution method to estimate leakage during regional isolated perfusion of the extremity (author's transl)].

In experiments on dogs (n=13) we tested a procedure for estimating leakage during regional perfusion of the extremity by means of a dye dilution method. After systematic application of 0.5% Evans blue solution (0.1 ml/kg b.w.) we measured the dye concentration in plasma by means of a spectral photometer. The plasma volume (5.5 ml/100 gb.w.) and the disappearance rate of the dye (10%/h) were calculated. In a second procedure the supplying artery and vein of the extremity were proximally clamped and distally connected to an extracorporeal circulation unit consisting of oxygenator, pump, and heat exchanger, and the isolation of the extremity was tested. By simulated of leakage it was possible to detect a little amount of shunt of about 1% escaping from isolated region into the systemic circulation. There were no hints to toxicity when the same dye concentration. There were no hints to toxicity when the same dye concentration was applied. The standardized method was using during 132 cytostatic hyperthermic perfusion in man. In 20 patients we determined shunts of less than 5%, in 104 patients shunts between 5 and 10%, and in eight patients shunts of 10-20% of the extracorporeal circulation. The benefits of the described method are simplicity to carry out and missing of toxicity or radiation.

Animals↗

Survival in primary soft tissue sarcoma of the extremities and trunk.

BACKGROUND: Soft tissue sarcomas (STS) of the extremities are rare. The purpose of this study was to identify prognostic risk factors associated with survival in patients with primary extremity and truncal STS. METHODS: Patient, tumor, and pathologic data from 149 consecutive patients with localized primary STS of the extremities and trunk were analyzed using Kaplan-Meier and Cox regression techniques to identify univariate and multivariate risk factors. A subgroup analysis was performed to compare factors predictive of survival in patients who received treatment before (n = 50) and after (n = 99) treatment was standardized in 1988. RESULTS: The 5-year survival rate was 76.5% with an average follow-up of 6 years. Local recurrence occurred in 23% of all patients, 40% before 1988 and 15% after 1988 (P < 0.0001). Risk factors associated with survival included resection quality (R0 vs. R1; P < 0.0001), era of operation (P = 0.002), local recurrence (P < 0.001), UICC stage (P < 0.0001), tumor size (P < 0.001), tumor depth (P = 0.002), regional lymph nodes (P < 0.0001), and histology (P < 0.0001). Multivariate analysis revealed that tumor size, tumor depth, and resection quality were independent risk factors of survival. CONCLUSIONS: These results indicate that management of STS in a specialized institution improves overall survival. Resection quality is the most important risk factor of survival. Therefore, effort should be made during primary treatment of STS to achieve wide, tumor-free resection margins.

Adolescent↗

[Multimodal interdisciplinary therapy to improve function and long-term survival in patients with high-grade osteosarcoma of the extremities].

INTRODUCTION: The probability for survival of patients with highly malignant osteosarcoma of the extremities was essentially improved by (neo-)adjuvant chemotherapy. The goal was to further improve survival rates by introducing operative treatment of metastases. In the last 20 years, the percentage of limb-preserving operations has increased. PATIENTS AND METHODS: A consecutive series of 23 patients with localized highly malignant osteosarcoma of the extremities received (neo-) adjuvant chemotherapy according to the Cooperative Osteosarcoma Study (COSS) protocol. Local treatment was performed by wide (22 patients) or radical (1 patient) resection (17 limb salvage procedures, 5 amputations, and 1 rotationplasty). In four of seven patients who developed pulmonary metastases, the metastases (up to four) were resected. The patients did not receive salvage chemotherapy. RESULTS: The cumulative survival was 87% at 174 months. The four patients who underwent metastasectomy survived between 42 and 116 months without evidence of disease. One local recurrence developed outside the operative field and could be resected without influencing the functional or oncological outcome. After limb salvage procedures, the mean score according to the Musculoskeletal Tumor Society (MSTS) was 83%, and after rotationplasty 67%. CONCLUSIONS: In patients with osteosarcoma of the extremities, limb salvage is possible in a high percentage of cases with a low risk of local recurrence by an effective interdisciplinary cooperation between diagnostic radiologists, oncologists, and orthopedic surgeons. The midterm survival could be improved by the multimodal therapy from about 20% up to about 80%. Metastasectomy gives patients with a limited number of pulmonary metastases a realistic chance to survive.

Adolescent↗

[Synovial sarcoma of the extremities. Results of surgical and multimodal therapy].

BACKGROUND: Synovial sarcoma is a rare tumor frequently occurring in adolescents and young adults. The preferred location, at the distal parts of the extremities, and the high incidence of distant metastases represent major challenges for treatment. The purpose of this study is to analyze the indications for surgery in the context of a multimodal therapy. PATIENTS AND METHODS: Between October 1992 and December 2000, 41 patients were treated surgically for synovial sarcoma of the extremities. Their mean age was 35 years (range: 11-75 years). Extracompartmental tumor growth was present in more than 90% of the patients. Fourteen patients (34%) presented with a tumor size of less than 5 cm (T1) while 27 patients (66%) showed lesions greater than 5 cm (T2). At the time of presentation, seven patients had distant metastases; three located in the lung and four in lymph nodes. RESULTS: Due to a locally unresectable tumor or synchronous distant metastases, 28 patients (68%) underwent preoperative systemic chemotherapy (n=9) or isolated limb perfusion (n=18) or both (n=1). In 29 patients, limb-conserving surgery was possible. Of the 12 patients (29%) who required amputation, 85% had a distal tumor. Sixteen patients received additional postoperative radio- and/or chemotherapy. After a median follow-up of 30 months, only two patients (7%) had developed a local recurrence, while seven patients (20%) suffered from new, distant metastases. The 5-year overall survival rate was 82%. CONCLUSION: With a combined modality treatment, it is possible to achieve excellent local control rates and long-term survival in patients with synovial sarcoma of the extremities. Neoadjuvant treatment can enable limb-sparing surgery in most patients, even if the tumors are located in anatomically difficult areas.

Adolescent↗

[Reconstruction of burned extremities by free flap transplantation].

Free tissue transplantation in burn reconstruction presents a major challenge to reconstructive surgeons. The results of a retrospective analysis of 68 free flaps in 55 patients are reported. This experience facilitated the establishment of reconstructive principles and a decision-making algorithm for primary and secondary reconstruction of burned extremities. Fourty-two free flaps were used for primary reconstruction. The indications were predominantly extremity salvage.The safety of the microsurgical procedures is correlated with the timing of the reconstruction. The failure rate of the free flaps was 24% in primary reconstruction. Due to an increased post-traumatic thrombogenicity, the period between 5 and 21 days had the highest risk of flap failure (40%).Twenty-six flaps were used for secondary reconstruction, with a success rate of 100%. Due to their elasticity, adipo- and fasciocutaneous flaps provide a useful option for the release of contractures. The large variability demonstrated by the use of 19 different types of free flaps showed that the reconstruction of burned extremities requires a reconstructive concept individualized to each patient as well as sophisticated microsurgical techniques. This clearly demonstrates the importance of a close link between primary burn treatment and reconstructive plastic surgery.

Adolescent↗

[Management strategies in the first operative phase after long-bone injury of the lower extremity in multiple-injured patients. A systematic literature review].

OBJECTIVE: Lower extremity injuries make up a substantial proportion of the injuries in multiply injured patients. The aim of this systematic literature analysis was to give an overview of the levels of evidence for different management strategies in the first operative phase after long-bone injuries of the lower extremity in multiply injured patients to enable, in the presence of adequate evidence, the development of clinical management corridors or, if the evidence was found to be inadequate, to document the necessity for scientific proof. METHODS: Clinical trials were systematically collected (Medline, Cochrane and hand searches) and classified into evidence levels (EL 1 to 5 according to the Oxford system). RESULTS: The necessity for primary or secondary definitive osteosynthesis of femur/tibia shaft fractures is still a matter of discussion. Intramedullary nailing is the preferred operative procedure for definitive treatment of femur shaft fractures. Stabilization of proximal and distal femur and tibia fractures is predominantly based on expert opinion. According to the literature, perioperative antibiotic prophylaxis is essential in fracture treatment. CONCLUSION: Numerous comparative studies (EL 2) dealing with management strategies in the first operative phase after long-bone injuries of the lower extremity in multiply injured patients are available, but there are only a few randomized studies. Based on the available data, it is possible to develop a rational therapy for this patient population.

Clinical Trials as Topic↗

[Inner amputation of an upper extremity with impaired cerebral perfusion and lethal outcome].

An inner amputation of the upper extremity is understood to be a rupture of the arm's vascular nerve bundle as well as the shoulder's and scapular's stabilizing muscles and fibrous joints without damage to the dermal soft tissue sheath. This injury is a rare and grave incident which mostly occurs within the scope of a high-energy trauma and in cases of polytraumatized patients in combination with additional life-threatening injuries. In the literature this is referred to by the terms scapulothoracic dissociation (SD) and closed forequarter amputation--the entity of SD was first described by Oreck et al. in 1984. Traction of the upper extremity leads to the rupture of the m. pectoralis major, m. pectoralis minor, the musculi rhomboidei, the m. levator scapulae, the m. latissimus dorsi as well as the m. trapezius. Furthermore the traction causes acromioclavicular or sternoclavicular bursting of the joints or a displaced clavicle fracture. Among the more than 50 cases depicted until now, 94% of the patients exhibit a neurological (plexus) and 80% a vascular lesion, thus corresponding to a genuine inner amputation. The prognosis for this injury is consistently poor: 10% of the patients die, in 52% an nonfunctional extremity remains, and in 21% a untimely amputation has to be performed. The observed complication of cerebral hypoperfusion caused by increasing pressure in the neck compartment, which ultimately leads to the death of the patient, has, as far as we know, not yet been specified and emphasizes the gravity and the magnitude as well as the necessity of rapid diagnosis and appropriate therapy of this infrequent injury.

Adult↗