Presentation
Five patients presented a limp. Residual anterior-medial bowing of the tibia was observed in four patients. Calf atrophy was present in all seven patients. Valgus deformity of the ankle was remained in two patients. [ncbi.nlm.nih.gov]
[…] which case the foot remain unaffected; - it is prudent to remember that although congenital absence of fibula is evident, this condition is actually a total limb involvement; - males are affected twice as often as females in most series; - Clinical Presentation [wheelessonline.com]
In Type 1, the fibula is present but hypoplastic, whereas in Type 2, it is completely absent. [posna.org]
In case of syndromic presentation, prognosis depends on the nature of the associated anomalies. The documents contained in this web site are presented for information purposes only. [orpha.net]
Other skeletal anomalies (craniosynostosis, syndactyly, brachydactyly, oligodactyly and ectrodactyly) may also be present. Fibular hemimelia is also found in several generalized skeletal dysplasias and dysostoses. [medigoo.com]
Entire Body System
- Pain
(mean pain score, 0.2 compared with 1.2 points; p = 0.091), were more satisfied and had a lower complication rate (0.37 compared with 1.91; p < 0.05). - patients who underwent amputation also had fewer procedures (1.9 compared with 7.0; p < 0.05), at [wheelessonline.com]
This was true even though good results were obtained with the lengthening procedures and most patients achieved limb-length equality, were able to walk, had minimal pain, and were quite active. [ncbi.nlm.nih.gov]
Interestingly, what I've found searching "fibular hemimelia" in the last year or so is that amputation is once again the most common (even most desirable) treatment--and that apparently "children who undergo early amputation are more active, have less pain [poesygalore.blogspot.com]
They should also look into the techniques used to minimize the possibility of phantom pain post amputation. Individuals with chronic pain prior to amputation are at greater risk for chronic phantom pain post-amputation. Yes. [opedge.com]
- Crying
Our experience with little kids with prosthetics was the occasional “make you cry” youtube video or inspirational story on Dateline. [findinglovely.com]
- Asymptomatic
FH can be asymptomatic, but is usually obvious at birth with limb shortening and limb length discrepancy [3]. It may be detected antenatally during obstetric ultrasound. [eurorad.org]
The final result was that 16 feet were plantigrade, stable and asymptomatic, and five had residual valgus with stiffness, requiring an orthosis to alleviate the symptoms. [ncbi.nlm.nih.gov]
Musculoskeletal
- Genu Valgum
Genu valgum continues to be a concern after amputation and may affect prosthetic fitting. Guided growth methods have been utilized effectively to treat this. [posna.org]
Physical examination showed genu valgum, anteromedial rotatory instability and recurvatum of the right knee. We decided to perform surgical correction of the angular deformities and ACL reconstruction in the same surgical time. [ncbi.nlm.nih.gov]
Outcome of eight-plate hemiepiphysiodesis on genu valgum and height correction in bilateral fibular hemimelia. J Pediatr Orthop B. 2014 Jan. 23(1):67-72. Pappas AM, Hanawalt BJ, Anderson M. Congenital defects of the fibula. [boneandspine.com]
- Fracture
Previous interventions, associated congenital anomalies, regenerate fractures were recorded with reference to medical charts and confirmed on appropriate radiographs. Successful lengthening was defined as the healing index [ncbi.nlm.nih.gov]
and CLAWSON, DK: Closed Intramedullary Nailing of Femoral Fractures. A Report of Five Hundred and Twenty Cases. الصفحة 256 - Ilizarov GA. The tension-stress effect on the genesis and growth of tissues: Part II. [books.google.com]
In some cases, the bone may heal in a crooked position, which is known as a fracture malunion. Other times, the fracture may heal straight, but the bone’s growth plate is partially damaged. [childrensortho.com]
- Long Legs
A long-leg standing series shows the overall picture of the affected lower extremity and permits use of the contralateral side as a control. Limb-length discrepancies and alignment can be measured. [emedicine.medscape.com]
Step 2: Epiphysiodesis: shorten the long leg We saw Dr. Scott on Sept. 21. [fibularhemimelialeglengthening.blogspot.com]
We use the Multiplier Method [ 23, 24 ] to predict the ultimate LLD and ultimate overall height (based on the long leg) at skeletal maturity. [obgynkey.com]
After the TSF is removed, a long leg cast is applied for 2 to 4 weeks. The post-removal cast protects both the new bone and the pin holes left by the external fixator from potential fracture. [limblength.org]
- Hip Dislocation
In this case report, we present a patient with right teratologic high hip dislocation, femoral hypoplasia and below-knee hemimelia associated with left fibular hemimelia. [ncbi.nlm.nih.gov]
[…] patterns and skeletal maturation in children with unilateral fibular hemimelia. ( 30269159 ) 2019 2 Hybrid ACL reconstruction in a 6-year-old female with fibular hemimelia. ( 30719315 ) Tate PG...Wojtys EM 2019 3 Bilateral fibular hemimelia associated with hip [malacards.org]
Pelvic hypoplasia, congenital hip dislocation, and absence or coalescence of the tarsal bones are additional findings. A peculiar constellation of abnormalities, sharing similarities with the syndromes of Fuhrmann and Al-Awadi/Raas- Fig. 5.34 a, b. [rrnursingschool.biz]
Dislocation of hip / Hip dysplasia - Upington disease feet ( Club foot, Flat feet, Pes cavus ) systemic dislocations Larsen syndrome head, face, spine and chest : skull, face and jaw ( Dolichocephaly, Greig cephalopolysyndactyly syndrome, Plagiocephaly [wikidoc.org]
- Joint Subluxation
The abnormal posterolateral structures, combined with cruciate ligament deficiency could be associated with joint subluxation, stress change and early degenerative findings [1]. [eurorad.org]
The knee must be assessed carefully before initiation of limb lengthening treatment in order to prevent joint subluxation or dislocation. [posna.org]
Psychiatrical
- Distractibility
The alternative of distraction lengthening using the Ilizarov technique is now available. We report three patients (four limbs) with type-II fibular hemimelia who were treated by the Ilizarov technique and followed up for two to six years. [ncbi.nlm.nih.gov]
The influence of the rate and frequency of distraction. Clin Orthop 1989;(239):263-85. الصفحة 66 - The rate of pseudarthrosis (surgical nonunion) in patients who are smokers and patients who are nonsmokers: a comparison study. [books.google.com]
Distraction at the osteotomy site of the tibia could not start in one patient who was needed to undergo a revision osteotomy on the 7 th post-operative day which followed by identical distraction of the osteotomized bone segments. [josr-online.biomedcentral.com]
Provides up-to-date details on rib-based distraction implants (VEPTR ) and remote-controlled growing rods (MAGEC) for scoliosis; diagnosis of femoroacetabular impingement (FAI) and its influence on development of osteoarthritis; and the treatment of FAI [books.google.ro]
- Fear
He is MY inspiration.. that all can be overcome & sometimes needs reminding of that on some tough days, but all in all theres nothing to fear about FH. [netmums.com]
"Poetry is what elevates the banal and neglected object to the realm of art; it can transform the thing that might have made people fearful into something that invites them to look and look a little longer.. . my legs could be wearable sculpture." [upliketoast.blogspot.com]
Perhaps you’re crippled by fear of failure or don’t see the innate value you have as a human being. We battle an almost constant influx of fears and failures. Or we really do have physical limitations that prevent us from running like the wind. [mjandcade.com]
- Grieving
Once we found out we were expecting a little boy with this abnormality and we had grieved and healed enough from the grief journey, we began scouring the internet (where else do people turn these days?) for information about a possible diagnosis. [findinglovely.com]
She cried and grieved for days. He ran into our arms and never looked back. It was obvious to us that day that we were dealing with more than a malformed foot. [nohandsbutours.com]
Neurologic
- Irritability
Cade has tolerated the liners well, he’s never had skin irritation or a rash, thank goodness. [mjandcade.com]
This does not even factor in the frequent adjustments and modifications to the prosthesis that are required, nor the intermittent skin irritation of the stump to the prosthetic that causes some pain and suffering and sometimes interrupts prosthetic use [ncbi.nlm.nih.gov]
Treatment
Treatment by Syme amputation: Indications and technique. Syme amputation: An evaluation of the physical and psychological function in young patients. Amputation and prosthesis as definitive treatment in congenital absence of the fibula. [wheelessonline.com]
Families often struggle with making treatment decisions in this scenario and often find it helpful to speak with families who have undergone either treatment. [posna.org]
A report of common post-treatment residual deformities should be valuable in best possible treatment planning of fibular hemimelia. [ncbi.nlm.nih.gov]
Fibular Hemimelia Treatment complete fibular hemimelia = ankle disarticulation in early childhood and fitting a Syme-type prosthesis. [eorif.com]
Prognosis
Prognosis Fibular deficiency is usually a benign condition, although in severe cases it can be debilitating. Acceptable functional results may be achieved by surgery. [orpha.net]
The early prognosis was that Jake would never walk...The Browns finally found comfort at Shriners Hospital in St. Louis when they met a family from Indiana with a daughter Jake's age who also had the condition. [poesygalore.blogspot.com]
In case of syndromic presentation, prognosis depends on the nature of the associated anomalies. Last updated: 10/24/2008 [rarediseases.info.nih.gov]
Coverage of each disorder includes more details on treatment and prognosis. [books.google.com]
Prognosis Because postaxial hypoplasia of the lower extremity represents such a wide range of abnormalities with varying degrees of involvement, no simple statement can be made regarding the patient's prognosis. [emedicine.medscape.com]
Etiology
[…] synonyms: Congenital absence of the fibula Fibular Hemimelia ICD-10 Q72.60 Longitudinal reduction defect of unspecified fibula Fibular Hemimelia ICD-9 755.37 longitudinal defiency, fibular, complete or partial Fibular Hemimelia Etiology, Natural History [eorif.com]
In this article, we review the embryology of the extremities, discuss proposed etiologies for fibular hemimelia, highlight associated abnormalities, and present the radiographic and imaging findings. [uncch.pure.elsevier.com]
Etiology The etiology is unclear. The deformity is probably due to disruptions during the critical period of embryonic limb development, between 4th and 7th week of gestation. [orpha.net]
(Birch, 2011; Acheterman, 1979; Rodriguez-Ramirez, 2010; Stevens, 2000) Epidemiology: The etiology of fibular hemimelia remains unclear. [posna.org]
Epidemiology
Summary Epidemiology Prevalence is estimated at 1 in 50,000. A slight male preponderance has been reported in some studies, whereas other reports describe an equal sex distribution. [orpha.net]
Introduction Define/describe the condition Anatomy Describe the pertinent anatomy Pathogenesis Describe the biomechanics/biologic basis of the disorder or the mechanism of injury Natural History Describe the natural history, epidemiology and prognosis [orthopaedicsone.com]
Epidemiology Prevalence is estimated at 1 in 50,000. A slight male preponderance has been reported in some studies, whereas other reports describe an equal sex distribution. [rarediseases.info.nih.gov]
Definition Deficiency of bony elements on lateral side of lower limb - characterised by hypoplastic or aplastic fibula Types Terminal - abnormal foot Intercalary - normal foot Epidemiology Most common congenital deformity of long bones - M:F 2:1 May be [52.62.202.235]
Prevention
[…] valgus reccurence & helps in lengthening, 3) to prevent damage during deformity correction 4) increase arc of motion of ankle joint 1. [slideshare.net]
It acts as a buttress to the talus and prevents the recurrence of valgus deformity. [cyberleninka.org]
The knee must be assessed carefully before initiation of limb lengthening treatment in order to prevent joint subluxation or dislocation. [posna.org]
[…] conclusion that the bifurcation operation recommended by Gruca produces a new lateral malleolus with an epiphysis that grows at the same rate as the remainder of the lower epiphysis of the tibia, does not affect the rate of growth of the tibial epiphysis, prevents [web.archive.org]
Children’s Healthcare of Atlanta (“CHOA”) provides free access to certain materials and information, documentation, forms, questionnaires and diagrams relating to the study, prevention, and treatment of concussions via this website and its related pages [choa.org]