Intrauterine gangrene of the right upper limb in a newborn baby: Case report

Abstract

Introduction and importance

Congenital gangrene of the limb in a newborn child is an extremely rare pathology with polyetiological causes that has not been fully studied. Therefore, each case of this pathology has its own characteristics of manifestations and allows you to get closer to solving this problem.

Case presentation

We present a clinical case of congenital gangrene of the right upper limb in a newborn child. Analysis of the newborn baby's medical history indicated gestational hypertension and pyelonephritis during pregnancy in the mother, hemostasis disorders in the newborn.

Computed tomography (CT) of the vessels of the right upper limb and thoracic segment confirmed brachial artery thrombosis. At the initial stage, conservative treatment was carried out, which included preventive antibacterial therapy and local care of the affected area. Only after a clear delineation of the dead tissue, the amputation of the segment of the right upper limb was performed. Amputation was performed with preservation of the proximal growth zone in order to provide an adequate stump for subsequent prosthetics.

Clinical discussion

Adverse factors during pregnancy can cause increased thrombosis. Through fetal communications, blood clots from the right atrium can enter the left atrium, the left ventricle and further into the large circulatory circle and cause thrombosis of the artery of the right upper limb. Another factor of increased thrombosis is congenital intrauterine infection of the newborn. Conservative treatment is carried out after diagnosis until the final determination of the boundaries of gangrene. When performing amputation, it is important to preserve the proximal bone growth zone in order to form an adequate stump for prosthetics.

Conclusion

Congenital gangrene of the limb in a newborn child is an extremely rare pathology.

Irreversible changes in the upper limb in the child were caused by a combination of two factors: gestational hypertension and pyelonephritis in a pregnant woman and hemostasis disorders in a newborn due to intrauterine infection. Therefore, the preparation of women for pregnancy, examination for intrauterine infection and treatment of extragenital pathology are important in the prevention of this disease.

Highlights

• •Intrauterine gangrene is a rare disease that requires differential treatment as it results in children being disabled

1. Introduction and importance

Gangrene of the extremities in newborns is an extremely rare condition caused by impaired blood supply to the limb vessels [1]. Less than 100 cases have been reported worldwide [2,3]. Intrauterine and neonatal thrombosis of arteries and veins are distinguished [4]. Neonatal vascular thrombosis is often caused by catheterization of the umbilical artery in a sick newborn or considered a complication of sepsis or blood clotting disorders. The pathogenesis of intrauterine gangrene is divided into intrauterine compression or thromboembolic phenomena, depending on the cause. Compression is usually caused by abnormalities in the development of the uterus, malpresentation of the fetus with limb prolapse, oligohydramnios, amniotic fluid, or entanglement of the umbilical cord. Emboli can occur in the placenta and pass through the oval opening, settling in the arterial system, causing mainly necrosis of the upper extremities [5]. An analysis of the intensive care unit's work of the Level III Children's Hospital in eastern Ontario for 15 years revealed ten infants with vascular limb damage. Factors predisposing to vascular occlusion were prematurity, polycythemia, umbilical artery catheterization, and intensive treatment of other life-threatening diseases [5,6]. Maternal diabetes has also been indicated as a risk factor for thrombosis of veins and arteries in a newborn [7]. The sources of embolization may include the renal vein, inferior vena cava, or placenta. Most researchers recommend conservative treatment at the initial stage until demarcation appears, followed by economical amputation of the affected limb, taking into account future prosthetics [[8], [9], [10], [11]].

2. Case presentation

The present paper was reported in line with the SCARE guidelines [12].

On the first day of life, a child was taken to the hospital from the regional perinatal center. Complaints from the words of the neonatologist of the perinatal center about the birth of a child with a dark skin color of the right upper limb from the fingertips to the middle third of the shoulder. From the perinatal anamnesis, it is known that the mother of the child at the age of 32, this pregnancy was the 6th in a row, proceeded against the background of gestational hypertension and pyelonephritis. The cardiologist also diagnosed neurocirculatory dystonia of the cardiac type in a pregnant woman. Blood tests for HIV, Wasserman's reaction and hepatitis B were negative. The first ultrasound of the fetus at 11 weeks of pregnancy showed marginal presentation of the chorion, and the second ultrasound of the fetus at 19 weeks revealed no pathology. However, the third ultrasound of the fetus at 32 weeks of pregnancy showed a tendency to a large fetus and placentomegaly. Taking into account the deviation of the mother's serum markers (an increase in the level of beta-free unit of chorionic gonadotropin and a decrease in the level of pregnancy-associated plasma protein A (RARP-A)) in the first trimester of pregnancy and the risk of congenital malformations and chromosomal aberrations, the geneticist recommended invasive prenatal diagnosis. The pregnant woman refused an invasive diagnosis.

Anamnesis during hospitalization: A child from 6 pregnancies and 6 births, urgent labor. Birth weight was 4500 g, height - 58 cm, Apgar score - 8/9 points. According to the neonatologist, immediately after the birth of the child, attention was drawn to the dark skin color of the right hand. In the perinatal center, immediately after birth, an aseptic bandage was applied to the affected limb and infusion therapy was performed to subsidize proteins (Aminoven infant 10 %), glucose 10 % and vitamin K (0.1 ml/m2). Then on the same day, the child was taken by air ambulance to the children's surgical hospital.

Upon admission to the hospital, the child's general condition was severe due to necrosis of the right upper limb. The child was conscious, but reacted negatively to the examination. The baby's cry was weak, and the skin and visible mucous membranes were pale pink and clear, except for the affected limb. The body temperature was 36.5 °C, and the large fontanel (1.0 × 1.0 cm) was not tense and did not pulsate. The osteoarticular system had no visible pathology, and subcutaneous fat was evenly distributed. The tone of the soft tissues was reduced, and the peripheral lymph nodes were not enlarged. Shallow breathing without wheezing was observed in the lungs, and the respiratory rate was 52 breaths per minute. The heart tones were clear and rhythmic, and the heart rate was 160 beats per minute. The abdomen was not swollen, accessible for deep palpation and painless. The liver protruded from the hypochondrium by 2 cm. Urination is not disturbed. There was no meconium release at the time of the examination. The external genitalia were formed correctly according to the female type, and the anus was formed in the right place.

Status localis: The right upper limb, from the fingers of the hand to the middle third of the shoulder, had a pale dark color, while the fingers of the right hand were black (Fig. 1, Fig. 2). The affected areas of the limb were swollen, in places with epidermis detachment. The limb was cool to the touch, pulsation on the radial and ulnar arteries was not detected, active movements in the joints were not noted.

Fig. 1.

View of the right upper limb from the inside.

Fig. 2.

View of the right upper limb from the outside.

In the hospital, the child underwent a laboratory examination. The value of the laboratory test indicators is given in Table 1.

Table 1.

Values of blood parameters in a child.

	The value of the indicator	Reference value
Total protein	39,6 g/l	42–62 g/l
Albumins	27 g/l	28–44 g/l
Bilirubin	52,3 mmol/l	To 60 mmol/l
C-reactive protein	41,7 mg/l	0–5 mg/l
Prothrombin index	59,7 %	95–105 %
Prothrombin time	16,3″	9,0–15,0″
International normalized attitude (INA)	1,4	0,81 to 1,2
Activated partial thromboplastin time (АPTT)	94,1 %	25,4–36,9 %
Fibrinogen	4,74 g/l	2,00–4,00 g/l
Hemoglobin	156 g/l	160 g/l
Erythrocytes	4,95 × 1012/l	3,9–6,2 × 1012/l
Hematocrit	45 %	40–60 %
Thrombocytes	222 × 109/l	150–400 × 109/l
Leucocytes	12,3 × 109/l	13–20 × 109/l
Neutrophils NE	55 %
Lymphocytes LY	45 %
Erythrocyte sedimentation rate (ESR)	6 mm/h	2–10 mm/h

Ultrasound examination of the veins and arteries of the upper extremities showed that the blood flow through the arteries was reduced, but the blood flow through the veins was not disturbed. Computed tomography of the thoracic and abdominal aorta with contrast showed small parietal defects in the lumen of the right subclavian and axillary arteries measuring 4.4 × 1.3 mm, which narrowed the lumen of the artery by less than 50 % (Fig. 3). The radial artery was pathologically dilated. The soft tissues were infiltrated and edematous, while the lung parenchyma was compacted along the dorsal surface. Computed tomography revealed signs of thrombosis of the artery of the right upper limb, as well as an open arterial duct, an open oval window and congestive changes in the lungs. Adrenal hyperplasia was also noted.

Fig. 3.

CT with vascular contrast.

A blood test for perinatal infections showed that the ELISA for herpes simplex virus showed IgG 3265 conl.units.

According to the results of the conducted studies, the diagnosis was made: intrauterine gangrene of the right upper limb, from the hand to the middle third of the shoulder. Intrauterine pneumonia, open ductus arteriosus, hypoxic-ischemic encephalopathy and intrauterine infection were also diagnosed.

To improve blood circulation, alprostadil was administered at the rate of 2 μg/kg/h and heparin at the rate of 5 units/kg/h per day. However, the intensive symptomatic therapeutic measures carried out did not give the expected positive effect.

On the sixth day of life after the appearance of a clear demarcation of the lesion, the child underwent surgery - amputation of the right upper limb to the level of the middle third of the shoulder (Fig. 4).

Fig. 4.

View of the right upper limb after amputation.

Histological examination of the skin, subcutaneous tissue and muscle tissue revealed necrosis with large focal hemorrhages in the intermuscular space and diffuse inflammatory infiltration of all layers (Fig. 5, Fig. 6) Blood clots, stagnation of erythrocyte sediment with diapedesis hemorrhages were observed in the lumen of blood vessels of large and small caliber in the skin and subcutaneous adipose tissue.

Fig. 5.

Histopathological slides of the amputated right upper limb. Staining with hematoxylin-eosin. Magnification ×10.

Fig. 6.

Histopathological slides of the amputated right upper limb. Staining with hematoxylin-eosin. Magnification ×40.

On the 30th day of the day, the child was discharged home in a satisfactory condition.

The observation period is 2 years and 9 months. There are no complaints from the words of the child's mother. Pain in the stump of the right shoulder does not bother. The child's weight is 14 kg, height 96 cm, age-appropriate. The child does not lag behind in mental development.

When examining the right shoulder: the length of the shoulder stump is 14 cm, the stump is cylindrical, the skin is pink, the turgor of soft tissues is preserved, sensitivity is not impaired, the skin of the shoulder is warm to the touch. The scar has a keloid character. The blood circulation of the stump is not disturbed. For 2 cm, there is a subcutaneous standing of the humerus stump without violating the integrity of the skin. Active and passive movements in the right shoulder joint in full. The volume of soft tissues on the stump of the right arm is 0.5–1.0 cm less than on the healthy shoulder. According to the mother, the child serves himself, helps with the stump of the right hand to the healthy hand.

3. Clinical discussion

In this clinical case, when studying the course of pregnancy, we identified such unfavorable factors as gestational hypertension, pyelonephritis of pregnant women, neurocirculatory dystonia. These factors could contribute to increased thrombosis in a pregnant woman. Studies [13] allowed the authors to conclude that hypertensive disorders of pregnant women are predictors of short- and long-term risk of venous thromboembolism. The mechanism of development of such a prothrombotic phenotype is multifactorial, which also includes endothelial dysfunction, coagulation and platelet activation [14]. Through the open ductus arteriosus and the open oval window of the fetus, which were installed in the child during examination, blood clots from the right atrium could get into the left atrium, into the left ventricle and further into the large circulatory circle [15] and cause thrombosis of the artery of the right upper limb.

A comprehensive study of the child's hemostasis revealed thrombocytopenia, a decrease in the percentage of prothrombin activity, an increase in the international normalized ratio, an increase in activated partial thromboplastin time, an increase in fibrinogen levels. The cause of these hemostasis disorders could be a congenital intrauterine infection. An indirect confirmation of this was an increase in the level of C-reactive protein, an indicator of the activity of the inflammatory process [16].

Thus, the state of the mother's health (gestational hypertension, pyelonephritis of pregnant women) could cause thrombosis of the arteries of the right upper limb in the fetus.

The resulting circulatory disorders were aggravated by hemostasis disorders in a newborn child and led to irreversible changes in the tissues of the right upper limb. CT with contrast is more informative in the diagnosis of circulatory disorders [17].

With the established diagnosis of intrauterine gangrene of the right upper limb at the initial stage of treatment, we conducted conservative therapy, including antiplatelet drugs, preventive antibacterial therapy and local care of the affected area.

Nonoperative anticoagulant treatment or follow-up (i.e., wait-and-see tactics) was successfully applied in 81 % of patients [18]. The authors concluded that conservative treatment can be an easily applicable, effective and safe method of treating thrombosis in newborns. In case of irreversible gangrene, amputation is necessary. According to the literature, there is a tendency to delay when amputation is not performed from the very beginning until the final determination of the gangrene boundaries, but in the case of established gangrene, amputation is the preferred method of treatment [19].

Subramanu V. et al. recommend that amputation should be planned in such a way as to preserve the growth plate and provide an adequate stump for the installation of the prosthesis in the future [20]. Amputation of the right upper limb was performed by us only after the appearance of a clear demarcation of the affected segment from the fingertips to the middle third of the shoulder of the right upper limb. Amputation of the limb was performed with preservation of the proximal growth zone of the humerus and closure of the humerus stump with healthy skin.

Thus, intrauterine limb gangrene in a newborn is an extremely rare pathology, the cause of which is polyetiological and has not been fully studied to date. Some researchers attach importance to the pathology of the mother, others – infections and hemostasis disorders, leading to arterial thrombosis. The outcome of intrauterine gangrene of the limb is its amputation, which leads to the future disability of the child. This is a great psychological trauma for the child's parents. At the same time, both targeted social support of the state and support of public non-governmental organizations will be of great importance.

4. Conclusion

In our opinion, risk factors for the development of limb gangrene in a newborn include age-related pregnancy, a woman who gave birth a lot, a large fetus, the presence of extragenital pathology of the mother, deviation of serum markers of the mother in the first trimester, congenital intrauterine infection of the newborn. The combination of these causes led to the development of arterial thrombosis and irreversible changes in the tissues of the right upper limb. Therefore, the preparation of women for pregnancy, examination for intrauterine infection and treatment of extragenital pathology are important in the prevention of this disease.

The patient's point of view

After discharge from the hospital, the patient's family was given recommendations on monitoring her condition, conducting physical therapy and monitoring by the hospital surgeon. Her family is in constant contact with the treatment team. Currently, the State provides financial assistance to the child. The treating team regularly gives recommendations on the rehabilitation of the child to the polyclinic doctor. The child's family is satisfied with the coordinated actions of the hospital and polyclinic. Prior to drawing up this document, the child's family was contacted, and they are currently satisfied with her general condition.

Consent

Written informed consent was obtained from the patient's parents for the publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Ethical approval

The approval of the Bioethics Commission of the West Kazakhstan Medical University named after Marat Ospanov was received.

Funding

This research received no specific grant from the public, commercial, or not-for-profit sectors.

Guarantor

Valentina Sarsenova, assistant of the Department of Pediatric Surgery, West Kazakhstan Marat Ospanov Medical University, Aktobe, Republic of Kazakhstan.

Research registration number

Unsuitable.

CRediT authorship contribution statement

All the authors participated in the treatment of the patients, writing, and approving the manuscript.

Declaration of competing interest

No conflict of interest to disclose.