Reconstructive By
David
B. Huang,
Mark
Surgery in Children After Meningococcal Purpura Fulminans Price,
Jeff
Pokorny,
Keith
St. Louis,
Background/Purpose: Purpura fulminans (PF) is a serious, often life-threatening disease. As more children are surviving their disease, surgeons are presented with increasing numbers of multiple and complicated wounds as sequelae of PF. The purpose of this paper is to review the management of nine cases of PF, and present the reconstruction method in treating bilateral exposed elbow and knee joints. Methods: All cases of pediatric patients by the division of plastic and reconstructive 1986 and 1998 were reviewed.
with
PF and treated surgery between
Resulta: Seven children (78%) had meningococcal PF, and one (11%) had PF after Haemophilus influenza septicemia. PF developed in one (11%) but with no growth in either blood or cerebrospinal fluid cultures. Five children (56%) required amputation procedures. Two children (22%) required knee disarticulation. Two patients (22%) had free myocutaneous
H
ENOCH’ FIRST APPLIED the term purpuuuf~lminuns (PF) in 1887 to describe a syndrome characterized by rapid onset of hemorrhagic skin lesions with formation of bullae in the area of hemorrhage and rapid progression to death with negative autopsy findings.* Cutaneous hemorrhage typically occurs in distal extremities and tends to be symmetrical with progression to skin necrosis and extremity gangrene.3,4 Other clinical features are (1) fever, (2) septicemia, (3) shock, and (4) disseminated intravascular coagulation (DIC).4 Although occurring in only 10% to 12%5-8 of meningococcal infection, meningococcal PF is a rare disease of childhood with high morbidity and mortality rates. The previously reported mortality rate ranged between 40% and 8O%.5 The most recent series3,4,6reported decreased mortality rate to 18% to 30% with improvement in early diagnosis and critical care. More and more surgical specialists are presented with the permanent musculoskeleta1 sequelae of those who survived their disease. The purpose of this paper is to review our management of nine pediatric cases of PF at our institution over the last 12 years, and present our reconstruction method in treating a 3%-year-old girl with bilateral exposed elbow and knee joints. To our knowledge, there have been no reported cases in the literature describing soft tissue coverage for bilateral exposed elbow and knee joints in children with PF.
Journal
ofpediatric
Surgery,
Vol34,
No 4 (April),
1999: pp 595-601
R. Gabriel,
Robert
Lynch,
and
Christian
E. Paletta
Missouri
flap transfers the face.
for bone
coverage.
One
(11%)
had PF involving
Conclusions: Meningococcal PF is a rare, often life-threatening disease generally of childhood. More children are surviving their diseases but with devastating sequelae. Successful reconstructive treatment outcome of these children requires a multidisciplinary team approach involving multiple specialties. The goal is to preserve function, maintain maximal length, and salvage limbs when possible. Flexibility and innovation are necessary in treating these multiple and complicated wounds. J Pediatr Surg 34:595-601. Copyright o 7999 by W.B. Saunders Company. INDEX WORDS: Meningococcemia, purpura nal oblique myocutaneous pedicle flap, myocutaneous free flap.
MATERIALS
AND
fulminans, latissimus
exterdorsi
METHODS
This study involved a retrospective chart review of all pediatric patients admitted to Cardinal Glennon Children’s hospital with the diagnosis of PF between 1986 and 1998. Diagnosis of PF was made based on the characteristic purpuric lesions (Fig 1) found on physical examination and the typical clinical presentations of fever, septicemia, shock, and coagulation disorder. All children were treated initially by the pediatric critical care service. Wound debridement, amputation, and reconstructive surgery were performed by the plastic surgery service in conjunction with the orthopedic surgery service as soon as the patients were medically stable. None of the children in this study received heparin infusion. RESULTS
There were nine cases of PF treated by the division of plastic and reconstructive surgery between 1986 and 1998 (Table 1). Their ages ranged from 5 months to 11 years old with an average age of 32 months. There were five boys and four girls. Seven children (78%) had From the Division of Plastic and Reconstructive Surgery, Department of Orthopedic Surgery, and Department of Pediatrics at Saint Louis Universit?, School of Medicine and Cardinal Glennon Children’s Hospital, St Louis, MO. Address reprint requests to Christian E. Paletta, MD, Division of Plastic and Reconstructive Surgery St Louis University Health Sciences Center; 3635 vista Ave at Grand Blvd, PO Box 15250, St Louis, MO 63110-0250. Copyright 0 1999 by WB. Saunders Company 0022.3468/99/3404-0018$03.00/O
595
HUANG
ET AL
Y P Fig 1. Characteristic purpuric lesions distal extremities. (B) Typical predilection
in a 2%year-old child with purpura for distal extremity involvement.
meningococcal PF, and one (11%) had PF after HaemophiEusinfluenza septicemia. PF developed in one (1 l%), but his blood and cerebral spinal fluid cultures showed no growth. Table 1. Clinical Patient No.
Age
SF2
1
19 mo
M
2 3
9mo 18 mo
M M
Summary
fulminans.
of Nine Patients
11 yr 19 mo
M M
With
Purpura
lesions
involving
the trunk
Fulminans Surgical Procedures
Etiology
N meningitidis Negative cultures N meningifidis
purpuric
group
C
N meningifidis H influenza Type B
Debridement and local wound Debridement and local wound Right below-knee-amputation, debridement
Debridement Transmetatarsal
care care transmetatarsal
and STSG
and STSG amputation
amputation
left
BLE
both feet, debridement
and primary
7
2%yr
F
N meningitidis
CW-135
wound closure Fasciotomies BLE, RUE; disarticulation BLE; amputation right midforearm; STSG BLE, RUE; debridement nose and upper lip Amputation left great toe, debridement left heel, latissimus dorsi
8
3% yr
F
N meningitidis
CW-135
free flap with STSG Bilateral knee disatticulation;
Group-C
knee; right latissimus dorsi free flap to left knee; debridement right hand, amputation right long finger; external oblique myocutaneous flap to bilateral elbow with external fixators Debridement both knee, left lower leg, left shoulder
6
9 Abbreviations:
13 mo
5mo RUE, right
F
N meningitidis
F upper
extremity;
N meningitidis ELE, bilateral
lower
extremity;
and
Five of nine children (56%) required various types of amputation to their distal extremities. Two required transmetatarsal foot amputations, one had a below-knee amputation, one required amputation of her midforearm,
foot, 4 5
(A) Symmetrical
STSG,
split-thickness
left latissimus
skin graft.
dorsi
free flap to right
PLASTIC
SURGERY
IN PURPURA
597
FULMINANS
and one had an amputation of her great toe. Two children (22%) required knee disarticulation, and two patients (22%) had free myocutaneous flap transfers for bone coverage. One (11%) had PF involving the face. Case Report A 3X-year-old white girl was admitted to an outlying hospital on May 23, 1995 with a l-day history of fever, vomiting, and diarrhea. The patient became increasingly lethargic and a rash developed on her buttocks. She was transferred to our institution with a presumed diagnosis of meningococcemia. On admission, she had purpuric lesions involving her trunk and extremities and was in septic shock. She required endotracheal intubation for ventilatory support and aggressive fluid resuscitation. Broad-spectrum antibiotics were given initially and later changed to penicillin when blood cultures returned with growth of Neisseria Meningitidies, group CW- 135. Ten days later, she was taken to the operating room for debridement after her clinical condition had stabilized, and the purpuric lesions on all four extremities had clearly demarcated (Fig 2). At the time of surgical exploration, muscles in all four compartments of both lower legs were noted to be necrotic, and bilateral knee disarticulations were performed. Her upper extremities showed variable degrees of distal finger necrosis, and both elbows showed signs of full-thickness necrosis. No attempts at closure were performed at that time. All open wounds were treated with wet-to-dry dressing changes. Approximately 2 weeks after the bilateral knee disarticulations, a left latissimus dorsi myocutaneous free flap was performed to cover her right knee disarticulation site. End-to-side arterial and venous anastomoses between the popliteal and the thoracodorsal vessels were performed with interrupted 9-O nylon sutures. Before the anastomoses,the vessel walls were examined for microthrombi and
Fig 2. ties with
Purpuric lesions involving bilateral upper and lower extremiclear demarcations between viable and nonviable tissue.
Fig 3. Bilateral exposed elbow joint. (B) Right elbow
elbow joint.
joints
after
debridement.
(AI Left
intimal injuries. Continuous systemic infusion of dextran (LMD4J was initiated at the completion of the anastomoses and continued for a total of 5 days. The dextran was infused at 7 ml/kg/d for 3 days and decreased to 3 ml/kg/d for the last 2 days. No systemic heparin or aspirin was given. On postoperative day 3, vascular compromise of the free flap was noted, and she was immediately taken back to the operating room for exploration. The anastomoses were found to be intact; however, there was no pulsatile arterial flow. Despite attempts at thrombectomy, arterial flow could not be reestablished into the flap, and it was subsequently removed. The wound was treated with wet-to-dry dressing changes. Four days after removal of the failed flap, a right latissimus dorsi myocutaneous free flap was transferred successfully to cover her left knee disarticulation site. Her right distal thigh wound was closed with local flaps after shortening of the distal femur. Two weeks later, she underwent further debridement of her exposed elbow joints (Fig 3). These areas were covered using laterally based bilateral external oblique myocutaneous flaps transferred as pedicle flaps (Fig 4). The forearms were immobilized for 3 weeks by securing to the iliac crests with external fixators. She also underwent debridement of her right hand and amputation of her right long finger at the PIP (proximal interphalangeal) level. The external oblique myocutaneous flaps were divided partially 10 days later and completely divided at 3 weeks. The donor sites were covered with split-thickness skin grafts. She
598
Fig 4. Design of laterally based external oblique myocutaneous pedicleflaps 18 x 10 cm each) for bilateral elbow joints coverage.
was discharged home for further recovery and rehabilitation 21/2months after her admission. During her recent ?-year follow-up visit, the child appeared well both physically and psychologically. The latissimus dorsi myocutaneous free flap to her left knee remained viable. Both her elbow joints were well covered and had good range of motion. The skin grafts to the donor sites on her abdomen and right knee remained soft without signs of contractures. She had been receiving physical therapy and gait training at a local Shriners Hospital for Children. She was able to ambulate with a walker and have full weight bearing on her prosthesis with minimal impairment in her upper extremity function. Pathophysiology Hjort et al9 reviewed 50 reported cases of PF before 1964 and provided the following characteristics of PF: (1) it is a rare disease of children; (2) it almost always follows a “preparatory disease,” usually a benign infectious event either bacterial or viral; (3) after a latent period, bleeding into the skin develops and progresses rapidly resulting in anemia and hemorrhagic shock; (4) the most involved area undergoes skin necrosis with bullae formation; (5) coagulation abnormalities compatible with DIC are demonstrable; and (6) pathological findings include widespread thrombosis of venules and capillaries of the affected sites. In 1982, Chu and Blaisdel14 reviewed 68 cases of PF since 1960 and differentiated PF into acute and chronic states. In the acute state, PF immediately follows an acute bacterial infection such as meningococcemia. In the chronic state, PF is preceded by a benign disorder such as a throat infection or a viral exanthem. Meningococcus is the most common bacterial organism identified in the acute state, whereas varicella is the most common viral agent found in the chronic state. Since the report of Hjort et al9 in 1964, Spicer3 and
HUANG
ET AL
others2g4*‘OJ1 have reported cases of PF in adults. However, meningococcal PF remains a rare disease generally found in children. It has been postulated that congenital and acquired deficiency in protein C and S may contribute to the development of PF in children.11-13There have been reported cases of infants born with homozygous protein C deficiency in whom PF developed shortly after birth.14J5 Powars et a112J3and others16have demonstrated decreased levels of protein C and S in children with severe PF, especially meningococcal PF. In addition, nonsurviving patients have lower levels of protein C than surviving patients.12 Furthermore, children younger than 4 years of age may have a greater risk for PF because their natural levels of prothrombin and protein C are lower than those of adults.“J7 Cutaneous manifestation of PF has been postulated as the result of endotoxin-induced DIC, which leads to hemorrhage and intravascular thrombosis in the venules and capillaries.4,9J1 Despite various experimental work and clinical studies, the exact mechanism of skin necrosis remains unclear. One of the proposed mechanisms can be demonstrated through a local Shwartzman reaction produced in experimental animals. The skin of a rabbit is injected initially with an endotoxin to simulate the “preparatory disease” in PF and intravenously 24 hours later to elicit the characteristic hemorrhagic necrosis as noted in PF.‘* Endotoxin coupled with direct bacterial action on the endothelial cells was thought to result in extensive damage to the blood vessels and intravascular thrombosis.4*s Bhullar and Hansbrough” and Powars et all3 speculated that the cutaneous manifestation of PF is a result of declining levels of protein C and S, creating a hypercoagulable state and DIC and leading to microvascular thrombosis and ischemic skin necrosis. Recently, Harris and Gosh19suggested that endotoxin released from the meningococcal cell wall initiates the release of a large amount of vasoactive cytokines such as interleukin-6 and interleukin- 1 leading to morbid outcome. DISCUSSION
With the improvement in early diagnosis and intensive care, more and more children with PF are surviving. Based on our experience and review of the literature, management of PF frequently involves a multidisciplinary team approach. The initial resuscitative phase typically involves pediatric critical care and infectious disease specialists. The later reconstructive-rehabilitative phase generally involves orthopedists, plastic surgeons, pediatricians, physiatrists, physical therapists, and psychologists. The initial resuscitative phase requires correction of the underlying infectious disease with appropriate antibiotics and treatment of shock with aggressive fluid resuscitation either in an intensive care unit or a bum unit.
PLASTIC
SURGERY
IN PURPURA
FULMINANS
Pulmonary artery catheterization is indicated if there is any question regarding adequacy of fluid resuscitation. Early use of cardiac inotropic agents is advocated.33 Alpha-adrenergic agents and peripheral vasoconstrictive drugs should be avoided. The use of heparin is controversial. In review of the literature, some investigators3~4~9~20 recommend heparin for all cases of PF. Others10J2s21 did not find heparin to be clinically helpful. Bhullar and Hansbrought’ argue that lack of randomized, prospective, controlled study and the risk of exacerbating bleeding discourage the use of heparin. When used, the recommended heparin dose is 100 to 150 U/kg every 6 hours and is administered as a continuous infusion for at least 1 to 2 weeks.2,3,20Cases of relapse have been reported when heparin is stopped after only a few days of infusion.3 At our institution, heparin is not used routinely for treatment of PF. We feel that the potential benefit gain is less than the risks and complications associated with systemic heparinization. Rivard et a123reported normalization of circulating protein C levels and rise in fibrinogen after administrating highly purified protein C concentrates in four patients. The investigators observed that two of four patients recovered completely with no sequelae. All four patients had prompt reversal of their organ dysfunction. They concluded that correction of plasma protein C level could improve the morbidity and mortality rates of patients with PF. However, the investigators noted that a double-blind, randomized, controlled multicenter trial study is needed before any final conclusions can be drawn. There are reported cases5+10*21.24 of using sympathetic blockade in treating PF-induced peripheral ischemia. The investigators noted improved circulation in the distal extremities with sympathetic blockade. This suggests that the initial pathophysiological mechanism for tissue ischemia in PF is vasospasm rather than vessel thrombosis.21 Other reported treatment regimens include tissue plasminogen activator, leeches, and steroids.4.25*26 If the child survives the acute phase of PF, the attention is turned to the debridement-reconstructive phase. The timing of surgical debridement is not defined clearly in the surgical literature. Some investigators8.“-22 recommend early aggressive surgical debridement of nonviable tissues and biological closure of all wounds to prevent or control potential wound sepsis. Others4,6,20J8advocate delayed debridement until a clear line of demarcation between viable and nonviable tissue is apparent. Although some investigators4 felt that compartment syndromes in PF are unlikely, most6,20,27recommend frequent monitoring of compartment pressures and performing fasciotomy as indicated. We prefer delaying surgical debridement until a clear demarcation between viable and nonviable tissue is apparent. However, prompt
599
surgical exploration is performed if there are any signs of wound infection or compartment syndrome. A close working relationship and clear communication between the orthopedic and plastic surgeons is essential in planning the most appropriate salvage and reconstructive procedures. Because distal femoral and proximal tibia1 epiphyses account for 60% to 70% of their respective bone growth,34s35 every effort should be made to preserve these epiphyses when performing amputation, especially in children. In our case report, we were able to preserve the distal femoral epiphysis by providing soft tissue coverage with free tissue transfer. This provides a chance for continued femur growth and maximal limb length for future prosthetic fitting and ambulation. An above-knee amputation in a child this age will severely limit her ability to ambulate because the amputated limb length remains the same while the rest of the child continues to grow. A potential long-term adverse orthopedic complication is the ischemic damage to the epiphysis resulting in “bone bridge” formation and premature epiphyseal fusion.29,30Significant limb shortening, angular deformity, osteophytes, and traumatic arthritis may develop in affected children, and they eventually may require revision amputation.8,32 Soft tissue coverage often depends on the extent of soft tissue necrosis. For small superficial skin necrosis, conservative treatment with debridement and local wound care usually will suffice. For more extensive skin necrosis, debridement with skin graft often is required. Adendorff et a12* reported “poor take” of their primary splitthickness skin graft on what appeared to be well-debrided wounds in patients with PF. They recommended obtaining quantitative assessment of the bacterial content before skin grafting and temporarily covering the wounds with either homografts or xenografts before skin grafting. Harris and Gosh,t9 recently reported their experience with the use of cultured epidermal autografts in two children with meningococcal PF. They noted that approximately 50% of the grafted skin “took.” Despite its poor take, the investigators felt that the use of cultured autografts provided stimulus for wound healing. Soft tissue coverage for amputated stump or exposed joints in PF poses a challenging problem for reconstructive surgeons. Local flaps typically either are not adequate in size to provide coverage, or not available because of the extent of surrounding soft tissue necrosis. Whenever possible, free tissue transfer is used to preserve function, maintain maximal bone length, and salvage limbs. In 1994, Herrera et al6 reported using free tissue transfer (temporoparietalis muscle flap) for limb salvage in two patients with PF. Recently, Yuen31 described the use of latissimus dorsi muscle free flaps for coverage of exposed knee joints after fulminant meningococcemia.
600
HUANG
ET AL
The loss of latissimus dorsi muscle function generally is well compensated by the remaining synergistic shoulder girdle muscles. 36,37However, sacrificing both latissimus dorsi muscles of a bilateral amputee may pose a significant decrease in the upper extremity and shoulder strength needed for self transport and ambulating with a wheelchair. Although the knee joints were not functional in the
subsequent functional loss associated with sacrificing both latissimus dorsi muscles. The reconstructive method used to cover bilateral elbow joints stemmed from our previous experiences in treating difficult lower extremity wounds in children.38*3g It demonstrates that flexibility and innovation are necessary in managing these multiple and complicated wounds in children with PF. Review of the literature and our own experience in
patient in our case report, we felt that by preserving the
managing a series of nine pediatric cases of purpura
distal femoral epiphyses, the child was given a chance for maximal femur growth to allow optimal prosthetic fitting and increased the likelihood of ambulating. In addition, the transferred myocutaneous flap would provide a much more durable coverage than epithelialized tissue or skin grafts over the amputation stumps. These potential benefits, in our opinion, outweigh the risks of flap failure and
fulminans illustrate three important points: (1) early multidisciplinary evaluation is essential for planning the most appropriate salvage and reconstructive procedures, (2) every effort should be made to maintain length when debriding or amputating digits or limbs, and (3) flexibility and innovation are necessary when confronted with these multiple and complicated wounds.
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deficiency in severe infectious purpura of children: A collaborative study of 40 cases. Int Care Med 18:1-4, 1992 17. Nardi M, Karpatkin M: Prothrombin and protein C in early childhood: Normal adult levels are not achieved until the fourth years of life. J Pediatr 109:843-845, 1986 18. Hjort PF, Rapaport SI: The Shwartzman reaction: Pathogenetic mechanisms and clinical manifestations. Ann Rev Med 16:135-168, 1965 19. Harris NJ, Gosh M: Skin and extremity loss in meningococcal septicaemia treated in a burn unit. Bums 20:471-472, 1994 20. Silbart S, Oppenheim W: Prupura fulminans: Medical, surgical and rehabilitative considerations. Clin Orthop Rel Res 193:206-213, 1985 21. Johansen K, Murphy T, Pavlin E, et al: Digital ischemia complicating pneumococcal sepsis: Reversal with sympathetic blockade.CritCareMed19:114-116,199l 22. Andendorff DJ, Lamont A, Davies D: Skin loss in meningococcal septicemia. Br J Plast Surg 33:251-255,198O 23. Rivard GE, David M, Farrell C, et al: Treatment of purpura fuhninans in meningococcemia with protein C concentrate. J Pediatr 126:646-652, 1995 24. Anderson CT, Berde CB, Sethna NF: Meningococcal purpura fulminans: Treatment of vascular insufficiency in a 2-yr-old child with lumbar epidural sympathetic blockade. Anesthesiology 71:463-464, 1989 25. Aiuto LT, Barone SR, Cohen PS, et al: Recombinant tissue plasminogen activator restores perfusion in meningococcal purpura fulminans. Crit Care Med 25:1079-1082,1997 26. De Chalain T, Cohen SR, Burstein FD: Successful use of leeches in the treatment of purpura fuhninans. Ann Plast Surg 35:300-304,1995 27. Jacobsen ST, Crawford AH: Amputation following meningococcerma. Clin Orthop Rel Res 185:214-219,1984 28. Cohen JR, Lackner R, Keller A, et al: The surgical implication of purpura fulminans. Ann Vast Surg 4:276-279, 1990 29. Patrquin HB, Travis A, Jecquire S, et al: Late sequelae of infantile meningococcemia in growing bones of children. Radiology 141:77-82, 1981 30. Robinow MA, Johnson GF, Nanagas MT, et al: Skeletal lesions following meningococcemia and disseminated intravascular coagulation. A recognizable skeletal dytrophy. Am J Dis Child 137:279-281, 1983
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31. Yuen J: Free-muscle-flap coverage of exposed knee joints following fulminant meningococcemia. Plast Reconstr Surg 99:880884,1997 32. Genoff M, Hoffer MM, Achauer B, et al: Extremity amputations in meningococcemia induced purpura fuhninans. Plast Reconstr Surg 89:878-881, 1992 33. Hinshaw LB: Myocardial function in endotoxin shock. Circ Shock Suppl 1:43-5 1,1979 34. Ogden JA: Current concepts review: The evaluation and treatment of partial physeal arrest. J Bone Joint Surg 69A:1297-1302. 1987 35. Sponseller P, Beaty JH: Distal femoral epiphyseal fractures, in
Rockwood CA, Wilkins KE, Beaty JH (eds): Fractures Philadelphia, PA, Lippincott-Raven, 1996, pp 1233-1263 36. Bostwick J, Nahai F, Wallace JG, et al: Sixty flaps. Plast Reconstr Surg 63:31-41, 1979 37. Mathes and technique.
SJ, Nahai F: Reconstructive New York, NY, Churchill
in Children. latissimus
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38. Paletta C, Bartell T, Shehadi S: Application flap. Ann Plast Surg 30:41-47, 1993
dorsi anatomy
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39. Paletta C, Campbell E, Shehadi S: Tissue expanders Pediatr Surg 26:22-25, 1991
thigh
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J