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Congenital Pressure Injuries

Children With Wounds: Asking the Right Questions

Congenital Pressure Injuries

INTRODUCTION

This month’s column begins with a case report. A full-term girl was born via cesarian delivery due to a failure to descend and complex presentation. Maternal history was not significant for any illnesses; this pregnancy was complicated by polyhydramnios. The author’s team was consulted by the newborn nursery pediatrician after multiple lesions were found immediately after delivery. The largest, a 3 cm × 1 cm dusky purpuric retiform patch, was noted over the left medial ankle area. The peri-lesion rim was erythematous, and the foot in general was swollen compared with the right foot. Two (2) tiny vesicles were noted within the patch. Three (3) smaller purpuric lesions were appreciated over the dorsal lateral ankle area and heel (Figure 1). Another erythematous, irregularly shaped, angulated blanchable patch was appreciated over the left parietal scalp area (Figure 2). No peri-wound edema or erythema were noted. 

No discomfort was appreciated on palpation. The patient was admitted to the neonatal intensive care unit, where immediate management included application of nitroglycerine ointment 2% (Savage Laboratories, Berkeley, CA) twice a day to the left foot lesion, alternating with warm compresses (the team’s standard management of potential ischemic injury), and observation of the head lesion.

Given the differential diagnoses of ischemia, thrombosis (vasculopathy/hypercoagulopathy), congenital vascular malformation, or pressure injury (specifically deep tissue injury, based on the physical examination findings) hematology and dermatology services were consulted. The following studies were completed:

Coagulopathy work-up (complete blood count, coagulation, D-dimer, fibrinogen, protein C and S, antithrombin 3, factor V Leiden gene mutation, beta-2 glycoprotein, and anti-cardiolipin antibody): normal

Ultrasound of the area with arterial and venous doppler studies: normal flow, no thrombi

Point-of-care ultrasound: this study showed the break in the normal subcutaneous fat tissue and hypoechoic changes underlying injured area of the left ankle.

One of the vesicles within the lesion progressed to develop a blister within 24 hours after presentation (Figure 3). The blister was punctured through and through with a sterile needle to avoid tension and worsening skin injury, but without complete deroofing to act as a natural biologic dressing. Because the ultrasound reveled normal vascular flow, nitroglycerine ointment was discontinued. Surfactant-based wound and burn dressing (PluroGel; Medline, Northfield, IL, USA) was added on day 2 for cell salvage1 and moist healing as the injured superficial skin layer started to desquamate. On day 3, point-of-care ultrasound captured a deep tissue injury path toward the epidermis. The smaller lesions over the left medial ankle area and the head lesion were getting smaller, without skin breakdown.

On day 4, the patient was discharged. The parents were comfortable with applying surfactant-based gel once a day to the ankle area, covered by silicon dressing. The patient was seen 1 week later. At that time, the larger ankle lesion was almost healed; the head deep tissue injury and the smaller ankle deep tissue injury were completely healed.

CONGENITAL PRESSURE INJURY

Congenital pressure injuries have not been commonly accepted as a separate entity. Practitioners normally think of them as sequelae of traumatic birth processes or visual displays of ongoing ischemic injuries. The author’s personal experience has led her to believe that congenital pressure injuries do occur. 

Certain conditions need to be ruled out before a pressure injury can be diagnosed because physical appearances can be misleading. Traumatic injuries (such as those caused by instrumental deliveries and monitoring devices), abrasions, and bruises developed during vaginal birth can masquerade as pressure injuries. Congenital cutaneous conditions, such aplasia cutis congenita, vascular tumors and malformations, cutaneous manifestation of viral infections, and Langerhans cell histiocytosis, should be considered as well. Congenital ischemic injuries, such as Volkmann ischemic contracture or neonatal thrombosis, can also similarly present on physical examination. 

Vascular tumors and malformations can be evaluated by biopsy, ultrasound, and magnetic resonance imaging. Most have a specific appearance and can be differentiated easily from a pressure injury. Lack of blood flow, pulsations, and typical vascular malformation characteristics on ultrasound can dissuade the practitioner from this diagnosis. Vascular ultrasound should be performed with Doppler study to evaluate arterial and venous patency as well as to ensure thrombus absence and continuous and adequate blood flow. Volkmann ischemic contracture, which is a result of increased pressure within the closed compartment of an extremity, can present with identical physical injury. Increased pressure leads to ischemia and necrosis, although no thrombotic foci are appreciated. If a biopsy would be performed, coagulative necrosis of the dermis, subcutaneous tissue, or muscle would be seen, something that can be seen in a biopsy of a pressure injury, depending on stage. Phenotypically, ischemic purplish macule or plaque may be seen, with or without clear borders; it may progress to an eschar and eventually to an open ulceration, again almost identical to a pressure injury. Congenital ischemic venous or arterial thromboses can present with purple, delineated lesions, although fingers and toes are more commonly affected and the initial appearance does not have clear borders. 

Maternal prothrombotic disorders, infections, hypertension, and placental abnormalities can lead to clot formation and travel from maternal to fetal vasculature and eventually to fetal end-organs, manifesting as cutaneous lesions. The mother of the patient in the current case report did not have a preexisting condition during pregnancy. Nonetheless, an extensive coagulation work-up was performed on the newborn and ruled out a prothrombotic or hemorrhagic event.

After an extensive literature search, 2 articles were found that describe congenital pressure injuries: a case series of 3 preterm and term neonates2 and a case report of a full-term neonate.3 Torrelo et al2 hypothesized that congenital pressure injury occurred as a result of sustained pressure from the maternal bony prominence, leading to subcutaneous fat necrosis and increased pressure transmission. They noted a dimple over the involved area; it has been hypothesized that congenital dimples occur due to atrophy of subcutaneous tissue secondary to pressure between the bone and the uterine wall. The current patient did not have any dimples and had a normal karyotype. Karyotype is an important element of the evaluation, as disorders such as Silver-Russell syndrome, popliteal pterygium syndrome, congenital rubella syndrome, and various deletions and duplications are associated with skin dimples. Johnson3 related her patient’s congenital pressure injury to prenatal oligohydramnios, leading to a loss of cushioning of the fetus from the maternal uterine wall and bony elements.

It is likely that the etiology of the current case is deep tissue injuries due to the infant’s intrauterine positioning and tissue deformation against maternal bony prominences. It was surprising since polyhydramnios should have cushioned the tissues, but it is known that pockets of amniotic fluid are not distributed equally in utero.

Deep tissue injury is believed to originate from within fascial, muscle, or skin layers, a combination of cells’ deformation and cytoskeleton disruption with resultant intracellular substance leakage. The inflammatory milieu leads to edema, increased oxygen consumption, and stagnant blood flow in the distal vessels. Eventually dermal bleeding ensues, contributing to ecchymotic discoloration. Ultrasound can be useful in deep tissue injury identification, capturing a dark hypoechoic shadow from the muscle layer upward, breaking through fascia and eventually reaching the skin.4 After all other etiologies have been excluded and a deep tissue injury has been diagnosed, management is approached in the same way as any other pressure injury. Pressure injury management has been discussed in many previous issues of this column. The readers is invited to revisit those if guidance is needed.

CONCLUSION

Congenital pressure injuries are rare but do occur. Excluding other diagnoses is paramount because limb- and organ-threatening ischemia can be missed. Management of congenital pressure injuries is the same as postnatally acquired pressure injuries.

Dr. Boyar is director of Neonatal Wound Services, Cohen Children’s Medical Center of New York, New Hyde Park, and assistant professor of Pediatrics, Zucker School of Medicine, Hofstra/Northwell, Hempstead, NY. All photos provided are with the consent of the patients’ parents. This article was not subject to the Wound Management & Prevention peer-review process.