Journal of Infection (1992) 25, 197-2oo

CASE REPORT H a e m o p h i l u s influenzae type b purpura fulminans treated with hyperbaric oxygen S. Dollberg,*§ Z. Nachum,t A. Klar,* D. Engelhard,$ T. Ginat-Israeli,* H. Hurvitz,* Y. M e l a m e d t and D. Branski*

* Department of Paediatrics, Bikur Cholim Hospital, affiliated with the Hebrew University-Hadassah Medical School, Jerusalem, t The Israeli Naval Hyperbaric Institute, Haifa and 5;.Department of Clinical Microbiology, Hadassah Hospital, Jerusalem, Israel Accepted for publication 19 December 1991 Summary We report the case of a 4-month-old child with purpura fulminans caused by Haemophilus influenzae type b. In addition to conventional therapy, she was treated with hyperbaric oxygen, and made a full recovery. Hyperbaric oxygen as an adjunct to other therapy in purpura fulminans is discussed.

Introduction Purpura fulminans is a rare complication of bacterial or viral infections characterised by widespread haemorrhage into the skin, mucous membranes and, occasionally, the adrenal glands, disseminated intravascular coagulation (DIC) and shock. 1 It has been described following varicella, rubella, scarlet fever, meningococcaemia and Haemophilus influenzae infection. ~-~ One of the hallmarks o f this condition is ischaemic necrosis of the extremities caused by the occlusion of small blood vessels by microthrombi and by tissue oedema. 6 Antimicrobial therapy alone is often not successful in preventing the ischaemic necrosis and its severe and chronic complications. A recent report suggested that hyperbaric oxygen (HBO) therapy may prevent or reverse the ischaemic damage. 7 We report the case of an infant with Haemophilus influenzae type b septicaemia and meningitis, complicated by purpura fulminans, in whom treatment with hyperbaric oxygen was successful in preventing necrosis of the extremities.

Case report A 4-month-old previously well Arab female was admitted with a history of high fever for several hours, convulsions and skin lesions on her face and extremities. On examination, she was stuporous with tonic and clonic movements of the right half of her body. Rectal temperature was 35"5 °C and blood pressure was 90/50 m m H g . Respiratory rate was 4o/min and heart rate I r o / m i n . She had generalised hypotonia, more prominent on the right side; her left side was in a decerebrated posture with a localised reaction to painful ~Address correspondence to: Dr S. Dollberg, Department of Paediatrics, Bikur Cholim Hospital, 5 Strauss St, P.O. Box 492, Jerusalem 91oo4, Israel. oi63-4453/92/o5o197+o4 $o8.0o/o

© 1992 T h e British Society for the Study of Infection

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stimuli. Her anterior fontanelle was flat and there were no signs of meningeal irritation. Extensive p u r p u r a was present on the cheeks, nose and oral mucosa (Plate r). T h e skin of her fingertips and soles of her feet was purple-black and cold (Plate 2). Peripheral pulses were not palpable and the limbs were cold with poor capillary refill. T h e rest of the physical examination was normal. Laboratory tests revealed a leucocytosis of I9-2 × I09/1 with 63 % neutrophils, Io % band forms, 22 ~/o lymphocytes, and 5 % monocytes. T h e haemoglobin was 9'2 g / d l and the platelet count was 34 x tog/1. Blood urea was I7"3 mmol/1 and creatinine IO3 #mol/1. Blood gases showed metabolic acidosis (pH = 7" I9, base excess = - I 6 , P C O 2 = 26 m m H g , HCOz = I o m m o l / 1 ) . Chest X-ray showed bilateral patchy lung infiltrates. T h e C S F was under normal pressure using a 23 gauge spinal needle and had I36O x Io6/1WBCs of which more than 9 0 % were neutrophils. Glucose was not detected (serum glucose was 7.2 mmol/1 at that time) and protein was I'2 g/1. Gram-negative rods were seen in the stained C S F and these were identified as H. influenzae type b by a direct Latex agglutination test (Phadebact ® C S F test 20, Pharmacia Diagnostics AB, Uppsala, Sweden). Intravenous ampicillin 350 m g / k g / 2 4 h and cefotaxime 20o m g / k g / z 4 h and phenobarbital (one loading dose of 9o mg) were started. T h e patient was p u t in an oxygen hood. One hour after admission she developed hypotension of 3 o / I o m m H g and was treated with IV fluids and fresh-frozen plasma. A few hours later moderate gastric bleeding developed and packed red blood cells (I o ml/kg) and cimetidine were administered. Her p r o t h r o m b i n time then was 72 % of normal control. Abdominal ultrasound showed no evidence of adrenal haemorrhage. Blood and C S F cultures taken on admission grew H. influenzae type b biotype II (Indole production positive, urease activity positive, Ornithine decarboxylase activity negative). D u r i n g the following 3 days, the patient became more alert. T h e convulsions stopped and the decerebrated posture disappeared but she remained severely hypotonic. Her blood pressure remained about I o o / 6 o m m H g and she had good urine output. Rectal temperature rose to 39-4 r °C. Only minimal i m p r o v e m e n t in her skin lesions was observed. At that point the child was transferred to the Israeli Naval Hyperbaric Institute for hyperbaric oxygen therapy. Bilateral m y r i n g o t o m y was performed before H B O treatment in order to equilibrate the pressure in the middle ear to that of the ambient pressure. T h e patient received oxygen via a hood in a walk-in chamber pressurised to 2-2" 4 atmospheres. She had eight 90 min treatments in 6 days. An air break of 5 m i n was given in the middle of each session to prevent oxygen toxicity. D u r i n g the hyperbaric oxygen therapy the temperature difference of 2 °C measured between the lesions and the normal skin before the start of H B O therapy, disappeared. T h e blue-black skin lesions p r o m p t l y became red in the hyperbaric chamber and progressively improved from session to session. After completing the treatment only small necrotic areas at the tips of her toes had remained, otherwise the patient's general and neurological examination was normal (Plates 3 and 4). T w o days after completing the hyperbaric therapy, the patient developed right hemiparesis and generalised hyperreflexia. C T of the brain revealed a

Journal of Infection

Plates i and 2

Plate I. Face of the patient before treatment with hyperbaric oxygen.

Plate 2. Feet of the patient before treatment with hyperbaric oxygen.

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(Facing p. 198 )

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Plates 3 and 4

Plate 3- Face of the patient after treatment with hyperbaric oxygen.

Plate 4. Feet of the patient after treatment with hyperbaric oxygen.

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large fronto-temporal epidural abscess. After surgical drainage there was full resolution of the neurological signs. N o w aged I8 months, the patient is well with normal neurological examination and developmental assessment commensurate with her age. Discussion

T h e presumed pathophysiological process which contributes to purpura fulminans is a positive feedback mechanism which begins with occlusion of small blood vessels by microthrombi. This leads to acute peripheral ischaemia and bleeding into soft tissues due to necrosis of the small arterial walls. 1' 6.8-1~ Tissue oedema develops due to venule occlusion and endothelial ischaemia, which increases permeability with leakage of fluids and protein into the extravascular space. 13' 1~ This oedematous fluid forms a mechanical barrier, causing tissue hypoxia. Together with the bleeding, the oedema results in compartment syndrome, which further compromises perfusion, eventually leading to necrosis. Hyperbaric oxygen helps to break this vicious circle by overcoming the mechanical diffusion barrier and restoring tissue oxygenation, which in turn prevents necrosis and promotes healing. This is achieved by an increase in the partial pressure of oxygen within the small blood vessels, i.e. the arterial oxygen pressure obtained at 3 atmospheres can cause a IO-i 5-fold increase in the partial pressure of oxygen. This, in turn, brings about a three- to four-fold increase in the diffusion distance from capillaries to cells. 15 We have found only eight reports in the literature describing I I patients who were treated with HBO for purpura fulminans. 1~,~6-~ T h e clinical findings described during HBO therapy, including those of our patient, are disappearance of the temperature gradient between healthy and affected tissues, a colour change from blue to red, resolution of the oedema, the return of capillary filling and the reappearance of peripheral pulses. These indicate improved perfusion, in contrast to the vasoconstriction known to be induced by HBO in normal blood vessels. ~5 This phenomenon may be explained by the loss of autoregulation by the damaged blood vessels. A central point to be stressed is the importance of early HBO treatment, as the shorter the delay from diagnosis to HBO therapy, the better the outcome. However, despite delay and the fact that HBO-was only considered after failure of conventional therapy, 83 % of the patients, including ours, showed marked improvement or full recovery. T h e rarity of purpura fulminans and the diversity of its clinical picture make a prospective study of the benefit of HBO, impracticable. However, after considering the rationale for HBO, the lack of effective conventional treatment and the impressive results reported in the literature, including our report, purpura fulminans should be considered an indication for HBO therapy. (The authors are grateful to Melvin I. Marks M.D., Michal Rose M.D., Ms Varda Amir and Mr Richard Lincoln for their assistance in editing and typing the original manuscript.)

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References I. Hjort PF, Rapaport SI, Jorgensen I. Purpura fulminans--report of a case successfully treated with heparin and hydrocortisone. Review of 50 cases from the literature. Stand J Haematol 1964; I: I69-I92. 2. Becker FT, Buckley RP. Purpura fulminans associated with Varicella. Arch DermatoI I966; 94:613 -618. 3. Bouhasin JD. Purpura fulminans. Pediatrics I964; 34: 264-270. 4. Morse TS, Row MI, Hartiga M. Purpura fulminans. Arch Surg 1966; 93: 268-27o. 5. Santamaria JP, Kenney S, Stiles AD. Purpura fulminans associated with Haemophilus influenzae type b infection. N C M e d J I985; 46: 516-517. 6. Spicer TE, Ran JM. Purpura fulminans. Am J Med I976; 6I : 566-57I. 7. Kindwall EP, Goldman RW. Hyperbaric medicine procedures, purpurafulminans (secondary to meningococcus or pneumococcus). Milwaukee, WI: St Luke's Hospital, I984: I94-I95. 8. Leavell UW. Cutaneous changes in a case of purpura fulminans following pneumococcal pneumonia. J Ky Med Assoc I972; 7o: 853-856. 9. Dodge WF, Travis LB, Daeschner CW. Anaphylactoid purpura. Polyarteritis nodosa and purpura fulminans. Pediatr Clin North Am 1963; IO: 879-897. IO. Castleman B, McNeely BU. Case records of the Massachusetts General Hospital. N Engl J Med 1969; 281: 153-162. I I. Dudgeon DL, Kellogg DR, Gilchrist GS, Woolley MW. Purpura fulminans. Arch Surg 1971; lO3: 351-358. 12. Rainier-Pope CRC. Purpura fulminans and disseminated intravascular coagulation. A report of five cases in childhood. S Afr M e d J 1973; 47: 142o-1422. 13. Haimovici H. Myopathic-nephrotic-metabolic syndrome associated with massive acute arterial occlusions. J Cardiovasc Surg 1973; 14: 589-6o0. 14. Wang MCH, Reich T, Lesko WS, Jacobson JH. Hyperbaric oxygenation. Oxygen exchange in an acutely ischemic vascular bed. Surgery 1966; 59: 94-1Ol. 15. Committee on Hyperbaric Oxygenation. Fundamentals of Hyperbaric Medicine. (Publication number 1298; Library of Congress, Cat. No. 65.61928.) Washington DC: National Academy of Sciences--National Research Council, 1966: Chapter I, 3-1I, 12-2o, 33-35. 16. Karz SC, Thompson RE, Depenbusch FL, Hart GB. Hyperbaric oxygen treatment of gangrene in pneumococcemia, ff Am Med Assoc 1971; 217: 962-963. 17. Waddel WB, Saltzman HA, Fuson RL, Harris J. Purpura gangrenosa treated with hyperbaric oxygenation, ff Am Med Assoc 1965; 191: 971-974. 18. Monies-Chass I, Here D, Alon U, Birkhann HJ. Hyperbaric oxygen in acute ischaemia due to allergic vasculitis. Anaesthesia 1976; 31: 1221-1224. 19. Rosenthal E, Benderly A, Monies-Chass I, Fishman J, Levy J, Bialik V. Hyperbaric oxygen in peripheral ischemic lesions in infants. Arch Dis Child 1985 ; 6o: 372-374. 2o. Bland DK, Klooster MJ. Hypoglycaemia related to hyperbaric oxygen in Haemophilus influenzae purpura fulminans. J Hyperbaric Med 1988; 3: 65-71. 21. Kuzemko JA, Loder RE. Purpura fulminans treated with hyperbaric oxygen. Br Med J 197o; 4: 157.

Haemophilus influenzae type b purpura fulminans treated with hyperbaric oxygen.

We report the case of a 4-month-old child with purpura fulminans caused by Haemophilus influenzae type b. In addition to conventional therapy, she was...
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