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Fibular nerve palsy after hip replacement: Not only surgeon responsibility. Hereditary neuropathy with liability to pressure palsies (HNPP) a rare cause of nerve liability
Abstract
Mononeuropathy after surgery may occur and hereditary neuropathy with liability to pressure palsies is a possible pathological condition related to paresis after hip surgery. We present a case of 66-year-old man presenting severe weakness at inferior limb muscles after hip prosthesis revision. Clinic and electrophysiology showed severe right fibular nerve damage and ultrasound found a marked enlargement of the same nerve, associated with focal enlargements in other nerves. A diagnosis of hereditary neuropathy with liability to pressure palsies was suspected and confirmed by genetic test. The patient gradually recovered returning to a normal daily active life. Ultrasound was crucial for diagnosis. The suspicion and diagnosis of latent neuropathy, which can occur after surgical intervention, may lead to a better understand of the risks of the surgery, specific for the patient, and avoid the wrong attribution to surgical malpractice.
... Certain prolonged positions, such as flexion and extension of limbs, are potential triggers, highlighting the importance of careful intra-operative positioning for these patients, especially during prolonged surgical procedures [17,35,46,53]. HNPP primarily affects nerves that are vulnerable to compression due to their anatomical location relative to bony prominences, and particularly the radial, median, ulnar, and common fibular nerve fibers [53,57,58]. While less frequently affected, the facial nerve has been previously described as also liable to pressure palsy [58]. ...
... While less frequently affected, the facial nerve has been previously described as also liable to pressure palsy [58]. As such, common sites include, in descending order of frequency: peroneal nerve entrapment at the fibular head leading to foot drop, ulnar nerve compression at the elbow with hypothenar and interossei paresis, classical carpal tunnel syndrome, and brachial plexus injuries [54,[57][58][59][60][61]. Chronic or prolonged entrapment may also cause muscle wasting, such as seen in carpal tunnel syndrome [30,46,59]. ...
... Multidisciplinary discussions can aid in treatment planning when anticipated deviation from the standard of care is necessary. Patient involvement in this process, and implementing the "shared consent" concept, seems prudent [57,79]. Documenting any pre-existing neuropathies also seems prudent practice. ...
Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant demyelinating neuropathy characterized by an increased susceptibility to peripheral nerve injury from trauma, compression, or shear forces. Patients with this condition are unique, necessitating distinct considerations for anesthesia and surgical teams. This review describes the etiology, prevalence, clinical presentation, and management of HNPP and presents contemporary evidence and recommendations for optimal care for HNPP patients in the perioperative period. While the incidence of HNPP is reported at 7–16:100,000, this figure may be an underestimation due to underdiagnosis, further complicating medicolegal issues. With the subtle nature of symptoms associated with HNPP, patients with this condition may remain unrecognized during the perioperative period, posing significant risks. Several aspects of caring for this population, including anesthetic choices, intraoperative positioning, and monitoring strategy, may deviate from standard practices. As such, a tailored approach to caring for this unique population, coupled with meticulous preoperative planning, is crucial and requires a multidisciplinary approach.
... He reported a case of post-operative foot drop after a total hip replacement with total spontaneous recovery in three months in a patient who later was found to have HNPP. 5 In Logroscino's case, ultrasound was employed to show enlargement in the fibular nerve, just as MRI demonstrated in our case. ...
There are many causes of acute onset foot drop ranging from deep fibular nerve or sciatic nerve injury caused by trauma or a compressive mass such as a neuroma, to spinal cord disorders like disc herniation causing L4-5 radiculopathy, and various muscular dystrophies affecting the tibialis anterior muscle responsible for foot dorsiflexion and eversion. Even brain disorders like MS, stroke or ALS can result in foot drop. We present a case of bilateral foot drop as a complication of rapid 70 lb weight loss which was described in literature previously as “slimmer’s palsy”.
Hereditary neuropathy with liability to pressure palsy (HNPP) is a rare peripheral neurological disorder that manifests with increased sensitivity to pressure. In people with this disorder, the peripheral nerves are unusually sensitive to pressure. Minor trauma or compression causing paralysis in the extremities is a hallmark of this disorder. Ensuring there is no pressure on the extremities is recommended as a preventive measure. We describe for the first time, postoperative vocal cord paralysis in a patient with HNPP due to left recurrent laryngeal nerve palsy. Anesthesiologists and surgeons should be aware of this possible complication in patients with HNPP.
The aim of this single-centre retrospective study was to evaluate the outcomes of carpal tunnel release surgery in patients with hereditary neuropathy with pressure palsies (HNPP). The secondary aims were to identify prognostic factors for the outcome of carpal tunnel release and to assess the outcome of cubital tunnel release. Our primary hypothesis was postoperative improvement. In total, 18 patients (26 carpal tunnel releases) with at least one symptomatic carpal tunnel syndrome were included. At a median follow-up of 8.5 years, more than 73% of the patients were satisfied with the results. The visual analogue scale (0 to 10) for discomfort decreased by 2.2 points ( p < 0.001). The Boston Carpal Tunnel Questionnaire symptom severity scale decreased by 1.3 points ( p < 0.001). The decrease in the Functional Status Scale was not significant. No significant prognostic factor for outcome was identified. A total of 12 patients also underwent cubital tunnel release, and three patients underwent just this procedure (23 procedures). Despite the lack of preoperative data, cubital tunnel release provided encouraging results.
Level of evidence: III
Hereditary neuropathy with liability to pressure palsy (HNPP) is a rare autosomal dominant disease characterized by focal, recurrent, demyelinating peripheral neuropathies. It is caused by deletions of the gene encoding for peripheral myelin protein 22 (PMP22) on chromosome 17. While it may range widely, the most common clinical presentation is an acute, focal mononeuropathy with numbness or muscle weakness after trauma or compression. Diagnostic tools include electrophysiological studies, genetic tests and nerve biopsies. There is no standard surgical or pharmacological treatment. The course of the disease is usually benign, with spontaneous improvement after most episodes of peripheral nerve palsy. HNPP is best managed by early detection, preventative measures, and subsequent treatment of symptoms. According to the medical literature, operative treatment was undertaken in few cases and limited to decompression of the nerve at the classic entrapment sites of the carpal or cubital tunnels. We present a case of multiple tendon transfer (pronator teres to extensor carpi radialis brevis and flexor carpi radialis to extensor digitorum communis) with a two-year follow-up in a 24-year-old woman with HNPP who was affected by irreversible radial nerve palsy, and conclude with a review of the medical literature related to the disease.
The incidence of nerve injury ranges from 0.17% to 3.7% during primary hip arthroplasty and is reported to be as high as 7.5% in revision surgery. It is an often serious complication arising from this frequently performed surgical procedure. There is a wide range of severity of nerve injury, with more severe injuries having a notable impact on the patient’s overall functional outcome. In describing the pathophysiology and risk factors for nerve injury during total hip arthroplasty, the authors will delineate preventative measures and appropriate steps for referral and subsequent management.
Background
Individual participant data was reviewed of patients who sustained isolated common peroneal nerve (CPN) injuries resulting in foot drop. Functional results were compared between eight interventions for CPN palsies to determine step-wise treatment approaches for mechanisms of nerve injury.
Methods
PubMed, EMBASE, Cochrane Library, Web of Science, Scopus and CINAHL databases were searched. PRISMA-IPD and Cochrane guidelines were followed. Eligible patients sustained isolated CPN injuries resulting in foot drop. Patients were stratified by mechanisms of nerve injury, ages, durations of motor symptoms, and nerve defect/zone of injury sizes and compared by functional results (poor=0, fair=1, good=2, excellent=3), using meta-regressions between interventions. Interventions evaluated were primary neurorrhaphy, neurolysis, nerve grafts, partial nerve transfer, neuromusculotendinous transfer, tendon transfer, ankle-foot orthosis (AFO), and arthrodesis.
Results
One hundred and forty-four studies included 1284 patients published from 1985 through 2020. Transection/Cut: Excellent functional results following tendon transfer (OR:126, 95%CI:6.9, 2279.7, p=0.001), compared to AFO. Rupture/Avulsion: Excellent functional results following tendon transfer (OR:73985359, 95%CI:73985359, 73985359, p<0.001), nerve graft (OR:4465917, 95%CI:1288542, 15478276, p<0.001), and neuromusculotendinous transfer (OR:42277348, 95%CI:3001397, 595514030, p<0.001), compared to AFO. Traction/Stretch: Good functional results following tendon transfer (OR:4.1, 95%CI:1.17, 14.38, p=0.028), compared to AFO. Entrapment: Excellent functional results following neurolysis (OR:4.6, 95%CI:1.3, 16.6, p=0.019), compared to AFO.
Conclusions
Functional results may be optimized for treatments by mechanism of nerve injury. Transection/Cut and Traction/Stretch had best functional results following tendon transfer. Rupture/Avulsion had best functional results following tendon transfer, nerve graft, or neuromusculotendinous transfer. Entrapment had best functional results following neurolysis.
Development of peripheral nervous system (PNS) myelin involves a coordinated series of events between growing axons and the Schwann cell (SC) progenitors that will eventually ensheath them. Myelin sheaths have evolved out of necessity to maintain rapid impulse propagation while accounting for body space constraints. However, myelinating SCs perform additional critical functions that are required to preserve axonal integrity including mitigating energy consumption by establishing the nodal architecture, regulating axon caliber by organizing axonal cytoskeleton networks, providing trophic and potentially metabolic support, possibly supplying genetic translation materials and protecting axons from toxic insults. The intermediate steps between the loss of these functions and the initiation of axon degeneration are unknown but the importance of these processes provides insightful clues. Prevalent demyelinating diseases of the PNS include inherited neuropathies Charcot-Marie-Tooth Disease, Type 1 (CMT1) and Hereditary Neuropathy with Liability to Pressure Palsies (HNPP) and the inflammatory diseases Acute Inflammatory Demyelinating Polyneuropathy (AIDP) and Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). Secondary axon degeneration is a common feature of demyelinating neuropathies and this process is often correlated with clinical deficits and long-lasting disability in patients. There is abundant electrophysiological and histological evidence for secondary axon degeneration in patients and rodent models of PNS demyelinating diseases. Fully understanding the involvement of secondary axon degeneration in these diseases is essential for expanding our knowledge of disease pathogenesis and prognosis, which will be essential for developing novel therapeutic strategies.
Hereditary neuropathy with liability to pressure palsies (HNPP) is characterized by recurrent sensory and motor neuropathy in individual nerves starting in adolescence or young adulthood, focal conduction abnormalities at entrapment sites on nerve conduction studies, and sausage-like swellings (tomacula) of the myelin sheaths by nerve biopsy. It is characterized genetically by the deletion of the chromosome 17p11.2–p12 region including the peripheral myelin protein-22 gene in the overwhelming majority of cases. HNPP may be frequently underdiagnosed or misdiagnosed owing to the heterogeneity of clinical and electrophysiological appearance. The main objective of this review is to describe clinical manifestations, paraclinical features such as electrodiagnostic, pathological, radiological and genetics findings, and possible treatments.
Genetic factors may be learnt from families with gene mutations that render nerve-injury susceptibility even to ordinary physical activities. A typical example is hereditary neuropathy with liability to pressure palsies (HNPP). HNPP is caused by a heterozygous deletion of PMP22 gene. PMP22 deficiency disrupts myelin junctions (such as tight junction and adherens junctions), leading to abnormally increased myelin permeability that explains the nerve susceptibility to injury. This finding should motivate investigators to identify additional genetic factors contributing to nerve vulnerability of injury.
The main objective of this study is to assess the course of peroneal mononeuropathy (PM). The study design includes Clinical and Prospective study. The setting involves neurophysiological Service. From November 2002 to January 2004, we enroled 69 consecutive patients and prospectively followed up 49 patients with multiple measurements. Comparison was made between follow-up and baseline values, and baseline factors were used to predict the PM evolution in multiple regression analysis. At follow-up, we observed a significant improvement of all clinical, neurophysiological and disability measurements, and physical aspects of quality of life (QoL). Greater muscle strength of tibialis anterior and higher conduction velocity of peroneal nerve at baseline were seen to be positive prognostic factors. A better evolution of mental aspects of QoL was observed in the subacute group and in younger patients, while a better physical evolution in QoL was observed in women. Rehabilitation is ambiguously associated with a better Deambulation Index but lower ratings in mental aspects of QoL. In conclusion, PM shows a positive spontaneous course and rehabilitation seems to help the recovery of deambulation. Further studies on the effects of conventional rehabilitation are needed.
Background
Repair of soft tissue in favour of the posterior approach for total hip arthroplasty is still under discussion and few studies are assessing this issue. Therefore, we performed a meta-analysis to compare the effectiveness and safety of the posterior approach for total hip arthroplasty with and without soft tissue repair. We focused on these questions as follows: does primary posterior approach for total hip arthroplasty with soft tissue repair has better result regarding dislocation rate, Harris hip score and the sciatic nerve palsy rate compared with posterior approach without soft tissue repair.
Patients and methods
We conducted electronic literature searches using CENTRAL (Issue 1 of 12, Jan 2014), PUBMED (1980 to Jan 2014), and EMBASE (1980 to Jan 2014). Clinical studies evaluating the posterior approach for total hip arthroplasty with and without soft tissue repair were collected. After independent study selection by 2 authors, data were collected and extracted independently. The methodological quality of the studies was assessed by the Cochrane Collaboration's tool for assessing risk of bias and the Newcastle-Ottawa Scale.
Result
Seven clinical trials with 4594 hips using the posterior approach for total hip arthroplasty were included. The pooled data indicated a lower rate of dislocation (OR: 0.14, 95% CI: 0.08–0.26, P < 0.00001) and higher Harris hip score (1.75, 95% CI: 1.19 to 2.32, P < 0.00001, I² = 26%) after the posterior approach to total hip arthroplasty using soft tissue repair than without using soft tissue repair. There was no statistical difference in sciatic nerve palsy between the use of soft tissue repair and without it in posterior approach to total hip arthroplasty (OR: 5.34, 95% CI: 0.25–112.25, P = 0.28).
Discussion
Our meta-analysis included data from more studies than were previously available and demonstrated that the use of soft tissue repair and without it in posterior approach to total hip arthroplasty are similar in safety. Using repair resulted in a lower dislocation rate and higher Harris hip score than without repair.
Levels of evidence
Level 2 meta-analysis of low-powered prospective randomised trial.
An epidemiological study of hereditary neuropathy with liability to pressure palsies (HNPP) was carried out in south western Finland, with a population of 435,000. The diagnosis was established in 69 patients from 23 unrelated families through family and medical history, clinical neurological and neurophysiological examinations and with documentation of the deletion at gene locus 17p11.2 in at least one member of each family. This gave a prevalence of at least 16/100,000, which is remarkably high. However, due to the insidious nature of HNPP, most probably it is still an underestimation. This is the first population-based prevalence figure reported for HNPP. The prevalence is somewhat lower than that obtained for CMT in the same population, which agrees with the proposal that HNPP and CMT 1A are reciprocal products of the same unequal crossing-over. The clinical pictures of our patients were, in general, similar to those previously described in HNPP.
The authors review the molecular genetics and pathophysiology of hereditary recurrent focal neuropathies: hereditary neuropathy with liability to pressure palsies (HNPP) and hereditary neuralgic amyotrophy (HNA). Significant progress in the understanding of HNPP and HNA has been achieved. HNPP and HNA are distinct clinical and pathologic disease entities with autosomal dominant inheritance. Molecular genetic studies have shown that HNPP and HNA are located on chromosome 17 but at distinct genetic loci (17p11.2 for HNPP, 17q25 for HNA). The 1.5 megabase deletion in 17p11.2 is the major cause of HNPP. This interstitial deletion causes the complete loss of one allele of the peripheral myelin protein 22 (PMP22) gene. Interestingly, rare HNPP patients are found without the 1.5 megabase deletion. However, these patients have distinct mutations in the PMP22 gene resulting in altered expression of the PMP22 protein. Current molecular genetic tests and clinical guidelines allow improved diagnosis, prognosis, and genetic counseling for patients with HNPP. Such tests are not available for HNA, because the disease-causing gene remains unknown. Molecular genetic advances in HNPP and HNA, as well as the study of transgenic animal and cellular models, will provide a more precise understanding of the disease mechanisms and will lead to the development of effective therapeutic tools for patients with inherited and sporadic recurrent peripheral neuropathies.
The most common mononeuropathy in the lower extremity involves the nerve. We retrospectively evaluated the etiological predisposing factors and clinical-neurophysiological features of 36 patients affected by peroneal mononeuropathy (PM). In 30 patients, a clear predisposing factor was identified. PM was more frequently perioperative (11 cases), associated with axonal involvement. Unexpectedly, PM was not only due to surgery close to the peroneal region, but was mostly associated with hip surgery and, rarely, with thoracic-abdominal surgery. A postural predisposing factor of PM was also frequently observed, usually associated with a pure conduction block. Conversely, most patients with bedridden predisposing factor presented axonal involvement, which was rarely associated with conduction block. In 25 of 36 PM cases, a long-term follow-up lead to an improvement (12 cases) or to good recovery (13 cases) of PM. In conclusion, our study shows that: (1) in most PM cases it is possible to identify a predisposing factor; (2) there is a good correlation between predisposing factor and neurophysiological involvement, and (3) PM usually has usually a good prognosis.
Hereditary neuropathy with liability to pressure palsies (HNPP) is a disorder characterized by a tendency to develop focal neuropathies after trivial traumas. On teased nerve fiber studies, sausage-shaped myelin sheath swellings (tomacula) are found. We report the sonographic findings in a patient with genetically proven HNPP. We were able to demonstrate enlargement of several peripheral nerves, even nerves that were clinically unaffected. Enlargement was found not only at typical nerve entrapment sites but also outside these sites. This diffuse nerve enlargement may play an important role in the pathogenesis of entrapment neuropathies in HNPP patients.
Delayed lesions of the femoral or sciatic nerve are a rare complication after total hip arthroplasty. Several cases in association with cement edges, scar tissue, broken cerclages, deep hematoma, or reinforcement rings have been published. We report about a 62-year-old female who developed a pure motor paresis of the quadriceps muscle 2 weeks after total hip arthroplasty. After electrophysiological evaluation had revealed an isolated femoral nerve lesion, revision of the femoral nerve was performed. During operative revision no pathologic findings could be seen. One week later the patient developed paralysis of the left wrist and finger extensors after using crutches. Electrophysiological evaluation revealed several nerve conduction blocks in physiological entrapments and the diagnosis of hereditary neuropathy with liability to pressure palsies (HNPP) was established. Hereditary neuropathy with liability to pressure palsies (HNPP) is a rare disease with increased vulnerability of the peripheral nerve system with mostly reversible sensorimotor deficits. It should be taken into consideration in cases of atypical findings of compression syndromes of peripheral nerves or delayed neuropathy, e. g., after total hip arthroplasty.
The study was performed to evaluate neurological complication of 562 patients who had undergone primary total hip replacement. Lesions of sciatic, femoral and common peroneal nerve occurred in 18 patients (3.2%). Paresis of peroneal nerve was demonstrated in 15 patients. Within this group 7 patients had elongation of the extremity above 4 cm, and 8 patient had it in the range of 2-3 cm. Paresis of common peroneal nerve took place after direct compression of region of fibular head. Lesion of femoral nerve happened twice. In the group of 14 patients clinical symptoms of nerve paresis healed spontaneously in 6-12 month time. In the group of 4 patients (0.7%) constant lost of dorsal flexion of the foot was observed.