Meningococcemia And Diarrhea With Gangrene
Richard E. King, M.D. Thomas W. Marks, M.D. Richard Canaan, M.D.
In a recent article on "The Child With an Acquired Amputation," Aitken1 reported that, in a series of 718 children, approximately 31 percent sustained their amputations as the result of a disease process. Malignant tumors were responsible for more than half of these disease-caused amputations, leaving some 15 percent of causative agents to fall in the general categories of "vascular and/or neurogenic lesions" and "miscellaneous."
One becomes aware that these broad classifications can include a number of situations where amputations have ensued from conditions so unusual that an individual clinic might only encounter one or two cases in a decade. This paper presents three patients whose amputations derived from gangrene of the extremities-in two instances as an aftermath of meningococcemia and the other following diarrhea-as examples of these uncommon instances.
Meningococcemia With Gangrene of the Extremities
Gangrene of the extremities, as a complication of meningococcal meningitis, is a rare occurrence; and even rarer is auto-amputation. Neither one of the two major pediatric textbooks mentions this entity6,7. In a 1917 article Elliott and Kaye4 mention a case that had associated frostbite with meningococcal infection, with resultant bilateral amputation of both feet above the malleoli, and another case is reported by Herrick5 in 1918. Since 1944 there have been only fifteen reported cases of gangrene of the extremities associated with meningococcemia 2,3,8,10,11, and only one definite case of auto-amputation8. There has been no mention of this complication in the English literature since 1958. Our two cases underscore the devastation caused by this little-known sequence of events.
Almost invariably the patients who develop this complication are very seriously ill, and probably would not have survived the disease in the preantibiotic era. There appears to be an orderly distribution of lesions3. In the total population, the feet are most commonly affected but, in infants, the buttocks are the most frequent site. As the patient gets older the hands are more likely to be affected. The toes were involved in all cases reported, with generally symmetrical lesions. In all patients the gangrene progressed relentlessly despite intensive treatment. The skin lesions that develop also follow somewhat of an evolutional pattern, the sequence typically being 2,3:
phase of appearance and generalization - 48 hours
phase of necrosis - four to five days
phase of separation of sloughs -two to four weeks
phase of repair - five to eight weeks
The pathology of these lesions is probably secondary to a combination of factors. There appears to be an overwhelming bacteremia and, if the organisms are in sufficient abundance, they may act as nuclei for the formation of thrombi in the small arterioles. The endothelial lining of arterioles may be damaged by the meningococci with subsequent necrosis, thrombosis and extravasation of the blood and fluid from an inflammatory reaction. Any factors which increase the agglutinability of the blood usually predispose the patient to intravascular thrombi. Events that cause sludging of the blood will probably lead to thrombi formation. Arteriolar spasm may also cause tissue damage. Tissue necrosis may be a reflection of shock. It is known that the meningococcus produces endotoxin and exotoxin that cause direct tissue damage. Meningococci endotoxin probably localizes in the endothelial cells causing inflammation, thrombosis and necrosis. These lesions bear a remarkable similarity pathologically to those of the Schwartzman phenomenon. Some investigators, in fact, feel that the Schwartzman phenomenon may play a part in the process of tissue damage2,3. It is not possible at this time to indict any of these factors separately or in combination as the cause of the gangrene although, if gangrene of the extremities is to be eliminated from future cases, the cause must first be understood. Patients with this disease should have the meningococcemia treated in the appropriate manner with special care taken to prevent extension of the gangrenous areas or formation of decubitus ulcers.
R.M., a 6 1/2-year-old colored male, was in good health until January 6, 1967, when he developed a high fever. He was seen at Phoebe Putney Memorial Hospital at Albany, Georgia, on January 7, 1967. He was noted to have high fever, nuchal rigidity, and ecchymotic areas on the feet and left hand. The admission diagnosis was meningococcal meningitis which was confirmed by examination of the spinal fluid. Initially he was very ill and comatose. He developed dry gangrene of both feet at the ankle level, and also of the fingers of the left hand. Intravenous medication was immediately initiated and three days after his admission the patient was mentally alert and has never shown any evidence of central nervous system damage. Before he was discharged, both of his feet and the distal phalanx of the fifth finger underwent auto-amputation.
He was admitted to the Scottish Rite Hospital for Crippled Children on February 13, 1967. On February 15, 1967, the amputations of both feet were revised. On March 8, 1967, he underwent further revision of the amputation of the left lower extremity, and removal of the gangrenous tips of the second, third, and fourth fingers (Fig. 1 A/B ). The left lower extremity required further revisions on March 29, 1967, and on April 19, 1967 (Fig. 1 C/D ). At the end of May 1967, he was fitted with bilateral patellar-tendon weight-bearing prostheses and did well. He has been admitted twice since the initial admission, once in December 1967, and again in July 1968; both times for adjustments to his prostheses.
C.W., a Negro female, was born July 23, 1961. On January 26, 1966, at the age of five years, she was admitted to Grady Memorial Hospital with a temperature of 105 degrees, general malaise and meningismus. Physical findings were compatible with the diagnosis of severe acute meningitis, and it was also noted that she had pustules on her thighs. (Culture of these lesions grew out meningococcus.) Immediate antibiotic therapy was started. The patient continued to be febrile with marked prostration, chills, and vomiting. Approximately 24 hours after admission, she developed areas of ecchymosis and purpura and became hypotensive. The ecchymosis and purpura were scattered over the body but were especially localized to the extremities, more so on the left than on the right side of the body.
A week after admission, the patient's general condition had improved. There were regions of frank tissue necrosis in all areas of the left upper extremity distal to the elbow; the left lower extremity distal to the mid-thigh; and below the knee on the right leg. By the second week of February, the skin was well demarcated. On the 16th of February, 1966, she had left above-knee and right knee-disarticulation amputations. On the 19th of February, she underwent surgery for a left below-elbow amputation. The postoperative course was complicated by bacteremia and infected stumps. Systemic and local antibiotics were continued. The patient's condition showed steady improvement and on May 4, 1966, she was discharged from Grady Memorial Hospital with minimal drainage from her stumps.
She was first seen at the Aidmore Amputee Clinic in July 1966. Physical examination at that time revealed a very short left below-elbow amputation with a scar contracture in the ante-cubital space. The stump was at 90 degrees of flexion with a range of motion of 30 degrees of flexion and 30 degrees of extension. Multiple areas of granulating tissue were present on the end of the right lower extremity stump. The left above-knee stump and the elbow were well healed with minimal scar tissue.
In August 1966, the patient was fitted with a leather knee-disarticulation socket, standard outside knee joints, a standard shin and Otto Bock SACH foot for the right lower extremity. On the left limb she was fitted with a total-contact quadrilateral suction socket, a single-axis knee, standard shin piece and Bock foot. The below-elbow stump was not fitted at this time.
Initially, the patient did well but, in December of 1966, she started to have trouble with the right knee-disarticulation stump and on the 31st of January, 1967, required a split-thickness skin graft in that area. In May, the patient again started wearing her prosthesis on the right limb and this time was fitted with a total-contact quadrilateral socket. In June, a pre-flexed forearm prosthesis was fitted to the left upper extremity and she has since been seen regularly in our clinic and is doing well (Fig. 2A , Fig. 2C ,Fig. 2B , Fig. 2 D ).
In a recent article Shehadi et al9 described six cases of gangrene of the lower extremities which occurred in infants following acute attacks of gastroenteritis. In one of these children the gangrene resulted in bilateral knee-disarticulations while a second child lost the third and fourth toes of the right foot only. The other four cases had gangrene of the skin of the lower extremities but no amputations.
Our case, G.F.J., was admitted to an outlying hospital at the age of two months with severe diarrhea and gangrene of the right and left hands. This condition eventually resulted in partial amputation of the fingers on the left side and a below-elbow amputation on the right. The child's hemoglobin electrophoresis was normal. The appearance of the child's limbs at the age of eight years is shown in Fig. 3 A/ B , Fig. 3 C/D .
Two cases of meningococcal meningitis complicated by partial auto-amputation of the extremities have been presented. The clinical course and possible pathological basis of the condition have been discussed.
An additional case of gangrene of the extremities due to non-specific diarrhea was presented.
Richard E. King, M.D., Thomas W. Marks, M.D.,and Richard Canaan, M.D. are associated with the Georgia Juvenile Amputee Clinic and Scottish Rite Hospital Atlanta, Georgia
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3. Dunn, H.G.: "Gangrenous Purpura and Its Occurrence in Meningococcal Septicaemia," Arch. Pis, in Childhood, 26:184-193, Apr. 1951.
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