Home Current Issue Previous Issues Collections For Authors Journal Info
Skip Navigation LinksHome > January 2012 - Volume 24 - Issue 1 > The Prosthetic Rehabilitation of a Patient With Hip Disartic...
JPO Journal of Prosthetics & Orthotics:
doi: 10.1097/JPO.0b013e318240e066
Article

The Prosthetic Rehabilitation of a Patient With Hip Disarticulation as a Result of Klippel–Trenaunay–Weber Syndrome: Case Report

Ülger, Özlem PT, PhD; Topuz, Semra PT, PhD; Bayramlar, Kezban PT, PhD; Erbahçec[Combining Dot Above], Fat[Combining Dot Above]h PT, PhDıı; Ş[Combining Dot Above]mşek, I. Eng[Combining Dot Above]n PT, PhDıı; Şener, Gül PT, PhD

Free Access
Article Outline
Collapse Box

Author Information

ÖZLEM ÜLGER, PT, PhD, SEMRA TOPUZ, PT, PhD, KEZBAN BAYRAMLAR, PT, PhD, FATIH ERBAHÇECI, PT, PhD, I. ENGIN ŞIMŞEK PT, PhD, AND GÜL ŞENER, PT, PhD, are affiliated with the Prosthetics and Biomechanics Unit, Physiotherapy and Rehabilitation Department, Health Sciences Faculty, Hacettepe University, Ankara, Turkey.

Disclosure: The authors have no conflicts of interest to disclose.

Correspondence to: Kezban Yiğiter-Bayramlar, PT, PhD, Department of Physical Therapy and Rehabilitation, Prosthetics and Biomechanics Unit, Faculty of Health Sciences, Hacettepe University, 06100 Samanpazari, Ankara, Turkey; e-mail: kezbanyigiter@yahoo.com

Collapse Box

Abstract

ABSTRACT: There are various reasons for an amputation. Sometimes a systemic disease as chicken pox or a neurological disease such as spina bifida and sometimes a rare neurocutaneous disease such as Klippel–Trenaunay–Weber (KTW) syndrome can cause amputation. This article presents the prosthetic rehabilitation of a 16-year-old boy with KTW syndrome. In our examination, we observed that there were no wounds at the distal part of the residual limb; however, we found eczema in some regions. The patient had no phantom pain/feeling or any limitation concerning the sound side joint ranges; however, hamstring and gastrosoleus muscle groups were short, abductors and adductors were weak, and upper limb gross muscle strength was good. Sensory evaluation of the residual limb revealed hyperesthesia on the anterior distal part of the residual limb. The physiotherapy-rehabilitation program consisted of stretching and strengthening exercises, residual limb dynamic and isometric exercises to prepare the amputee for gait, as well as balancing and weightbearing exercises. As the program continued, the measurements for the prosthesis were taken. The amputee was able to walk with his prosthesis without bilateral crutches at the end of 2 weeks. The patient was evaluated with a gait trainer, footprint, Nottingham Health Profile, and the Medicare Classification Functional Level system before and after the rehabilitation program. It can be said that physiotherapy-rehabilitation approaches and prosthetic training were important facilitators for regaining independence.

Impairments, such as result from chronic childhood diseases, can lead to permanent disability, leading to expenditure of family and community time and effort for the lifetime of the person with a disability.1,2

Amputation may possibly cause a number of impairments in the child's motor and psychosocial development in addition to its permanent disability effects. It is possible that these impairments may be transformed into acquired or regained skills with a successful prosthetic fit and rehabilitation. A rehabilitation program, physiotherapy approaches, and a well-designed prosthesis can only be successful with an appropriate evaluation of the child's functional competencies and functional limitations with the goal of encouraging and assisting the child in accordance with his or her developmental status.1,3

There are numerous factors affecting prosthetic training and rehabilitation. The most important factors are age, reason for the amputation, level of amputation, sociocultural level of the patient, and the presence of accompanying systemic diseases. These factors and the amputee should be evaluated with different methods, and in the light of these assessments, a program should be prepared with a multidisciplinary approach. Then, with a custom prosthetic design, the rehabilitation process should begin at once.3

If the amputation age is young, the rehabilitation program should begin as soon as possible. During prosthetic training and rehabilitation, sitting-standing, ascending-descending the stairs, balance, weightbearing on the prosthetic side, and ambulation activities training must be given. In the rehabilitation of an upper limb amputee, daily activities such as eating, personal care, dressing, and fine motor skills such as opening a door, carrying a bag, buttoning up, and shoe lacing should be introduced to the child in accordance with his or her physical and functional status and motor development level.4

Moreover, the amputation level affects a child's functional level. Impaired function in daily activities normally increases at the hip or shoulder disarticulation amputation levels. In addition to the rehabilitation program, the prosthetic components also have an effect on developing functional skills. Electronic arm prostheses or hydraulic knee joints consonant with the child's level and age will support the child in daily living.4,5

The reason for amputation and accompanying systemic diseases will affect the rehabilitation process. Thus, during prosthetic fabrication and training, a history of the underlying problem should be carefully taken while considering the findings of the evaluations, and the program should be determined according to these results. If the reason of an amputation is a circulation problem, care should be taken to determine sensory losses, pain, and fatigue. Then referral to physiotherapy approaches such as electrotherapy applications, connective tissue manipulations, and desensitization training may be indicated.4,6

There are various reasons for an amputation. Sometimes a systemic disease such as chicken pox or a neurological disease such as spina bifida and sometimes a rare neurocutaneous disease such as Klippel–Trenaunay–Weber (KTW) syndrome can lead to amputation. KTW is a rare syndrome characterized with hemangioma resembling wine stains, varicose veins, and hypertrophic soft tissue and bones. Moreover, finger malformations can develop. Although cases with localized symptoms have better prognosis, there is insufficient information about cases with extensive symptoms. Cases without cerebral hemangiomas have a better chance of survival and a higher level of mental status.712 In the literature, there are several cases with amputation caused by KTW syndrome.13,14

Back to Top | Article Outline

CASE REPORT

A 16-year-old male student with a left hip disarticulation came to Hacettepe University School of Physical Therapy and Rehabilitation Prosthetics and Biomechanics Department for his first prosthetic fitting on April 4, 2006.

His medical file and the assessments conducted showed that he was amputated at hip level, with a diagnosis of KTW syndrome (Figure 1). He was his parents' second child born normally without any complications on April 4, 1990. However, his left lower limb appeared to be hypertrophic when compared with his right side. When he was 6 months old, he was brought to Hacettepe University Hospital with complaints of swelling and abnormal appearance of toes because of the swelling. Examination showed hemangiomas on his left hypertrophic limb and on his left gluteal region. His left lower limb was measured at 41 cm and his right was 36 cm; there were no bone malformations. After a number of detailed evaluations, he was diagnosed with KTW syndrome with a 40 × 40-cm tumor in the left lower limb. Afterward, a left hip disarticulation was planned for the patient.

Figure 1
Figure 1
Image Tools

The amputee was evaluated by different departments including pediatric oncology, pediatric surgery, and dermatology. At the age of 3, he underwent the planned amputation, conducted by orthopedic surgeons. The patient was referred to Hacettepe University School of Physical Therapy and Rehabilitation, Prosthetics and Biomechanics Department, after the surgery. He was given a home program consisting of exercises and was told to come back for follow-up and a prosthetic fit when the open surgical wounds had healed. However, he was not able to come back until he was 16 years old because of temporary healing wounds, effluences, and pulmonary and urinary infections. In his last medical follow-up, his wounds were found to be healed, and again he was referred to our department.

In our examinations, it was observed that there were no wounds at the distal part of the residual limb, but there was eczema in some regions (Figure 2). He had no phantom pain/feeling or any limitation concerning the sound side joint ranges; however, hamstring and gastrosoleus muscle groups were short, abductors and adductors were 3, and the upper limb muscle strength was between 4 and 5, according to the Dr. Lovett muscle test.15 Sensory evaluation of the residual limb revealed hyperesthesia on the anterior distal part.

Figure 2
Figure 2
Image Tools

He had been an amputee for 13 years and was used to walk with bilateral crutches. In the posture analysis, right thoracic “C” scoliosis was determined, and, in the authors' opinion, the reason for the scoliosis is excessive weightbearing through the sound lower limb. The rigidity test showed that the curve is a structural one, as it is not diminished during forward flexion of the trunk. Stretching and strengthening exercises, balancing and weightbearing exercises, and residual limb dynamic and isometric exercises were given to prepare the amputee for gait. Strengthening exercises combined with respiration in cat–camel position, upper and lower limb stretching exercises in crawling position, and lateral flexion exercises were applied. Using special monofilaments, sensory education was applied for his scoliosis. The muscle strength of shoulder depressors (m. latissimus dorsi, m. trapezius, and m. pectoralis), adductors (m. pectoralis major and m. latissimus dorsi), shoulder flexors (m. deltoideus), extensors of elbow (m. triceps and m. anconeus), wrist extensors (m. extensor carpi radialis and m. extensor carpi ulnaris), and finger flexors are found to be 4 and 4+. A strengthening program was planned to strengthen the muscles that are important in three-point gait using crutches by a progressive resistive exercise regime after determining 10 maximum method.16 As the program was continuing, the measurements for the prosthesis were taken, whereas the residual limb was covered with a stretch film in order to provide sterilization to prevent bleeding of the eczema and to decrease infection risk.

To prevent bleeding of the residual limb areas with hemangioma and eczema, the inside of the socket was covered with Plastazote and then the residual limb-socket fit was initiated with short-duration weightbearing to avoid any irritation on the residual limb. The weightbearing period was gradually increased within the assessment framework of weightbearing tolerance.

When no complications were observed, the lower connections of the prosthesis were completed. Then prosthetic training began with a prosthesis consisted of a free hip and knee joint and a dynamic foot.

During the 3 weeks of training, the patient was trained in free walking, forward-backward-side stepping, squatting, sitting up and down on a chair, maintaining static and dynamic balance, and weightbearing on the prosthetic side (Figure 3). The amputee was first trained to walk in the parallel bars and then outside the bars with verbal stimulation. In addition, at the end of a 2-week period, he walked on the Gait Trainer and was evaluated before and after the training (Figure 4).

Figure 3
Figure 3
Image Tools

The amputee was able to walk with the prosthesis without bilateral crutches at the end of a 2-week period. He was trained for ambulatory activities including ascending/descending stairs, inclined walking, and getting in and out of a car. All assessments were repeated after the training.

Figure 4
Figure 4
Image Tools

According to the footprint method, right and left step lengths were 31 and 26.5 cm and foot angles were 5° and 12°, respectively. Double-step length was 57.5 cm, step width was 21 cm, and cadence was recorded as 90 steps/minute. In the second evaluation by the footprint method, right and left step lengths were 45 and 42 cm and foot angles were 7° and 15°, respectively. Double-step length was 87 cm, step width was 15 cm, and cadence was recorded as 105 steps/minute. Weightbearing percentage evaluated by Laser Assisted Static Alignment Reference (LASAR) Posture was found to be 42% on the amputated side in the first evaluation and 45% in the second evaluation (Table 1).

Table 1
Table 1
Image Tools

In the 6-minute walk test conducted with the help of the Gait Trainer, right and left step lengths were 51 and 45 cm, respectively; time on the right foot was 56%; time on the left foot was 44%; and ambulation index score was 55. In the second evaluations performed 1 week later, right and left step lengths were found to be 58 and 53 cm, respectively, time on the right side was 51% and time on the left side was 49%, ambulation index score was 62 (Table 2).

Table 2
Table 2
Image Tools

On the completion of a 3-week period of training, a Nottingham Health Profile (NHP) questionnaire was completed by the patient; the section scores recorded were as follows: energy level score was 24, pain score was 9.99, emotional reaction score was 60.99, social isolation score was 44.54, sleep score was 77.63, and physical activity score and mobility scores were 12.69. The total score was 229.84 (Table 3).

Table 3
Table 3
Image Tools

According to the Medicare Classification Functional Level (MCFL) classification system, the amputee was determined to be included in functional level 3 and his functional status was determined to be “amputee having skill or potential to move or walk in various speeds” after completion of all the evaluations and training. When the patient was asked to rate his overall satisfaction with the prosthesis on the visual analog scale (VAS), he marked 10 indicating “excellent satisfaction.”

Back to Top | Article Outline

DISCUSSION

The amputee who had never known for 16 years that he could use a prosthesis decided to be fitted with a prosthesis during his last follow-up with his medical doctor.

Considering the low emotional and social isolation section score that he got from the NHP, it can be said that he was running away from his social roles and isolating himself from the community.

In the first visit to the Prosthetics-Orthotics Department, the authors thought that there would probably be difficulty in fitting this amputee with a classical prosthesis because of his serious cutaneal problems arising from vascular insufficiency. To overcome the problem, the inside of the socket was covered with Plastazote to attain a comfortable contact area, and the residual limb-socket fitting was achieved in a shorter period than expected. A researcher had previously stated that trophic changes and bleeding would occur on the residual limb. It was also determined that edema was produced on the buttock area in higher amputation levels.13,14 We did not experience such problems during the socket-residual limb fit and prosthetic training period.

When the amputee's gait was assessed by the Gait Trainer and footprint, improvements were especially found in the aspect of step inequality between the first and second evaluations. According to the ambulation index that is between 0 and 100 score, the patient's second score was found to be better (62) than the first score (55). It was observed that on the Gait Trainer, with the utilization of visual and audio feedback and by being able to hold the bars of the Gait Trainer, which created a feeling of safety for the patient, the amputee was much more motivated, and his gait was closer to a normal pattern (Biodex Gait Trainer).17

When the patient became aware of the fact that he could manage the activities of daily living including walking, ascending and descending stairs, inclined walking, crossing obstacles, sitting up/down on a chair with his prosthesis, he accepted his prosthesis easily and rated his overall satisfaction on VAS as 10 indicating very much satisfied.

The amputee's gait was assessed to be nearly normal without the usage of assistive devices, and the success in managing activities can be explained with Physical Activity and Mobility Section scores of the NHP. We believe that with the increase of his prosthesis usage in social and daily life, his scores on the sections of Emotional Reaction, Pain, Sleep and Energy will increase, resulting in a higher overall score.18,19

The patient is included in functional level 3 according to the MCFL classification. On his discharge, he was informed that he can play table tennis with his prosthesis. We believe that when he has the opportunity to play table tennis, he will see that there are many more roles and activities he can perform, and that his self-confidence will increase and his personality will become much more extroverted. In this way, his quality of life will improve, placing him in functional level 4.20

The amputee was discharged, with his follow-up control to be conducted 3 months later. He now has a prosthesis suitable to his disease, age, and level of amputation; he has a fairly good functional status and an increased quality of life with the opportunity of becoming a more active and social individual.

Although he has a high level of amputation—hip disarticulation—and KTW syndrome, he increased his functional abilities and developed a nearly normal gait pattern in 3 weeks. Also having an important effect on the patient's successful rehabilitation were his youth and effort, his family's support, good prosthetic fit, and a physiotherapy program including different kinds of exercises and prosthetic training, such as walking, balance, and weightbearing on the prosthetic side.

Back to Top | Article Outline

CONCLUSIONS

The acceptance and effective utilization of a child's prosthesis can only be achieved by physiotherapy approaches, fabricating a prosthesis that provides the comfort and functionality to replace his amputated limb, and by helping him to realize that prosthetic training and rehabilitation can enable him to gain desired abilities.

Helping an amputee child to achieve a productive, satisfying life in society is a hard and lengthy process, but it is not impossible. Eliminating obstacles with the help of prosthetic applications, physiotherapy, and rehabilitation will ensure that the child becomes an independent individual able to undertake an active role in the community.

Back to Top | Article Outline
ACKNOWLEDGMENTS

A written consent of the patient was obtained for publication of his case report.

Back to Top | Article Outline

REFERENCES

1. Banasik JL. Genetic and developmental disorders. In: Lee-Ellen C, ed. Prespectives on Pathophysiology. Philadelphia, PA: WB Saunders; 1994.

2. Larson JP. Congenital amputation. In: Gale Encylopedia of Medicine; 2003.

3. Donald RC, Kapp SI. Lower limb pediatrics prosthetics: general considerations and philosophy. JACPOC 1992;4:196–206.

4. Şener G, Yiğiter K, Bayar K, Erbahçeci F. Effectiveness of prosthetic rehabilitation of children with limb deficiencies present at birth. Prosthet Orthot Int 1999;23:130–134.

5. Yiğiter K, Ülger Ö, Şener G, et al.. Demography and function of children with limb loss. Prosthet Orthot Int 2005;2:131–138.

6. Jain S. Rehabilitation in limb deficiency. II. The pediatric amputee. Arch Phys Med Rehabil 1996;77:9–13.

7. Hızarcıioğlu M, Gülez P, Ütük EB, et al.. Klippel– Trenaunay–Weber Sendromu: bir olgu sunumu ve literatür taraması. T Klin Pediatr 2003;12:93–96.

8. Anlar B, Yalaz K, Erzen C. Klippel–Trenaunay–Weber Syndrome: a case with cerebral and cerebellar hemihypertrophy. Neuroradiology 1988;30:360.

9. Lorda-Sanchez I, Prieto L, Rodriguez-Pinilla E, Martinez-Frias ML. Increased parental age and number of pregnancies in Klippel–Trenaunay–Weber syndrome. Ann Hum Genet 1998;62:235–239.

10. Meine JG, Schwartz RA, Janniger CK. Kilppel Trenaunay Weber Syndrome: Cutis 1997;60:127–132.

11. Darwish K, Bleau BL. Extensive small bowel varices as a cause of severe anemia in Klippel–Trenaunay syndrome. Am J Gastroenterol 1998;93:2274–2275.

12. Castilla-Guerra L, Fernandez-Moreno MC, Franco E. Sturge-Weber syndrome: a rare cause of gastrointestinal hemorrhage. Case reports. J Clin Gastroenterol 2000;1:89–90.

13. Sooriakumaran S, Landham TL. The Klippel–Trenaunay syndrome. J Bone Joint Surg Br 1991;73:169–170.

14. Paudel B, Shrestha BK, Banskota AK. Two faces of major lower limb amputations. Kathmandu Univ Med J 2005;3:212–216.

15. Kendall HO, Kendall FP, Wadsworth G. Muscles: Testing and Function. 1st ed. Baltimore, MD: Williams and Wilkins; 1949.

16. De Lorme T, Watkins A. Techniques of progressive resistive exercises. Arch Phys Med Rehabil 1948;29:263.

17. Whittle MW., Amputee Gait. Gait Analysis: An Introduction. 3rd ed. Oxford, UK: Butterworth-Heinemann; 2002:115–119.

18. Hunt SM, McKenza SP, McEwen J, et al.. Aquantitative approach to perceived health status: a validation study. J Epidemiol Commun Health 1980;30:829–835.

19. Demet K, Martinet N, Guillemin F, et al.. Health related quality of life and related factors in 539 persons with amputation of upper and lower limb. Disabil Rehabil 2003;25:480–486.

20. Gailey RS, Roach KE, Applegate EB, et al.. The amputee mobility predictor: an instrument to assess determinants of the lower limb amputees' ability to ambulate. Arch Phys Med Rehabil 2002;83:613–627.

KEY INDEXING TERMS: Klippel–Trenaunay–Weber syndrome; prosthetic rehabilitation; amputee

© 2012 American Academy of Orthotists & Prosthetists

Login

Search for Similar Articles
You may search for similar articles that contain these same keywords or you may modify the keyword list to augment your search.