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 Table of Contents  
ORIGINAL ARTICLE
Year : 2017  |  Volume : 3  |  Issue : 2  |  Page : 60-66

Amputation Following Meningococcal Septicaemia in Children: the Surgical Management of the Residual Limb


1 Medical Student, University of Bristol, England, United Kingdom
2 Consultant Orthopaedic Surgeon, Bristol Royal Hospital for Children, Bristol, England, United Kingdom

Date of Web Publication16-Jul-2018

Correspondence Address:
Fergal P Monsell
Bristol Royal Hospital for Children, Upper Maudlin St, Bristol BS2 8BJ, England
United Kingdom
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/pors.pors_9_17

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  Abstract 

Background: Meningococcal septicaemia is a potentially life-threatening disease and remains the most common infective cause of mortality in the UK. Improvements in healthcare have led to early recognition and treatment, and a decrease in mortality. As more children now survive the initial acute illness, the long-term musculoskeletal consequences have become more prevalent. These include growth plate injury, tissue loss and amputation. Patients with limb loss present specific difficulties due to the effect of remaining longitudinal growth on the function of the residual limb, and often require surgical treatment that continues throughout childhood. Patients and Methods: This case series reviews the histories of 13 children who underwent amputation as a complication of meningococcal septicaemia. All patients attend a specialist clinic and our experience in the management of the residual limb is described. Results: Thirteen patients, with a mean age of 16 months at the onset of meningococcal septicaemia, required amputation in the management of the skeletal consequences of the infection. Revision surgery was necessary for all 13 patients and involved management of bone overgrowth, growth arrest, scar and soft tissue contracture, neuroma development, and infection. The details of our approach to each of these complications is described. Conclusion: Due to improvements in diagnosis and initial management, a significant proportion of patients are surviving infantile meningococcal septicaemia. Many develop musculoskeletal consequences including amputation, and this case series serves to increase knowledge in the complex managements of the residual limb in these patients.

Keywords: Amputation, meningococcal septicaemia, residual limb


How to cite this article:
Aherne BM, Monsell FP. Amputation Following Meningococcal Septicaemia in Children: the Surgical Management of the Residual Limb. Paediatr Orthop Relat Sci 2017;3:60-6

How to cite this URL:
Aherne BM, Monsell FP. Amputation Following Meningococcal Septicaemia in Children: the Surgical Management of the Residual Limb. Paediatr Orthop Relat Sci [serial online] 2017 [cited 2018 Dec 11];3:60-6. Available from: http://www.pors.co.in/text.asp?2017/3/2/60/236715


  Introduction Top


Meningococcal septicaemia is a potentially life-threatening disease caused by  Neisseria More Details meningitides, a Gram-negative diplococcus, found only in human respiratory secretions.[1],[2],[3]

Meningococcal sepsis has a rapid onset and is characterised by fever, purpura and shock.[4],[5] The endotoxin initiates a complement cascade resulting in vasculitis, disseminated intravascular coagulation and end organ damage.[6]

It remains the most common infective cause of death in children,[1],[2] and mortality rates were previously as high as 20–50%.[4],[6] However, with improved healthcare leading to early recognition and treatment, mortality has decreased to 15–30%.[1],[2],[3],[4],[5]

Many children now survive the initial phase of shock and develop secondary problems due to peripheral ischaemia.[3],[5],[6],[7],[8] Growth plate injury often leads to limb length discrepancy and angular deformities, a common long-term musculoskeletal presentation in this patient population.[9]

In severe cases, children who survive meningococcal septicaemia may require amputation of affected limbs.[3],[5],[10]

Child amputees present specific problems due to the remaining longitudinal growth of the residual limb,[9] affecting function and use of prosthesis, and generally requires surgical treatment that continues throughout childhood.[9]

The aim of this case series was to review the case histories of children who required amputation as a complication of meningococcal septicaemia, focusing on the management of the residual limb.


  Patients and Methods Top


The records of patients attending a specialist clinic, providing ongoing care to children who have complications after septicaemic illness, were reviewed. The clinic consists of a highly skilled multi-disciplinary team including specialists in orthopaedic surgery, plastic surgery and allied health.

A total of 34 children were reviewed, 13 of which required amputation following meningococcal septicaemia. The medical records and radiographs were reviewed retrospectively, and data were collected on an Excel spreadsheet (Microsoft Corp., Seattle, Washington).

There were six boys and seven girls, who were aged between neonate and 4 years, with a mean age of 16 months, when they developed the acute phase of the meningococcal septicaemia, all requiring intensive care support [Figure 1].
Figure 1: Patient with meningococcal septicaemia in intensive care unit

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  Results Top


Clinical presentation

Thirteen patients, with a mean age of 16 months at the onset of meningococcal septicaemia, required amputation.

[Table 1] provides a summary of these cases, reporting amputation, subsequent management and complications including bone overgrowth, growth arrest, scar contracture, neuroma formation and soft tissue infection.
Table 1: Clinical details of 13 patients

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Limb involvement and amputation varied from four limb amputations in three patients to toe amputations in one case. There was also one case of toe auto-amputation, which was excluded, as it did not represent a surgical procedure.

[Table 2] and [Table 3] provide further details of the amputations. There were a total of 21 lower extremity amputations (limb, foot, toe) and 11 upper extremity amputations (limb, finger).
Table 2: Details of amputation

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Table 3: Details of level of amputations

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Management of the residual limb

Surgery may be necessary to manage bone overgrowth, growth arrest, neuroma formation, scar contracture and soft tissue optimisation, and was necessary in 12 of the 13 patients in this case series.

Bone overgrowth and growth arrest

Bone overgrowth occurred in 10 amputated limbs, seven lower limbs and three upper limbs. Growth arrest occurred in seven cases, all affecting residual lower limbs, and five patients developed both complications [Figure 2].
Figure 2: Bone overgrowth and growth disturbance

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Three patients required four-limb amputation, and all have required stump revision. Patient one had bilateral through knee amputations, left through elbow and right mid-humeral level amputations. This patient developed recurrent bone overgrowth of the right humeral stump, resulting in two revision procedures, 2 and 3 years after initial presentation.

Patient 3 had bilateral above knee amputations and bilateral proximal forearm amputations, and developed right femoral and ulnar overgrowth. Bilateral lower limb revisions were required 11 years after original amputation. Right femoral overgrowth and a neuroma affecting the left leg resulted in an inability to wear prosthetics.

Both patients with finger amputations (patients 12 and 13) required revision surgery for bone overgrowth, while the one patient (patient 11) with toe amputations did not.

Two cases of below knee amputations (BKAs) resulted in two different patterns of growth arrest. Patient 7 had bilateral BKAs after meningococcal septicaemia at 5 months of age. The patient developed right tibial growth arrest, requiring surgical intervention 2 years later. Initial management consisted of placement of an Ilizarov external fixator to lengthen by distraction osteogenesis. Recurrent deformity developed, and 2 years later, the patient underwent right tibial epiphysiodesis and limb lengthening, and re-alignment with an external fixator. Seven years later, 11 years after initial presentation, infection in the right residual limb required surgical incision and drainage and refashioning of the bony component. The patient also had ongoing scar contracture of both BKAs.

Patient 10 developed acute meningococcal infection as a neonate, which resulted in right BKA and developed a flexion contracture and partial distal femoral growth arrest. Nine years later, the patient required combined reconstruction with differential lengthening of the tibia, lengthening of the femur and soft tissue distraction to correct a fixed flexion deformity of the knee. Previous level of functional prosthetic use was achieved, and 3 years after reconstruction, the patient sustained a fracture of the right femur while skateboarding, requiring plate fixation.

Two other patients (patients 6 and 8) with growth arrest remain under regular review [[Figure 3] and [Figure 4]].
Figure 3: Pre- and post-TSF external fixator to correct flexor deformity and treat growth disturbance

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Figure 4: Taylor spatial frame (TSF) external fixator right lower limb and post-operatively wearing prosthesis

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Late surgical amputation

Growth arrest in the non-amputated lower limb of one patient (patient 5) resulted in complex residual deformity. Right tibial growth plate arrest and right fibular overgrowth resulted in varus malalignment leading to weight bearing on the lower end of the fibula and lateral border of the calcaneus. Three years after original presentation, the patient required right through knee amputation. Eight years later, the patient developed a painful peroneal neuroma and extensive scar contracture of the right lateral thigh. Revision surgery was required to excise the neuroma and release the scar contracture.

One case (patient 4) had right mid-tarsal spontaneous amputation soon after initial presentation. Despite two surgical debridements, a right BKA was required 1 year later. Soft tissue complications and growth dysfunction of the left lower limb resulted in a through knee amputation the following year. At a recent review, 9 years after the septicaemic illness, this patient presented with right fibular overgrowth, right proximal tibial and distal femoral growth arrest, and left femoral growth arrest.

Neuroma excision

Neuroma requiring surgical excision occurred in three cases (patients 3, 5 and 12). In all cases, this was a late complication. The sciatic nerve was involved in one case (patient 3), and the peroneal nerve in one case (patient 5), both which required surgery 11 years following amputation. Patient 12 required excision of a left middle finger neuroma 8 years after amputation [Figure 5].
Figure 5: Excised neuroma

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Scar contracture

Eight patients (patients 2, 3, 4, 5, 7, 8, 10 and 11) have required surgical interventions to manage scar contracture. Patient 5 required a left through knee amputation, a right BKA and a right hand partial amputation following the initial septicaemic illness. Z-plasty revision for upper limb scars was required 11 years later. The patient also required excision of a scar on the right lateral thigh 8 years after right BKA and conversion to through knee amputation.

Patient 7 required bilateral BKAs in the first year of life following the initial septicaemic illness. Scar contracture and growth arrest of the right lower limb required proximal tibial epiphysiodesis and soft tissue distraction with an Ilizarov external fixator.

Patient 8, aged 4 years at time of infection, required bilateral BKAs. At present, the left knee has a fixed flexion deformity of 45°, with soft tissue contracture and an adherent scar field. These soft tissue complications, accompanied with tibial growth arrest, are likely to require external fixator re-alignment management in the future.

Extensive operative interventions with multiple skin grafts have been required to manage scar and soft tissue contracture after a patellectomy (patient 11), and is discussed below.

Infection

Soft tissue infection of the residual limb occurred in three cases (patients 7, 8 and 11). One patient (patient 7) developed an infection after revision surgery, and one (patient 8) due to recurrent skin breakdown over a bony remnant. Patient 7 required surgical incision and drainage, and both patients required antibiotic treatment, resulting in resolution of infection.

Patient 11 was 3 years old at the time of infection. Surgical management involved left 2nd and 3rd toe amputations, and a patellectomy with a medial gastrocnemius flap to the right knee. The patient developed a chronic wound infection 6 years after the initial amputation. This has required multiple skin grafts, and remains under review.


  Discussion Top


Meningococcal septicaemia remains a major cause of morbidity and mortality.[11] Advances in disease recognition and resuscitation have led to improvements in survival, with development of significant long-term consequences of peripheral ischaemia.[3],[5],[6],[8]

The ongoing management involves the musculoskeletal consequences of the illness, with the aim of maintaining and optimising long-term function.[6]

In severe cases, irreversible necrosis of skin, muscle and bone can necessitate amputation.[12] The literature supports initially delaying amputation, as early surgery during the acute phase of infection, is not believed to alter the orthopaedic outcome.[5],[10] Importantly, with surgical delay, the loss of tissue may be less than what is predicted at the initial presentation.[5] Auto-amputation of the ischaemic area may also occur, negating the need for surgery.[9]

When amputation is necessary it is ideally performed as an elective procedure, with planning and input from orthopaedic surgeons, plastic surgeons and prosthetics services.[5],[9] Surgery aims to provide bony stability, good soft tissue and skin coverage, and a functional residual limb.[6] Amputation often proves technically difficult in this population, as the muscles may have an absent or severely compromised blood supply, with suboptimal soft tissue coverage, which is often also scarred.[9]

It is often necessary to perform repeated surgical procedures before skeletal maturity, and the family should be informed of this possibility at an early stage.[9]

The decision-making process is specifically based on improving function, irrespective of the condition of the limb. A variety of treatments are available and include extension of the amputation, resection of residual bone, epiphyseodesis with or without acute deformity correction, corrective osteotomy with segmental lengthening using distraction osteogenesis and soft tissue distraction for joint contractures.

In this case series, all but one patient required revision surgery, and details of management are provided in [Table 1]. The mean age at onset of infection was 16 months with upwards of 10 years longitudinal growth remaining.[9] The relative overgrowth of the bony components compared to the soft tissues during this period of remaining growth means that revision surgery is likely to be necessary and in this series occurred in seven lower limbs and three upper limbs.

It is common for an amputated limb to have associated ischaemic injury to remaining growth plates.[9] Growth arrest encountered in meningococcal septicaemia is more extensive than that resulting from other infective causes. The distal fibula is almost always spared with the distal tibia affected leading to progressive ankle varus.[4] It is also invariable to encounter growth arrest, whenever there is cutaneous scarring in the vicinity of a growth plate.

Nogi[13] reported on three cases where the amputated limb displayed physeal arrest. Wheeler et al.[14] reported the surgical interventions in 21 children following meningococcal purpura fulminans, with amputation performed in nine, two patients requiring revision. Grogan et al.[15] reported that in their series of nine children with BKAs, three knee disarticulations had been necessary.

In our series, growth arrest occurred in six cases, all lower limbs, of which three cases required further amputation. One patient required revised surgical amputation, converting a BKA to a through knee amputation as a consequence of femoral growth arrest. One case of auto-amputation required a higher-level amputation, due to growth arrest and soft tissue complications. The remaining patient required delayed amputation due to a combination of overgrowth and physeal arrest.

Nectoux et al.[10] reported that of seven patients with BKAs, six required further surgery to improve soft tissue coverage.

Scar contracture and soft tissue complications were an ongoing issue for our patient population, with numerous surgical interventions including scar excision, soft tissue revision surgery, Z-plasty procedures and skin grafts.Revision may also be necessary for the release of joint contractures and optimisation of soft tissue coverage.[9]

Neuroma development also required surgical excision in three cases in our series.

The management of the residual limb following meningococcal septicaemia is complex and requires a surgical approach to manage bone overgrowth, growth arrest, scar and soft tissue contracture, neuroma development and soft tissue infection.

As increasing numbers of patients are surviving neonatal meningococcal septicaemic illness, this will become an important part of the repertoire of surgeons involved in the long-term management of these patients.

This paper has attempted to provide an overview of the musculoskeletal consequences and describe potential solutions for their management.[9]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Paize F, Playfor SD. Improvements in the outcome of children with meningococcal disease. Crit Care 2007;11:172.  Back to cited text no. 1
    
2.
Maat M, Buysse CM, Emonts M, Spanjaard L, Joosten KF, De Groot R et al. Improved survival of children with sepsis and purpura: Effects of age, gender, and era. Crit Care 2007;11:R112.  Back to cited text no. 2
    
3.
Nectoux E, Mezel A, Raux S, Fron D, Maillet M, Herbaux B. Meningococcal purpura fulminans in children: I. Initial orthopedic management. J Child Orthop 2010;4:401-7.  Back to cited text no. 3
    
4.
de Kleijn ED, Hazelzet JA, Kornelisse RF, de Groot R. Pathophysiology of meningococcal sepsis in children. Eur J Pediatr 1998;157:869-80.  Back to cited text no. 4
    
5.
Davies MS, Nadel S, Habibi P, Levin M, Hunt DM. The orthopaedic management of peripheral ischaemia in meningococcal septicaemia in children. J Bone Joint Surg Br 2000;82:383-6.  Back to cited text no. 5
    
6.
Belthur MV, Bradish CE, Gibbons PJ. Late orthopaedic sequelae following meningococcal septicaemia. J Bone Joint Surg Br 2005;87:236-40.  Back to cited text no. 6
    
7.
Booy R, Habibi P, Nadel S, de Munter C, Britto J, Morrison A et al. Reduction in case fatality rate from meningococcal disease associated with improved healthcare delivery. Arch Dis Child 2001;85:386-90.  Back to cited text no. 7
    
8.
Monsell FP, McBride AR, Barnes JR, Kirubanandan R. Angular deformity of the ankle with sparing of the distal fibula following meningococcal septicaemia: A case series involving 14 ankles in ten children. J Bone Joint Surg Br 2011;93:1131-3.  Back to cited text no. 8
    
9.
Monsell F. The skeletal consequences of meningococcal septicaemia. Arch Dis Child 2012;97:539-44.  Back to cited text no. 9
    
10.
Nectoux E, Mezel A, Raux S, Fron D, Klein C, Herbaux B. Meningococcal purpura fulminans in children. II: Late orthopedic sequelae management. J Child Orthop 2010;4:409-16.  Back to cited text no. 10
    
11.
Park DH, Bradish CF. The management of the orthopaedic sequelae of meningococcal septicaemia: Patients treated to skeletal maturity. J Bone Joint Surg Br 2011;93:984-9.  Back to cited text no. 11
    
12.
Buysse CM, Oranje AP, Zuidema E, Hazelzet JA, Hop WC, Diepstraten AF et al. Long-term skin scarring and orthopaedic sequelae in survivors of meningococcal septic shock. Arch Dis Child 2009;94:381-6.  Back to cited text no. 12
    
13.
Nogi J. Physeal arrest in purpura fulminans − A report of 3 cases. J Bone Joint Surg Am 1989;71:929-31.  Back to cited text no. 13
    
14.
Wheeler JS, Anderson BJ, De Chalain TM. Surgical interventions in children with meningococcal purpura fulminans − A review of 117 procedures in 21 children. J Pediatr Surg 2003;38:597-603.  Back to cited text no. 14
    
15.
Grogan DP, Love SM, Ogden JA, Millar EA, Johnson LO. Chondro-osseous growth abnormalities after meningococcemia − A clinical and histopathological study. J Bone Joint Surg Am 1989;71:920-8.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]



 

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