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| ORIGINAL ARTICLE |
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| Year : 2023 | Volume
: 9
| Issue : 1 | Page : 18-24 |
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Iliopsoas bleeds in patients with hemophilia: A single-center experience from South India
Meera Varadarajan, Smitha Ramaiah
Department of Clinical Hematology, Bangalore Medical College and Research Institute, Bangalore, India
| Date of Submission | 13-Jan-2023 |
| Date of Acceptance | 30-Mar-2023 |
| Date of Web Publication | 28-Apr-2023 |
Correspondence Address:
Meera Varadarajan
Associate professor and Head, Department of Clinical Hematology, Bangalore Medical College and Research Institute, Bangalore, 560002
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/mamcjms.mamcjms_8_23
Background: Iliopsoas bleed is a serious complication in people with hemophilia (PWH), with significant morbidity. Studies examining the profile, incidence, and outcomes in psoas bleeds are scarce and will shed more light and increase awareness about its management. Material and Methods: Data of 453 PWH with confirmed iliopsoas bleed treated at Bangalore Medical College were retrospectively analyzed. Results: Eighty-five (18.8%) PWH presented with total of 154 psoas bleeds. Their mean age was 21.96 years. Common symptoms were thigh/hip/groin pain (100.0%), hip flexion spasm (41.2%), numbness/tingling in quadriceps muscle (5.9%), abdominal tenderness (3.5%), hematuria, and anemia requiring blood transfusion in 2.4% each. Long-term complications were quadriceps atrophy (9.4%), permanent abnormal posture (10.6%), transient paresthesia in the distribution of femoral nerve (3.5%), and pseudo tumor in pelvis (1.2%). The overall average duration of therapy with clotting factor concentrate was 1.7 days with a mean duration of therapy of 1.4 days in patients without inhibitors and 2.8 days in patients with inhibitors (p = 0.010). The overall mean duration of hospitalization was 7.2 days with 5.3 days in PWH without inhibitors and 8.3 days in PWH with inhibitors (p = 0.342). Conclusion: Pain in hip joint/groin/hip flexion spasm suggests the possibility of an iliopsoas hematoma and early factor replacement therapy should be started to prevent complications. Early treatment at first sign of discomfort reduces the duration of treatment, and prevents severe complication and invasive interventions. Patients with inhibitors were overrepresented in the cohort who needed longer duration of factor replacement therapy.
Keywords: Bleeding disorder, hemophilia, hip bleed, iliopsoas bleeds, muscle bleed
How to cite this article:
Varadarajan M, Ramaiah S. Iliopsoas bleeds in patients with hemophilia: A single-center experience from South India. MAMC J Med Sci 2023;9:18-24 |
How to cite this URL:
Varadarajan M, Ramaiah S. Iliopsoas bleeds in patients with hemophilia: A single-center experience from South India. MAMC J Med Sci [serial online] 2023 [cited 2023 Jun 4];9:18-24. Available from: https://www.mamcjms.in/text.asp?2023/9/1/18/375340 |
| Introduction | |  |
Hemophilia A and B are the most common severe hereditary hemorrhagic disorders which result from factor VIII and IX deficiency, respectively. It is characterized by prolonged bleeding with or without trauma, depending on the factor activity. The estimated frequency of hemophilia is around 1 in 10,000 live births, and the number of people worldwide living with hemophilia is about 4,00,000.
Hemophilia A is more prevalent (80%–85%) than hemophilia B and seen in 1 in 5000 live male births, whereas hemophilia B presents in 1 in 30,000 live male births. Severity of both hemophilia A and B ranges from mild to moderate to severe. Factor VIII/IX levels in mild, moderate and severe categories are 5–40% of normal, 1–5%, and less than 1%, respectively. Joint bleeds are the most common bleeds in hemophilia. Joint bleeds are the most common bleeds in hemophilia. They have a varied clinical manifestation, with mild-to-severe presentation. Some PWH (approximately 30% with severe disease) may develop “inhibitors,” which are alloantibodies against the replacement factor VIII/IX, making treatment more difficult and thereby at greater risk of life-threatening bleeding events.
Muscle bleeds are the second commonest bleed in hemophilia and constitute 10% to 25% of the bleeds. It is a serious complication associated with significant morbidity. Iliopsoas bleeding, either spontaneous or traumatic, is quite common in patients with coagulation disorders and a potentially serious complication because a delay in beginning treatment may result in permanent femoral nerve palsy. It has a unique presentation with signs varying from pain in the lower abdomen, groin, and/or lower back and pain on extension, but not on rotation, of the hip joint. There may be paresthesia in the medial aspect of the thigh or other signs of femoral nerve compression such as loss of patellar reflex and quadriceps weakness.
Routine therapy for iliopsoas hematoma is conservative with factor replacement till complete resorption. Combination of physiotherapy and clotting factor replacement along with protection, rest, ice, compression, and elevation should be implemented in the acute stage. Physical activity should be restricted until pain resolves and hip extension improves. Treatment may be long drawn as factor levels have to be raised to a high level for a longer time, along with strict bed rest. A physiotherapist should be involved in the team to provide advice during the acute stage of the bleed, and then to provide ongoing exercise instruction and an exercise regime as improvement is made. Full recovery takes time.[1],[3]
Physiotherapy and rehabilitation should be done in three phases. Control of hemorrhage is the aim in first phase; restoration of Range of Movement (ROM) and strength is targeted in the second phase; functional rehabilitation and return to normal living is the aim of third and final phase. Delayed treatment results in permanent femoral nerve palsy and muscle dysfunction.[1],[2]
In spite of decades of widespread usage of state of art therapies for hemophilia, there have been very few studies examining the profile, incidence, or outcomes of this well-known complication in hemophilia, thereby shedding more light and increasing awareness about management of the dreaded iliopsoas bleeds. In a retrospective review of 453 PWH followed at our centre, we identified 154 episodes of iliopsoas hemorrhage in 85 patients. We present the experience at our Hemophilia Treatment Centre at Bangalore Medical College with iliopsoas bleeds and its complications in this paper.
| Materials and Methods | | |
The ethics committee approval was taken.
Study design – Retrospective observational study.
A total of 453 patient records, registered with Hemophilia at the Department of Clinical Hematology, BMCRI from 2015 to 2019 were evaluated retrospectively to look for confirmed iliopsoas bleeds by ultrasound scan and they were included for detailed analysis.
Eighty-five PWH had confirmed psoas bleed. Patients without confirmed psoas bleed, bleeds from other sites, and those with other bleeding disorders were excluded from the study. Detailed history was ascertained and clinical examination findings analyzed. All relevant characteristics like symptoms, signs, complications, and treatment were analyzed. All patients presenting with psoas bleeds were initially given 40% factor correction on first day followed by gradual tapering over 3 to 5 days as per World Federation of Hemophilia guidelines.[3] Physical therapy under guidance of our experienced physiotherapist was initiated as soon as pain subsided and increased gradually to restore full muscle length, strength, and function. Those with recurrent psoas bleeds were put on long-term prophylaxis. Details of past treatment and bleeding episodes, mode of presentation, and associated symptoms were taken. The results of investigations like ultrasound scan/CT of abdominopelvic region/MRI done to confirm iliopsoas bleeds were studied. Routine investigations including complete blood cell count were done to all admitted patients with severe bleeds. The mean duration of therapy and mean duration of hospitalization were calculated.
Statistical analysis
Data were collected in a predesigned proforma. All qualitative variables were expressed as frequency (%). All quantitative variables were expressed as mean ± standard deviation for normally distributed data and median with interquartile range for nonnormally distributed data. Data were presented in graphs and tables. SPSS version 20.0 was used for data analysis. Mann–Whitney U test was used to determine significant difference between with inhibitors and without inhibitors for the variables for days of factor treatment and days of hospitalization. P < 0.05 was considered statistically significant.
| Results | | |
Among the 536 patients with coagulation disorders, 381 were hemophilia A and 72 were hemophilia B (453 patients with hemophilia). Eighty-five (18.8%) patients presented with iliopsoas bleeds. There was a total of 154 bleeds in these 85 patients. Bleeds were confirmed by ultrasonography in all patients. The hematoma was spontaneous in 87.7% and traumatic in 12.3%.
Mean age of patients was 21.96 ± 10.44 and median age was 20 years, interquartile range (IQR 15, 28.5). Male-to-female ratio was 84:1. Forty-six (54.1%) were adults and 39 (45.9%) were children. This included 73 (16.1%) of hemophilia A and 12 (2.7%) of hemophilia B. Among the 85 PWH with iliopsoas bleed, 58 (68.2%) were severe hemophilia A, 13 (15.3%) had moderate hemophilia A, 2 (2.4%) were mild hemophilia A, 5 (5.9%) had moderate hemophilia B, and 7 (8.2%) were severe hemophilia B [Figure 1]. Sixty-nine (44.8%) had right-sided psoas bleed, 77 (50.0%) had on left side, and 8 (5.2%) had bilateral bleeds. 13.1% (50/381) of PWH with hemophilia A had inhibitors, out of whom 13 (26.0%) had iliopsoas bleeds.
Among 85 PWH, 52 (61.2%) had only 1 episode of bleed, 16 (18.8%) had 2 episodes, 8 (9.4%) had 3 episodes, 4 (4.7%) had 4 episodes, 1 (1.2%) had 5 episodes, 3 (3.5%) had 6 episodes, and 1 (1.2%) had 7 episodes of psoas bleed [Figure 2]. Among 12 PWH with inhibitors, 3 (25.0%) had 1 episode of bleed, 4 (33.3%) had 2 episodes, 2 (16.7%) had 3 episodes, 1 (8.3%) had 4 episodes, and 2 (16.7%) had 6 episodes. None of patients on prophylaxis had iliopsoas bleeds.
The most common symptoms at presentation were thigh, hip, or groin pain 85 (100.0%), 35 (41.2%) had hip flexion spasm, 3 (3.5%) had abdominal tenderness, numbness or tingling in quadriceps muscle was seen in 5 (5.9%), 2 (2.4%) had hematuria, and 2 (2.4%) had anemia with >2 g/dL hemoglobin drop requiring blood transfusion [Figure 3].
Factor replacement and rehabilitation therapy were the treatment given. A total of 50 (58.8%) PWH required treatment for 1 day only. All of these came to seek treatment for complaints of hip/groin pain only and with symptoms of 0- to 1-day duration. Twenty-two (25.9%) PWH required treatment for 2 days, 7 (8.2%) for 3 days, 2 (2.4%) for 4 days, and 4 (4.7%) for >1 week [Figure 4]. Patients presenting with more than 2 days history had symptoms like hip flexion spasm, abdominal tenderness, numbness or tingling in quadriceps muscle, hematuria, and anemia. The overall average duration of therapy with clotting factor concentrate was 1.7 ± 1.9 days IQR (1, 2) with a mean duration of therapy of 1.4 ± 0.7 days, IQR (1, 2), Range (1–4 days) in patients without inhibitors and 2.8 ± 3.3 days, IQR (1, 2), Range (1–17 days) in patients with inhibitors (p = 0.010).
Hospitalization was required for 10 patients, 5 (50%) of whom had inhibitors. The overall mean duration of hospitalization was 7.2 ± 4.8 days (Range 1–17 days) with 5.3 ± 3.2 days (Range 2–8 days) in patients without inhibitors and 8.3 ± 5.4 days (Range 1–17 days) in patients with inhibitors (P = 0.342), which was not significant. Physical therapy was started in all as soon as pain subsided.
None of them required any surgical intervention. Two episodes of bleed needed erythrocytes transfusion. Sequelae included transient paresthesia in 3 (3.5%) patients in the distribution of femoral nerve, which recovered over 3 months. Eight (9.4%) developed complication of quadriceps atrophy, 9 (10.6%) had permanent abnormal posture and 1 (1.2%) developed pseudo tumor in pelvis [Figure 5].
| Discussion | | |
Iliopsoas bleeds may occur following an injury, or spontaneously in severe hemophilia without any apparent cause. Iliopsoas is the deepest muscle in the body and consists of the iliacus and psoas muscles. It attaches the spine, pelvis to femur and allows us to flex at the hip and helps to walk upstairs or pick something up from the floor, to sit up, and to move the leg forward to walk or run. It stabilizes the spine and helps with posture.[1],[5] Iliopsoas hemorrhage is a potentially serious complication because a delay in beginning treatment may result in permanent femoral nerve palsy. It has a unique presentation with common signs being pain in the lower abdomen, groin, and/or lower back, with inability to straighten or stand up from a seated position; and pain on extension, but not on rotation, of the hip joint. Iliopsoas hemorrhage sometimes has a misleading presentation and maybe mistaken for acute appendicitis, with even a positive Blumberg’s sign (rebound tenderness) or thought to be a hip joint bleed. There may be paresthesia in the medial aspect of the thigh or other signs of femoral nerve compression, such as loss of the patellar tendon reflex, quadriceps weakness, and ultimately muscle wasting. They require thorough clinical evaluation and monitoring.[1],[2],[3],[4]
Factor replacement until recovery is the cornerstone in the management of iliopsoas hematomas and should be started immediately, as soon as patient feels the first signs of discomfort. Patient’s factor level has to be raised immediately, and maintained for 5 to 7 days or longer, as symptoms indicate. Severe bleeds require repeated infusions and higher levels of factor replacement for 2 to 5 days generally or much longer.
Apart from factor replacement and other relevant hemostatic therapy, adjunctive management of acute muscle bleeds is paramount. A carefully supervised program of physiotherapy is key to restoring full activity and function and preventing rebleeding. These comprise the POLICE (Protection with prosthesis, Optimal Loading, Ice application, Compression bandage, Elevation) therapy of hemophilia. Restoration of complete hip extension before returning to full activity is recommended. The limb should be in rest in a position of function and adequate analgesia is necessary. Patient’s activity should be restricted until pain resolves and hip extension improves. Splinting the affected limb in a position of comfort and adjusting to a position of function till pain subsides is helpful, application of ice/cold packs around the muscle for 15 to 20 minutes every 4 to 6 hours for pain relief is recommended.[2],[3],[4],[5],[6] Ambulation with crutches has risk of muscle contractions and exacerbates pain and bleeding and hence be avoided. Diagnosis should be confirmed using imaging studies like ultrasound, CT scan, or MRI and for monitoring patient recovery. However, when a muscle bleed is suspected clinically, hemostatic treatment is advised immediately even before sending for confirmatory tests or awaiting such results.[2],[3],[4]
Patients may need hospitalization for observation and pain control. Continued evaluation is important to avoid compartment syndrome, to monitor hemoglobin levels and vital signs until bleeding is controlled, and/or function is restored. Hemoglobin level should be checked and corrected if needed as muscle bleeds can result in significant blood loss.[5],[6],[7],[8]
Subsequently, following the initial acute phase, physiotherapy should begin early in order to restore full function. A carefully supervised program of physical therapy is crucial to restore complete hip extension and full activity and function, and prevent rebleeding. Factor prophylaxis during physical therapy and rehabilitation is recommended. In limited resources settings, physical therapy may be performed without factor coverage during the rehabilitation, under supervision of an experienced multidisciplinary team with musculoskeletal expertise. Treatment must be continued till bleed resolves and patient has recovered as much of their Range of Motion and muscle strength as possible.
Muscle bleeds can lead to musculoskeletal damage; therefore, early and effective factor replacement therapy and other appropriate hemostatic therapies are crucial to minimize and prevent complications. Delayed or untreated muscle bleeds lead to complications like nerve injury, compartment syndrome, myositis ossificans, pseudo tumor, and infection (abscess). Patients should be assessed regularly for neurovascular compromise; fasciotomy may be required in some cases after factor replacement. Percutaneous ultrasound-guided aspiration of blood can be performed in hematomas of large size. Rate of failed aspirations and hematoma repetition is significant.[5],[6]
If residual neuromuscular deficits persist, further orthotic support may be necessary, particularly to prevent flexion of the knee due to quadriceps weakness. Serial casting or splinting as required to correct any iliopsoas contracture will be necessary. Supportive bracing will be needed if there has been nerve damage. Recurrence of pain during physical therapy suggests rebleeding.[7],[8],[9],[10],[11],[12],[13],[14]
None of cases in our series required any surgical intervention and improved with conservative treatment. Thus, early recognition and treatment prevents severe complication and thereby invasive interventions. None of our patients on prophylaxis had iliopsoas bleeds. Prophylaxis is an effective modality to prevent muscle bleeds in hemophilia. Thus, prophylaxis protects against major bleeds like iliopsoas hematoma and should be widely implemented.
This is one of the largest and the first Indian series reported on psoas bleeds in hemophilia. In this series, 58.8% of PWH had presented very early with only hip or groin pain and hence required treatment for only 1 day. Hence, the mean duration of treatment and hospitalization is much lower compared to the other studies [Table 1].[7],[8],[13] The patients who presented with early and shorter duration of symptoms recovered early with shorter course of treatment. Hence, timely diagnosis and prompt replacement therapy are paramount and decrease the morbidity [Table 1] & [Table 2]. Patients with inhibitors were overrepresented in the cohort who needed longer duration of factor replacement therapy.
The number of subjects in our study was comparatively larger and this could account for the difference in the data results. Thus, our study shows that early treatment at the first hint of discomfort is not only very effective but also reduces the duration of treatment and the need for hospitalization. Longer follow up is needed.
| Conclusion | | |
Pain around the hip joint/groin or hip flexion spasm in a patient with hemophilia suggests the possibility of an iliopsoas hematoma and early and appropriate factor replacement therapy should be started to prevent complications. Early treatment at the first hint of discomfort is ideal and also reduces the duration of treatment and the need for hospitalization. Early recognition and conservative treatment prevent severe complication and thereby invasive interventions. Timely diagnosis and prompt replacement therapy are paramount and decrease the morbidity.
Prophylaxis protects against major bleeds like iliopsoas hematoma and should be widely implemented. Patients with inhibitors were overrepresented in the cohort who needed longer duration of factor replacement therapy. Physiotherapy and rehabilitation treatment are equally important for hematoma absorption and recovery.
Acknowledgment
The authors would like to acknowledge The Departments of Physiotherapy and Radiology, Bangalore Medical College and Research Institute, for their roles in rehabilitation of people with hemophilia (PWH) and contribution in confirming the iliopsoas bleeds through imaging the PWH, respectively.
Funding sources
There was no funding from any source.
Financial support and sponsorship
Nil.
Conflict of Interest
There was no conflict of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
[Table 1], [Table 2]
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