Scalp congenital hemangioma with associated high-output cardiac failure in a premature infant: Case report and review of literature

Abstract

Introduction

Scalp congenital hemangiomas (CHs) are rare vascular malformations among infants; they can be associated with an array of complications, including cardiac and cosmetic issues. Here, we report the endovascular treatment of a premature infant with a suspected large right parietal scalp hemangioma and associated high-output cardiac failure.

Case description

A two-day-old female premature infant (29 weeks gestational age; 1330 g birth weight) was referred by the neonatologists to our department for consultation and potential treatment of a large right parietal CH causing abrupt hypotension and high-output cardiac failure. Doppler ultrasound imaging at bedside revealed areas of arterial-venous shunting from the scalp and the presence of a superior sagittal sinus waveform, consistent with intracranial venous drainage. To alleviate cardiac dysfunction secondary to this lesion, trans-arterial embolization via n-butyl cyanoacrylate (nBCA) glue and deployment of detachable coils was performed via umbilical artery to occlude the right superficial temporal and occipital artery branches supplying the CH. Following treatment, the infant continued to require ventilator management, vasopressor support, and correction of coagulopathy, but by post-operative day two, her condition improved remarkably and the mass size began decreasing. The patient was discharged after a relatively uncomplicated subsequent 2½-month course in the neonatal intensive care unit.

Conclusion

Endovascular therapy proved effective and safe in treating cardiac failure associated with scalp CH, despite potential complications associated with neuro-interventional surgery in premature infants. Appropriate consideration in this patient population should be given to factors including blood loss, contrast use, radiation exposure, operative time, and possible intra-/post-operative complications.

Introduction

Congenital hemangiomas (CHs) are unique vascular lesions that encompass approximately 3% of documented infantile hemangiomas, and present at birth as fully developed masses with unexceptional postnatal growth.¹ Two subsets of CHs–rapidly involuting congenital hemangiomas (RICHs) and non-involuting congenital hemangiomas (NICHs)–have been previously described based on their clinical course.² Many of these CHs require no active treatment during the postnatal period; however, life-threatening complications may arise.³ Here, we present a report of a high-risk premature infant with a suspected large right parietal scalp RICH (sRICH) and associated high-output cardiac failure treated by successful endovascular therapy at our institution.

Case report

Presentation and clinical characteristics

A two-day-old premature neonate (female; 29 weeks gestational age; 1330 g birth weight) delivered by emergency Cesarean section was referred to our department for possible treatment of a congenital, vascular lesion causing heart failure. Intrauterine magnetic resonance imaging (MRI) performed one month before delivery revealed an extracranial mass involving the right parietal cranium near the vertex measuring 3.2 × 6.2 × 3.5 cm³; cardiomegaly was also noted. At birth, the patient required intubation, positive pressure ventilation, and oxygen administration. She was transferred to the Neonatal Intensive Care Unit (NICU) where she was placed on conventional mechanical ventilation and given surfactant. While initially hemodynamically stable on ventilator support, she developed hematuria, coagulopathy, anemia, and hypotension, progressing to high-output cardiac failure with signs of end-organ injury on the second postnatal day. Echocardiography demonstrated right ventricle and common carotid artery dilation. She required increased ventilator settings, vasopressor support, correction of acidosis, nitric oxide administration, and infusions of multiple blood products for stabilization prior to endovascular intervention.

Physical examination at the time of the consultation revealed an enlarged right parietal scalp mass, which had an audible bruit and was pulsatile, purple in color, and warm to touch (Figure 1). Bedside ultrasound confirmed increased mass size (6.1 × 2.0 × 7.1 cm³) and demonstrated numerous areas of arteriovenous shunting from the scalp as well as intracranial arterial supply. Examination of superior sagittal sinus (SSS) venous waveforms was consistent with intracranial venous drainage, and vascular lakes within the mass showed arterialized venous waveforms. Ultrasound while compressing the right superficial temporal artery (STA) revealed decreased vascularity; however, there was still significant flow through the vascular mass.

Figure 1.

Image of a large right parietal mass measuring 6.1 × 2.0 × 7.1 cm identified in our patient. At the time of the consultation, the patient exhibited high-output cardiac failure.

Surgical intervention

Due to the enlarging mass and the patient’s clinical deterioration, emergent intervention was warranted. After angiography of the right external carotid artery (RECA) via umbilical artery (Figure 2), we performed a trans-arterial embolization using n-butyl cyanoacrylate (nBCA) glue and detachable coils to sacrifice arterial branches feeding into the mass.

Figure 2.

Pre-procedural angiogram revealed vascular supply to the congenital hemangioma.

The existing umbilical artery pressure monitor was exchanged for a 3.7F microsheath, which was affixed to a rotating hemostatic valve (RHV). A 0.027 in microcatheter, along with a 0.01 in soft microwire, was used to perform cervical and cerebral angiography of the right common carotid artery, which revealed robust filling of the lesion from STA and occipital artery (OA) branches with drainage into the SSS and extracranial drainage to the scalp veins. Using overlay guidance, the microcatheter was advanced over the wire into the parietal branch of the right STA. A detachable coil was placed into the feeding artery to decrease arterial flow and nBCA glue was injected to perform a point occlusion of the feeding artery without reflux. This process was repeated for the frontal STA branch and the distal OA.

A follow-up angiogram was completed revealing significantly decreased arterial flow (Figure 3), and the 3.7F sheath was exchanged again for an umbilical artery pressure monitor. By the completion of the procedure, the patient had hemodynamically improved.

Figure 3.

Post-operative angiogram of the scalp mass after coil and n-butyl cyanoacrylate (nBCA) glue point occlusion of the feeding arteries (frontal branch of superficial temporal artery (STA), parietal branch of STA, and distal occipital artery) to the lesion.

Postoperative course and follow-up

Hypotension, acidosis, oliguria, and coagulopathy resolved by the second post-operative day. Despite the early hemodynamic decompensation and emergency endovascular intervention, the patient did not develop any major complications associated with prematurity. She required mechanical ventilation for two weeks, followed by an additional two weeks of supplemental oxygen. Her hospital course was otherwise marked by routine problems of prematurity, including jaundice requiring phototherapy, some difficulty transitioning from gavage to oral feedings, and anemia of prematurity, for which iron supplementation was prescribed.

The right parietal mass continued to regress in size during the hospitalization. At seven weeks of age, MRI revealed multiple flow voids within the lesion and suggested arterial feeders extending off the RECA and distal middle cerebral arteries, which drained into the right jugular vein. High T1 and fluid attenuation inversion recovery (FLAIR) signals in the proximal segments of the right STA as well as focally within the frontal and parietal segments of the STA were also observed, suggestive of a partially thrombosed vessel (Figures 4(a)–(b)). Echocardiography at the same time revealed normal biventricular function with no evidence of heart failure; however, a mildly dilated and hypertrophied right ventricle was noted.

Figure 4.

(a) Lateral MR angiography and (b) magnetic resonance imaging (MRI) of our patient at radiographic follow-up one-month post-endovascular procedure.

The patient was discharged at age 2½ months (3020 g). The management plan included routine well-child pediatric visits and outpatient monitoring by pediatric cardiology and dermatology. At the first outpatient pediatric visit one week following discharge, the infant still exhibited a right parietal scalp lesion with notable vascularity; however, vital signs and physical examination, including growth parameters, were otherwise normal for age, adjusted for prematurity.

Discussion

While the correlation between heart failure and hemangiomas is more common in the context of visceral or multiple lesions, scalp CHs associated with heart failure are exceedingly rare.^4,5 In the current literature, only three cases of scalp CHs co-presenting with heart cardiac symptomology (example: cardiomegaly related to high cardiac output) have been identified–the youngest of which was 36 weeks gestational age at birth.^6–8 Among these three patients, treatment modality included ligation and excision, surface arterial ligation and embolization, and management through diuresis and digoxin. Patient characteristics can be found in Table 1. Though similar, our case is incredibly unique due to the extent of our patient’s prematurity, the treatment specifics unique to this situation, and the safety considerations of endovascular treatment of a premature neonate.

Table 1.

Characteristics of reported congenital hemangioma (CH) patients with associated cardiac dysfunction.

Authors (year)	Patient gender	Birth weight (g)	Mass size (cm)	Location	CH management	Complication	Outcome
Hsiao et al. (2007)	F	2316 ga	7 × 6 × 3.5	Right temporo-parietal scalp	Ligation and excision (day 5)	Blood loss (300 ml)	Lesion regression
Konez et al. (2003)	M	NR	NR	Scalp (unspecified)	Surface artery ligation; embolization (day 5)	Pulsatile bleeding from lesion	Lesion regression
Vaksmann et al. (1987)	F	NR	NR	Right temporo-occipital scalp	Diuresis, digoxin	Severe CHF; metabolic acidosis; consumption coagulopathy	Death (5 days)

CHF: congestive heart failure; F: female; M: male; NR: not reported.

^aPremature patient, born 36 weeks gestation.

Our patient presented with worsening high-output heart failure during the second day of life. Mechanisms of cardiac dysfunction secondary to high-flow CHs have been discussed in the past. The development of heart failure seems to correlate to hemangioma size as this complication has not been reported in association with smaller tumors (<7 cm diameter).⁵ Furthermore, low peripheral vascular resistance resulting from arteriovenous communications can increase cardiac output.⁹ Because the venous return to the heart is increased without any reduction in pulmonary vascular resistance, there exists hemodynamic strain placed on the right ventricle of the heart, which leads to right ventricular dilation (a finding noted by echocardiography in our patient).

Endovascular therapy using detachable coils and nBCA glue was performed to treat the progressive cardiac failure. Due to the presence of collateral vessels potentially supplying the lesion, identifying and treating the appropriate feeding arteries was difficult. The procedure was further complicated by the size of blood vessels, as well as limitations in administering contrast and risk of massive blood loss. Even in “minimally invasive” endovascular procedures, a small amount of blood loss, like that found when exchanging the umbilical arterial line for a sheath, can prove deleterious in premature infants. Finally, there are also important considerations regarding administration of the nBCA glue, specifically the possibility of the glue shunting to the lungs causing an iatrogenic pulmonary embolus. To mitigate this risk, detachable coils were deployed first.

Percutaneous access to the right STA was considered. However, after thorough discussion, an umbilical arterial approach was decided upon due to the vascular complexity of the lesion, our familiarity with an umbilical approach, and the consideration that percutaneous access may not provide enough space between the access point and the mass for endovascular equipment.

The large, subcutaneous, highly vascularized mass in this neonate, and the prompt clinical response from proximal occlusion of the feeder arteries, led us to believe a diagnosis of RICH. While differential diagnosis includes conditions such as congenital tufted angioma and kaposiform hemangioendothelioma, definitive identification of this mass relies on histological analysis, which we did not perform, as there was no indication for biopsy.

Ultimately, neuro-interventional treatment in premature infants requires meticulous preparation to address issues regarding which devices to use, limiting blood loss, contrast use, and radiation exposure (accomplished through decreasing frame and pulse rate, grid removal, and beam collimation), reducing operative time, and establishing the goals of the procedure. In our procedure, there were no intra/post-operative complications observed. The patient’s cardiac failure resolved after treatment, and she was discharged at 2½ months.

Conclusions

Hemodynamically significant scalp CHs are rare vascular entities, with this report being the first in literature regarding the neuro-interventional therapy on one in a premature infant. Prompt endovascular therapy at two days postnatal age in our patient proved safe and effective in alleviating high-output cardiac failure despite the overall anastomotic complexity of scalp lesions and the associated difficulty of treating high-risk, premature infants. Meticulous preparation is necessary before performing neuro-interventional procedures in premature infants in order to reduce operative risks particular to this patient population.

Declaration of conflicting interests

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding

The authors received no financial support for the research, authorship, and/or publication of this article.