JUVENILE GANGRENE BY WALTON MARTIN, M.D. AND BENJAMIN RICE SHORE, M.D. OF NEW YORK, N. Y. FROM THE FIRST SURGICAL DIVISION OF ST. LUKE S HOSPITAL I HAVE ventured to report four cases of gangrene of the extremities in young subjects, as such cases are unusual and their origin and manner of development imperfectly understood, I have thought it might be of interest to bring to your attention the reports of similar cases and to follow, as far as possible, the succession of happenings. The first case, a boy four and a half years old, became acutely ill with temperature and symptoms suggesting a generalized infection. When seen three weeks after the onset, he had gangrene of the left foot, of both ears and of a circular patch of skin over the left patella. There was a systolic murmur over the apex of the heart and a to-and-fro pericardial friction rub heard over the sternum. The gangrenous areas separated and the foot sloughed through at the ankle-joint at the end of two months. The boy recovered and is well today, five years after his original illness. The skin over the stump of his leg, only a few inches above the original line of demarcation, is normal looking and movable. The skin of the stump of the ear is white and the scar looks as if a portion of the ear had been severed by a surgical operation. (Fig. I, 2, 3, 4.) The second case I have recently seen in consultation, through the courtesy of Dr. J. V. Bohrer of New York. A child six years old was lying in bed in a small flat. One hand and both legs were coal black, there were zones of suppuration where the living tissue was being separated from the dead. There was a recent healing scar on the external ear where a portion of dead tissue had fallen off. The boy had had an acute illness, apparently diphtheria, preceding the onset of gangrene. (Fig. 5.) The third case, a boy seven years old, had widespread chronic tuberculosis. He had been chronically ill for six months with enlargement of the abdomen and cough when the left foot and lower part of the left leg became blue, swollen and tender. Gradually this gangrenous area became deep black and was separated from the living tissue. (Fig. 6.) Four months after the onset, when the soft parts had sloughed through to bone, an amputation was carried out through the thigh. The parts bled freely. The stump healed. Six months later the boy was still alive, although the general tuberculosis was more advanced. A section taken through the main vessels in the amputated leg showed endarteritis, confined largely to the intima. One of the veins showed some evi- dence of canalization as if it had been thrombosed. (Fig. 9.) The fourth case, a boy of fourteen, had an indolent perforating ulcer on the ball of the great toe. In the course of three years he developed gangrene of the tip of the second toe and a perforating ulcer of the sole of the foot. The anterior portion of the foot was amputated. The stump healed soundly. The boy is well today and free from pain. We have, then, four cases of gangrene in young subjects. The first and the second followed an acute general infection, in both of these the patches of dead tissue were multiple. The third occurred in a patient suffering from general tuberculosis with evidence of local endarteritis in the tissues above the 725 MIARTIN AND SHOkR ganigreniotus leg-; iii the foumrth local endarteritiS walS (leillonistrated in the foot, four or five iniches fromi the gangrenious toe. Thle (letailed report of three of these cases is given at the enid of this paper, with the microscopical studies micade by Doctor Shore of miiy staff, who has assistedl me in its preparation. In I904, Barraudl I published a paper giving the records of I03 cases of gangrene of the extremities, occurring in patienits uinder thirty and following acute infectioni. TIhe occasion of this essay was an unusual instance of gan- grene of both legs in a woman of twenty, follow- ing an inifection of the .fing er. The autopsy ^ showed an arterial throm- ,& bus from the aorta dowl- ward. Among the cases he had collected, in forty- M four the gangrene oc- _-|,_ ; curred in the course of typhoid and in eleven it followed typhus. In six the gangrenie developed during measles; in five ............. d~~~(uring scarlet fever; in two it followed diphtheria Case I. Gal,gree oand in five pneumonia. In .lii one case gangrene oc- of ...n ..atncurred afttare perityphlitis (appendicitis) in a six- year old boy; ic another, ani eight-day-old iin fan t, gangrene of Both legs fol- FIG. Ie lowedh lhlebitis of the uim- Case I Gangrene of foot, and area oeripateia. blilical veinr. In still another, ganigrenie of the left keg, in a boy seveniteen mioniths old, was preceded by to-n- sllitis. Many of these cases are accompaniiedI by autopsy reports or reports of examiNiination of the large vessels after operatioe. In 1914, Khatizt 2 published a paper o, "Spontaneous Gangree of the Extremities in Children" and reported two cases, o2e in a child three years old, with ganigrenie of both feet and the lobes of both ears and a patch on the back of the hiand. The gangrene was not preceded by any illness. Both legs were amputate(l; the patient recovered. No anatomical catise for the gan- grenie was found(. The vessels appeared niormial to the surgeon (loinig the amlptitationi. No miicroscopical examiniation was miade. The seconid patienit, a four-year-old boy, had ganigrene of both feet. The 726 JUVENILE GANGRENE gangrenie was preceded by an illness, possibly measles. Both feet sloughed off. The child recovered. Khautz collected and published the records of fifty cases; thirty of these are the same as those reported by Barraud. Every year or two, during the last twenty-four, similar cases have been reported. They are sufficieintly uncommonn to excite exceptional interest and( are usually reported with the prefatory remark that the rarity of the condition has occasioned the publication and, for the most part, with repro(luctions of photographs showing the lesions. Between 1914 and d 1925, Gerlach,3 Veau and ^ Weber,4 Hoyne,5 Hell- strom,6 G u n s o n,7 Rob- bins,8 Chodax,9 Michael 1"t 4 Frenkel,l Thorpe,l"C .,.; _ Kramer,13 Brusa,H Gor- don and Newman,15 Har- rell,16 Hoopma1n,17 Per- rier,'8 and Learmonth,"' have all reported cases of gangrene in childreni, fol- 7; lowing infection. In looking back on the autopsy findings and the observations made oni ves- sels of the amputated limbs in these reports. four groups can be distin- 7 / guishled. (i) The gan- FIG. 2. gren-ie has f ollowed an Case I. Gangrene of ear. emboluis, the primary thrombus being in the heart or the aorta; (2) there hlas been a primary thromobus in one of the large vessels supplying the extremity: (3) there has beeni evidence of local arteritis in the vessels above the gaii- grenous area; (4) no change has been founid in the vessels up to the line of (lemarcationi, there has been, presumably, a capillary thrombosis which ha; passed on to massive tissue death. The gangrene has been preceded by acute general infection in so malny instanices in all groups that it seems probable that microorganisms or toxic substanices liberated when micro6rganisms are growing in the body, are fac- tors that cannot be set aside. It is also obvious that some unusual circum- stance or combination of circumstances has been added, for it is a matter of commoni knowledge that measles, scarlet fever, diphtheria, typhoid and typhus fever, all manner of acute general infectionis, run their course commonly without any such pheniomena. 727 MARTIN AND SHORE The study, then, leads to a brief consideration of the occurrence of thronm- bosis under the influenice of infection in the heart, in the large vessels, or in the capillaries of a given area. As Aschoff expresses it, using a mathematical figure of speech, throm- bosis is the function of a number of variables. There are factors that have to do with injuries to the endothelial lininig of the various parts of the vas- cular system; there are factors that have to do with slowinlg of the 1)lood current an(l there are those which have to do with alterations in the makeup of the blood. All these factors play a part but we are still ignoraint of the or(ler of their occurrence. Damage to en(lotlhelial cells by toxins, slowing of the current, deposition of fibrin, perhaps a few mi- croorganisms caught in the fibrin meshes, further 10 c a 1 destruction and the formation of a thrombus, may in some instanices b)e the sequence. The (lomli- ;nant factor may be, oni the other hand, a slowing of the blood current. The velocity of the flow may be reduced to a point where FIG. 3. certain of the solidl ingre- Case 1. Five years later, showing deformity of ear following separation of gaingrenous tissue. dienits, like thebloo1 plate- lets, settle out. It is even possible that the endothelium dies as the result of being covered over by platelets, as suggested by Aschoff,20 and the institution of the changes leading to the separation of fibrin and the formation of clot mlay not be due in the first instance to (lamaged endothelium.