Childhood polyarteritis nodosa: a clinical diagnosis

A six-year old boy presented with dry gangrene of toes and fingers with hypertension with no other systemic abnormalities. He had persistently high inflammatory parameters, was diagnosed as childhood classic polyarteritis nodosa and showed improvement with immunosuppressants along with antihypertensives. Toe gangrene required amputation in view of superadded infection.     

Peripheral gangrene in children

The case histories of 36 patients treated for peripheral gangrene between 1973 and 1987 were reviewed. The commonest causes of gangrene were disseminated intravascular coagulation due to bacterial septicaemia (15 patients) and dehydration due to gastro-intestinal fluid loss (8 patients). In only 2 patients was gangrene of truly iatrogenic origin; in 4 no apparent cause could be found. Secondary or contributing factors were recognisable in the majority. The overall mortality was 30%. Major lower limb amputation was necessary in 5 patients (bilateral in 3). The aetiology of peripheral gangrene was therefore multifactorial in most patients and idiopathic in only a small number. Delay before amputation allows both adequate stabilisation of severely ill patients and clear demarcation of gangrenous tissue.Offprint requests to: D. H. Bass     

Symmetrical peripheral gangrene in four pediatric cardiac surgery patients receiving extracorporeal membrane oxygenation

Symmetrical peripheral gangrene (multilimb ischemia without large artery occlusion) is a rare condition usually associated with disseminated intravascular coagulation, hemodynamic compromise, and/or sepsis. However, it has not been described in patients on extracorporal membrane oxygenation (ECMO). Over a 5 year period, four pediatric patients developed symmetrical peripheral gangrene on ECMO after cardiac surgery. They subsequently died and came to autopsy. History, physical examination, and laboratory studies were examined. Gross and microscopic autopsy material was reviewed. Patients were 11 days to 13 years old. Extracorporal membrane oxygenation duration was 11-22 days, and limb ischemia began 2-4 days before death. Three patients had rapid onset, with ischemia developing in <48 hours. In the fourth, ischemic changes began as focal lesions and gradually spread. Two patients were septic. Three had evidence of other end-organ damage. Pressors were used in 3 patients before the limb ischemia. Autopsies disclosed ischemic changes involving all limbs, with confluent ecchymoses. In a detailed examination in 1 case, large arteries of the extremities were patent. Involved skin and soft tissue showed bland fibrin thrombi in the microcirculation, with tissue necrosis and hemorrhage. This report describes the first 4 cases of symmetrical peripheral gangrene complicating ECMO. The 4 pediatric patients all had recent surgery for congenital cardiac disease, and all had significant exposure to ECMO prior to developing limb ischemia. Symmetrical peripheral gangrene is an unusual complication of ECMO that may arise in the setting of disseminated intravascular coagulation, sepsis, or other hemostatic and/or hemodynamic imbalance.     

Preamputation MR imaging in meningococcemia and comparison to conventional arteriography

Meningococcemia is a life-threatening infection which produces purpura fulminans and extremity gangrene in its most severe form. In patients with gangrene, amputation is usually necessary. The amputations frequently need revision as ischemic changes in the underlying soft tissues and bone are difficult to evaluate at the time of surgery. These ischemic changes often have non-vascular distributions and progress over time. We present two patients in whom MR imaging and MR angiography were performed prior to planned amputation. These cases demonstrate the potential utility of MR imaging in this setting, and compare the MR angiographic results to conventional arteriography in one of these patients. Received: 29 October 1997 Accepted: 29 December 1997     

Peripheral ischaemia and gangrene presenting at birth

The aetiology of non-iatrogenic causes of peripheral ischaemia and gangrene presenting either at birth or within a few hours of delivery is unknown in the majority of 56 confirmed cases. In this review of 47 cases occurring since 1941 the aetiology was clear in only 6, four due to compression by the encircling umbilical cord. There was no clear association with gestational age, birth weight, maternal age or type of delivery. Seven were infants of poorly controlled diabetic mothers and these may constitute a subgroup due to altered haemostatic mechanisms. Pregnancy hypertension was an association in 7 cases, oligohydramnios in 6. There is only limited support for birth trauma, sepsis, and thrombo-emboli from the ductus arteriosus as causes. There is indirect evidence that thrombo-emboli can migrate from the placental bed to the fetus. In recent years death from this condition has been rare with surgical thrombectomy increasingly successful in late presenting cases. When gangrene is established at birth surgical amputation, autoamputation, or some loss of function is usual. Peripheral ischaemic insults presenting at birth may be part of a wider spectrum of disorders, both prenatal and perinatal, attributable to occlusive vascular disruption.     

Amniotic band sequence and bilateral choanal atresia: a case report

Amniotic band sequence (ABS) is a group malformation that mainly affects limbs; clinically, constriction rings and lymphedema of the fingers, arms and legs, acrosyndactyly and pseudosyndactyly are observed; also there is congenital amputation of limbs due to distal swelling. Less frequently, craniofacial and trunk involvement are reported in some patients. Etiology is still unknown and most cases are isolated. In this report we present the case of a 45-day-old male with diagnosis of SBA and bilateral choanal atresia as attached finding, and review possible causes of SBA and associated alterations.     

Irgapyrin toxicity

Summary A child with Irgapyrin toxicity manifesting as ulcers on the tongue, pleomorphic skin lesions and gangrene of the fingers, is presented. The probable pathogenesis of the gangrene, a brief review of the known toxic effects of butazones and a warning about their use are described. From the Department of Pediatrics, Medical College, Patiala.     

A Case of Refractory Kawasaki Disease Complicated by Peripheral Ischemia

Kawasaki disease (KD), an acute systemic vasculitis that primarily affects infants and young children, is occasionally refractory to initial high-dose intravenous immunoglobulin (IVIG). Some patients with KD develop peripheral gangrene as a fatal complication, though this is rare. There has been no case reported in the literature of complicated peripheral gangrene that completely resolved without sequelae. Recently, we encountered a patient with KD which was refractory to IVIG, later developing peripheral gangrene. For the treatment of a peripheral gangrene dexamethasone, methotrexate, and an anticoagulant were administered, resulting in resolution of the gangrenous lesions without sequelae. Early diagnosis and treatment can reduce permanent sequelae and mortality from refractory KD complicated by peripheral ischemia.     

Right Ventricular Dysplasia with Massive Thromboemboli in Tuberous Sclerosis Complex

A 12-year-old boy visited the emergency room for severe dyspnea. He was diagnosed with tuberous sclerosis at 5 years of age, and right ventricular dysplasia was detected at 11 years of age. Thromboemboli in right ventricle and bilateral pulmonary arteries were confirmed with two-dimensional echocardiography and computed tomography. We report the case of a patient with tuberous sclerosis who presented with huge thrombi in a dysplastic right ventricle and massive bilateral pulmonary thromboemboli without evidence of a cardiac tumor.     

Bilateral gangrene of the feet associated with Salmonella infection in children with sickle cell anaemia

Two cases of bilateral gangrene of the feet associated with salmonella infection in Nigerian children with sickle cell anaemia are described. Healing was spontaneous and complete in both cases. A search for sickling phenomenon and salmonella infection is advocated in all African children with so-called idiopathic bilateral gangrene of childhood.     

Neonatal lower extremity gangrene

Four neonates suffering from bilateral lower limb gangrene were referred to us for further management. Two neonates had no contributory etiology. All four received appropriate treatment thus avoiding mortality but morbidity could not be avoided. All four neonates recovered uneventfully.     

Effect of heparin infusates in umbilical arterial catheters on frequency of thrombotic complications

We studied 111 infants requiring an umbilical artery catheter, 59 with heparin and 52 without. Thirty-four thrombi were detected, 16 in the heparin group and 18 in the control group. The numbers of thrombi in the two groups was not significantly different, but the number of clotted or nonfunctioning umbilical artery catheters was greater in the control group (P less than 0.05), as was the incidence of hypertension (P less than 0.05). There were no other significant differences between the two groups. We conclude that the use of low doses of heparin may not change the incidence of umbilical artery catheter-related thrombi, but it does appear to lower the incidence of their sequelae.     

Danaparoid sodium (Orgaran) in four children with heparin-induced thrombocytopenia type II

We report on four children with heparin-induced thrombocytopenia type II. In three patients, therapy with unfractionated heparin was associated with development of cardiac thrombi or with thrombosis progression up to the inferior vena cava or with aggravation of peripheral arterial occlusion. In the fourth child, the disease was recognized early on, and no complication occurred. Heparin-induced thrombocytopenia type II was confirmed by heparin-induced platelet activation assay and/or heparin/platelet factor 4-ELISA. Concomitant elevated antiphospholipid antibodies were seen in all patients. Danaparoid sodium applied at a dosage of between 1.2 and 7.1 U/kg/h stopped the disease progression in each patient. Three children had a clinical recovery with partial recanalization, but for the child with peripheral arterial occlusion disease, amputation of some of the toes became necessary. Conclusion: Our data indicate that heparin-induced thrombocytopenia type II is a potential lifethreatening disease in children and danaparoid sodium is beneficial in this age group.     

Digital ischemia in the neonate following intravenous therapy

Ischemia and gangrene in the hand can result from attempted intravenous cannulation in the neonate. This disastrous complication can be avoided with an understanding of the blood supply of the hand. Once ischemia occurs, amputation is the usual end result, but it may be averted by early diagnosis and treatment.     

Profile of fisting in term newborns.

BACKGROUND: Persistence of fisting of hands and cortical thumb is a predictor of abnormalities in development and dysfunction of the central nervous system, though this may be a normal finding in the immediate newborn period. AIM: To study the profile of fisting in early neonatal period. METHODS: Five hundred and fifty healthy term neonates were studied. Hand position was examined by a single observer between 24-48h of birth when the baby was awake and quiet. Complete fisting was defined as flexed fingers covering both proximal and distal palmer creases. If only the distal crease was covered, it was defined as incomplete fisting. Thumb position was observed as thumb by the side of the fingers, under the fingers and above the fingers. RESULTS: Of the total 550 babies, 338 (61.45%) had bilateral, 39 (7.09%) had right sided and 38 (6.91%) had left sided fisting and 135 (24.55%) had open hands. Of the 1100 hands, 669 hands (60.8%) had complete and 84 (7.63%) had incomplete fisting. "Cortical thumb" was found in 57% of fisted hands. There was no significant difference (p>0.05) in fisting according to the sex and weight of the babies. CONCLUSION: Fisting was a predominant hand posture among the babies (75.45%) but cortical thumb with bilateral fisting was seen in only 30% of babies.     

Kawasaki Disease Complicated by Peripheral Gangrene

An 8.5-month-old male infant with Kawasaki disease (KD) received high-dose intravenous immunoglobulin (IVIG) therapy on the fifth day after fever onset. However, multiple peripheral limb ischemias occurred 2 days later. Accordingly, heparin followed by dipyridamole was administered. Aside from a small amputation at the tip of the right middle finger, all other digital ischemias resolved. This presentation demonstrates that early recognition and management of peripheral gangrene in KD may keep its sequela to a minimum.     

高压氧综合治疗小儿外伤后气性坏疽67例效果分析

目的 探讨小儿外伤后气性坏疽的早期诊断及高压氧（hyperbaric oxygen,HBO）综合治疗方案的疗效.方法 选择外伤后气性坏疽患儿67例,经急诊手术治疗后即采用高压氧治疗,每日两次,7次疗法,压力0.8～0.25 MPa,并配合抗炎、每日换药及对症处理.结果 67例患儿经HBO治疗后,复查梭状芽胞杆菌1日转阴率96.92％,2日转阴率100％.13例未行再次截肢,54例肢体保存,未发生继发、交叉感染和扩散.伤口痊愈,治愈率100％.结论 小儿外伤后气性坏疽早期症状、体症不典型,临床医师应提高警惕,抓住最佳时机,及早诊疗.合理、有效的高压氧抢救性治疗方案,配合手术、抗炎、换药等综合治疗,能最大限度缩小组织坏死范围,预防并发症的发生,有效保存肢体,降低截肢率,提高生存率及治愈率.     

De novo interstitial duplication 4(q28.1q35) associated with choanal atresia

We report a case of direct interstitial duplication of chromosome 4 from 4q28.1 to 4q35 associated with bilateral choanal atresia. The child also had dysmorphic features including a broad nasal bridge, telecanthus, downward slanting palpebral fissures, prominent ears, mild bilateral clinodactyly of the 5th fingers and bilateral hypoplasia of the 2nd-5th toenails. There was also a slightly dilated renal collecting system. At the age of 2.5 years, he had moderate global developmental delay, short, wide and tapering fingers, and short toes with hypoplastic toenails. To our knowledge, this is the second report of choanal atresia in a patient with trisomy 4q involving this region.     

Outcome of Cardiac Thrombi in Infants

Use of central lines in the neonatal intensive care unit (NICU) has led to the formation of intracardiac thrombi. A paucity of data exists on the management of neonatal cardiac thrombi, with the few reported cases focusing on outcomes following thrombolytic therapy. This study was undertaken to evaluate the outcome of cardiac thrombi in neonates who do not receive thrombolytic therapy. Nineteen patients younger than 3 months of age diagnosed with cardiac thrombi were included. All 19 patients had a central line. Management consisted of a combination of antibiotics and low-molecular-weight heparin (n = 16) or surgical removal (n = 2). In one case, no treatment was instituted. One patient was lost to follow-up after partial resolution of the thrombus. Complete thrombus resolution occurred in 18 patients, 9 with negative blood cultures and 9 with positive blood cultures. It took longer for resolution of thrombi associated with positive blood cultures than for sterile thrombi. No patient had evidence of thrombus embolization. From these data we concluded that the natural history of cardiac thrombi is resolution. Infected thrombi require more prolonged therapy. Surgery is seldom required and thrombolytics are not usually necessary for clot resolution.