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Neonatal Gangrene of the Extremity: A Report of Three Cases and Review of the Literature

Ahmed H. Al-Salem¹, Mohamed Ibrahim Naga¹, Ashraf A. Alnosair¹, Mohamed Ramadan Abdallah¹²

¹Department of Pediatric Surgery, Maternity and Children Hospital, Dammam, Saudi Arabia

²Pediatric Surgery Unit, Sohag Faculty of Medicine, Sohag, Egypt

 

Correspondence:

Ahmed H. Al-Salem

P. O. Box 61015

Qatif 31911, Saudi Arabia

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Gangrene of the extremities is extremely rare at birth. We report three newborns with gangrene of the upper extremities, two of them were newborns to an insulin dependent diabetic mother. One of them had extensive gangrene involving the whole left upper limb necessitating amputation at the shoulder joint. The literature on the subject is also reviewed.

Key words: neonatal gangrene, infants of diabetic mother

 

Introduction

Peripheral limb ischemia and gangrene are extremely rare in newborns [1, 2, 3, 4, 5, 6, 7]. Acute limb ischemia is most commonly caused by thromboembolic phenomenon and although there are several predisposing factors, in the majority of cases no etiological factor can be found. The extent of gangrene is also variable ranging from one or more fingers or toes to the whole upper or lower limb. Approximately 25% of neonatal gangrene cases have been reported to occur in infants of diabetic mothers [8, 9, 10, 11, 12, 13]. This is usually seen in insulin dependent and poorly controlled diabetic mothers. The role of diabetes in the pathogenesis of gangrene in newborns is however uncertain. We report three newborns with gangrene affecting the upper limbs; one of them had extensive gangrene affecting the whole left upper limb. The literature on the subject is also reviewed.

Case reports

Case No. 1

A male newborn, a product of full term normal vaginal delivery was born to an insulin dependent diabetic mother. His birth weight was 3.2 kg. Clinically, he was well and no other abnormality could be detected. His cardiovascular system was normal. He was found to have ischemia with gangrene affecting the right hand (Fig. 1). The area of gangrene affected mostly the middle and distal phalange of the fingers. His axillary, brachial and radial pulses were normal. It was dry gangrene and in all affected fingers the distal phalange was blackish in color. Abdominal ultrasound was normal. He was started on heparin and intravenous antibiotics. There was no progression of the gangrene and ultimately underwent amputation of the gangrenous part of each finger. Post-operatively, he did well and was discharged home in a good general condition.

Figure 1: A clinical photograph showing ischemic gangrene involving the distal part of the right hand fingers.

8.4-5.1

Case No. 2

A male newborn, a product of full term normal spontaneous delivery was born in another hospital and transferred to our hospital with gangrene involving his right hand. His birth weight was 3.7 kg and his mother was non diabetic. Clinically, he was well with no other abnormalities. His cardiovascular system was normal with no murmurs or added sounds. Examination of his right upper limb revealed gangrene involving the distal part of the right thumb and index fingers (Fig. 2a and 2b). His axillary, brachial and radial pulses were normal. He was treated with intravenous antibiotics and heparin. There was no progression of his gangrene and ultimately the distal part of these two fingers was amputated. Post-operatively, he did well and was discharged home in a good general condition.

Figures 2a and 2b: Clinical photographs showing gangrene involving the distal part of the thumb and index fingers

8.4-5.2a 8.4-5.2b

Case No. 3

A male newborn was born prematurely (35 weeks gestation) to a diabetic mother on insulin. His mother was gravida 8 para 8 and was delivered by caesarian section due to fetal distress. His birth weight was 3.4 kg. Immediately after delivery he was hypoglycemic with a blood sugar of 27 mg/dl. This was stabilized the next day with intravenous glucose. Clinically, he was found to have ischemic gangrenous left upper limb. The gangrene was extensive extending all the way up to the shoulder joint. This was wet gangrene with the whole arm soft and jelly-like in consistency (Figures 3a and 3b). There were no constriction bands.

Figures 3a and 3b: Clinical photographs showing extensive gangrene affecting the whole left upper limb. Note the extent of gangrene reaching the shoulder joint.

8.4-5.3a 8.4-5.3b

The cardiovascular system was normal with no added murmurs. He was started on Heparin prior to referral to our hospital. His investigations revealed a WBC: 24.7 x103/μl, Hb: 16.5 g/dl, and platelets: 221 x 103/μl. He was covered with antibiotics and continued on heparin. Doppler ultrasound showed no blood flow distal to the left axillary vessels. CT-angiography showed abrupt cut off at the left axillary artery with enlarged vessels lateral to the left first rib. It was decided that the limb was not salvageable and to do amputation for him. This was done at the level of the left shoulder joint where disarticulation was done.

The amputated limb was not submitted for histological examination at the parent’s request. Postoperatively, he did well and was discharged home in good general condition.

Discussion

Gangrene of the extremities in the newborn is extremely rare. Its etiology is obscure and in many cases no etiological factor can be found but the presence of ischemic changes at birth suggests an intrauterine etiopathogenesis. Acute limb ischemia is most commonly caused by thromboembolic phenomenon but the underlying precipitating cause is not known. There are however several factors that are known to be associated with neonatal gangrene. These include prematurity, polycythemia, hypernatremia, systemic infection, hypothermia, umbilical artery catheterization, hyperglycemia, congenital thrombophilia, maternal diabetes mellitus, congenital heart diseases, amniotic constriction bands, and constriction by umbilical cord [1, 2, 3, 5, 6, 12, 13, 14, 15].

Oligohydramnios, resulting in compression of the extremities within the uterus, was proposed as the cause of neonatal gangrene [16]. An embolus dislodged from the retroplacental hematoma that resulted from abruptio placentae was also suggested as a cause of neonatal gangrene [3, 14]. Onalo et al. reported the case of a baby with congenital bilateral lower limb gangrene caused by thromboembolic phenomenon from retroplacental hematoma following abruptio placentae [14]. A strong association between placental thrombosis and fetal somatic thrombi has been described by Kraus and Acheen [4]. In their study, autopsy findings demonstrated that 37.5% of fetuses with significant placental thrombi had associated somatic thrombi.

Gangrene of lower limb secondary to developmental abnormality of femuro-popliteal artery was reported by Hefelfinger et al. [5]. Letts et al. in a 15 year review found 10 infants who had a vascular insult to a limb necessitating amputation of a portion of the limb [17]. In their review, the predisposing factors to vascular occlusion were prematurity, polycythemia, umbilical artery catheterization, and intensive care treatment for other life-threatening illnesses. The lower extremity was most commonly affected in their series, requiring below-knee amputations in two infants, knee disarticulation in one, and toe amputations in five. The remaining infants required an elbow disarticulation and amputation of the fingers, and one child had a massive ischemic contracture of the lower limb. Nagai et al. described two cases of intrauterine gangrene of bilateral lower limb complicated by twin-to-twin transfusion syndrome requiring below knee amputation [18]. There is a strong association between neonatal gangrene and infants of diabetic mothers [9, 10, 11, 12, 13].

The first published report of peripheral gangrene in an infant of a diabetic mother was by Valderrama et al. in 1972 [19]. The reason for this association is not exactly known but coagulation abnormalities have been proposed as the likely cause for arterial as well as venous thrombosis. Venous thrombosis and thromboembolism are well established complications in infants of diabetic mothers. Van Allen et al. also proposed coagulation abnormalities in infants of diabetic mothers as the likely cause for arterial as well as venous thrombosis [20]. Oppenheimer and Esterley in a review of 4000 autopsies of newborn infants, found 45 infants with venous thrombosis, of whom 13 were infants of a diabetic mothers [21].

Decreased production of prostacyclin and low levels of antiplasmin has been shown in infants of diabetic mothers [22]. There have been two reported cases of peripheral ischemia in infants of diabetic mothers where abnormal coagulation studies have been demonstrated [20, 23]. In the majority of previously reported cases, a renal vein thrombus served as the source of embolization but there are reports of thrombi from the placenta in those patients without associated renal vein thrombosis [19, 24]. Two of our patients were infants of diabetic mothers but in both of them no definite cause could be found and CT-angiography failed to demonstrate renal vein thrombosis. Neonatal gangrene can affect either upper limbs or lower limbs. Rarely, either upper limbs or both lower limbs can be affected [14, 25].

Kothari et al. managed four cases of lower limb gangrene in neonates, all presented with bilateral gangrene and required amputation and in two of them no cause was found [2]. Nagai et al. described two cases of intrauterine gangrene of bilateral lower limbs complicated by twin-to-twin transfusion syndrome requiring below knee amputation [18]. The extent of the gangrene is also variable ranging from one or more toes or fingers to part of the limb and rarely the whole limb may be involved. In our patient, the whole left upper limb was gangrenous suggesting thrombosis at the level of the axillary artery. The management of neonatal gangrene is initially conservative. These patients should receive heparin and antibiotics to prevent secondary infection. It is also important to differentiate between dry and wet gangrene. Wet gangrene is most likely the result of venous thrombosis and these patients are more likely to develop superadded infection. A combination of a thrombolytic agent with an anticoagulant was recommended by Arshad and McCarthy in neonates with limb thrombosis and complete clot lysis was reported in 75% of cases [26]. Duncan et al reported a case of in utero arterial embolism from renal vein thrombosis that was successfully treated with postnatal thrombolytic therapy [27]. Stavorovsky et al treated successfully a seven day old dehydrated male infant with acute ischemia of the leg due to acute thrombotic occlusion of an external iliac, common and superficial femoral, and popliteal arteries with early thrombectomy and arterioplasty [28].

It is important not to do early amputation and wait for definitive demarcation of the gangrenous portion. In cases of established gangrene, amputation is the treatment of choice. The aim should be to optimize future reconstruction and rehabilitation. Early surgical amputation is indicated in the presence of severe or progressive ischemic changes and in those with wet gangrene.

 

 

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