Journal of Clinical Neonatology

CASE REPORT
Year
: 2022  |  Volume : 11  |  Issue : 3  |  Page : 187--191

Self-regressing scalp arteriovenous malformation in a neonate


Sudhir Malwade, Manas Nayak, Anand Gangadharan, Sharad Agarkhedkar 
 Department of Pediatrics, Dr. D. Y. Patil Medical College Hospital, Dr. D. Y. Patil Vidyapeeth, Pune, Maharashtra, India

Correspondence Address:
Anand Gangadharan
Department of Pediatrics, Dr. D. Y. Patil Medical College Hospital, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune - 411 018, Maharashtra
India

Abstract

Congenital AV malformations of scalp are rare congenital vascular malformations and often misdiagnosed as hemangiomas. Only 3 cases of neonatal scalp AVM have been reported till date. We report a rare case of self regressing scalp AV malformation in a neonate. An hour old full term female baby, presented with a soft, pulsatile large lesion (5cm x 4cm) with elevated borders and crusting over left parieto-occipital area, with bruit on auscultation. The infant had a soft cardiac murmur. USG cranium was likely suggestive of AV malformation or cephalhematoma. MR brain venography showed extra-calvarial network of multiple dilated tortuous veins draining into the left transverse sinus, sigmoid sinus and IJV. CT brain with CT carotid angiography revealed multiple arterial feeders from the left ECA and its branches, middle meningeal artery and left vertebral artery with multiple venous channels seen draining into the left transverse sinus, sigmoid sinus, IJV and EJV, the possibilities were arteriovenous malformation and less likely infantile hemangioma. Echocardiography showed small midmuscular VSD. Endovascular embolization was planned but didn't do immediately as the baby didn't show any signs of cardiac failure. The baby is on regular follow up and is growing well, with the lesion being regressing on its own. The baby doesn't have any signs of cardiac failure till now, hence only supportive care is being given. Early detection and comprehensive management of AVM result in a positive outcome. Embolization is only recommended if benefit outweighs the risk.



How to cite this article:
Malwade S, Nayak M, Gangadharan A, Agarkhedkar S. Self-regressing scalp arteriovenous malformation in a neonate.J Clin Neonatol 2022;11:187-191


How to cite this URL:
Malwade S, Nayak M, Gangadharan A, Agarkhedkar S. Self-regressing scalp arteriovenous malformation in a neonate. J Clin Neonatol [serial online] 2022 [cited 2022 Dec 9 ];11:187-191
Available from: https://www.jcnonweb.com/text.asp?2022/11/3/187/350033


Full Text



 Introduction



Arteriovenous malformations (AVMs) are vascular abnormalities where blood is shunted directly from the artery to the venous circulation via a fistula or nidus, without a capillary bed.[1] These lesions are pink-red and may have pulsations. Disfigurement, tissue destruction, ulceration, bleeding, and congestive heart failure may occur in patients.[2] Although AVMs are less common in children, they remain the most common anomaly of cerebral circulation in children and the most common cause of spontaneous intraparenchymal bleeding.[3]

To date, only a few cases of neonatal scalp AV anomalies have been reported. The pathophysiology of AVMs is unknown, but they are linked to both inherited and acquired factors. Diagnosis and treatment are frequently challenging and necessitate a comprehensive approach. We report a case of AVM in a newborn that was effectively treated in neonatal intensive care unit (NICU) at Dr. D. Y. Patil Medical College, Pimpri.

 Case Report



A full-term female newborn with a birth weight of 2.5 kg was born to a 32-year-old second gravida, with spontaneous conception, nonconsanguineous marriage, and uneventful antenatal history via cesarean section, in view of nonprogression of labor. At birth, the baby had respiratory distress with a respiratory rate of 62/min. The baby was shifted to the NICU for further care and management. On admission, the heart rate was 154/min with a mean blood pressure of 45 mmHg and SpO2 of 91% at room air. The baby was started on O2 support with continuous positive airway pressure, and euthermia and euglycemia were maintained. Apart from this, on examination, we found a 5 cm × 4 cm swelling with pulsations at the parieto-occipital region and had elevated borders with central crusting [Figure 1]. The swelling warranted thorough evaluation. Over the next 6–8 h, pulsations over mass regressed and stopped, and over a period of the next 24 h, oxygen support was weaned off and the baby was gradually shifted to room air. However, there were bruit and soft cardiac murmur on auscultation. There were no organomegaly, no liver, pulmonary, or cerebral bruit, and no dysmorphic features.{Figure 1}

The hemogram, platelets, prothrombin time/activated partial thromboplastin time/international normalized ratio, and biochemical parameters were all within normal limits. X-ray of the skull anteroposterior and lateral view was done to rule out bone defects. Ultrasonography (USG) of the cranium revealed an ill-defined heterogeneous hypoechoic structure measuring 7 cm × 6 cm × 1.5 cm, in the left parietal region, showing multiple internal septations and mild vascularity within, raising a possibility of cephalohematoma or AVM. After 24 h of life, we noticed visible pulsations over the left tragal area and bruit was also present.

Magnetic resonance (MR) brain venography showed an extra-calvarial network of multiple dilated tortuous and some smaller vascular channels including the multiple veins noted in the scalp in the left parieto-occipital region, multiple veins were seen draining into the left transverse sinus, sigmoid sinus, and internal jugular vein (IJV), and the left IJV appeared dilated and tortuous. Computerized tomography (CT) brain (plain and contrast) with CT carotid angiography findings showed, well-defined heterogeneous extra-calvarial scalp lesion in the left parieto-occipito-temporal region with few small hyperdense calcific foci within – likely phleboliths, it showed intense postcontrast enhancement with multiple dilated tortuous arterial as well as venous vascular channels and network of smaller vessels. Multiple arterial feeders were seen from the left external carotid artery and its branches, middle meningeal artery, and left vertebral artery with multiple venous channels seen draining into the left transverse sinus, sigmoid sinus, IJV, and EJV, which were suggestive of high-flow vascular lesion and the possibilities were AVM and less likely infantile hemangioma [Figure 2], [Figure 3], [Figure 4].{Figure 2}{Figure 3}{Figure 4}

Echocardiography revealed small midmuscular ventricular septal defect (VSD) left-to-right shunt, patent foramen ovale left-to-right shunt with normal AV semilunar valves, pulmonary artery pressure, and good biventricular function.

The baby was planned for endovascular embolization in future as per neurosurgery and interventional radiology opinion. However, the procedure was never performed because the baby did not go into cardiac failure. The baby was enrolled into the high-risk outpatient department at Dr. D. Y. Patil Medical College Hospital, Pimpri. On subsequent follow-ups, every 2 weeks for the first 2 months, then at 3 and 6 months, and in the latest visit at 9 months, the baby had good weight gain and attained age-appropriate milestones, and neurodevelopmental assessment was normal. The child never went into cardiac failure, there was no bleeding, no new masses were formed, and complete regression of mass was noted [Figure 5]. Follow-up USG revealed complete regression of AVM, and review echocardiography showed a decrease in size of VSD as well.{Figure 5}

 Discussion



AVMs are abnormal fistulas that join the arteries and veins without the presence of a capillary bed. Direct AV shunting occurs due to a lack of capillaries between the feeding arterial and draining venous components of AVMs, causing hypertrophy in both the arterial and venous components of the AVM.[3] AVMs have an embryological basis since the onset of a malformation occurs due to the persistence of an AV connection. The exact pathophysiologic mechanisms for AVM's occurrence are understudies. Although the majority of malformations are assumed to occur around the 3rd week of pregnancy,[4] in our case, antenatal care scans did not reveal any pathology.

Only a few examples of neonatal scalp AV anomalies have been reported to date.[5],[6] [Table 1] shows a comparison of the findings of two similar cases by Hussain AS et al.[5] Abreu et al.[6] with our case. In our case, the baby had no signs of heart failure. All three patients including ours had normal prenatal imaging and were diagnosed after birth. None of these cases had any history of any familial hemangioma. The male preponderance for AVM is a common factor among all three cases.[5],[6]{Table 1}

In 40% of the cases, the lesion presents at birth. Schobinger Clinical Staging System is used to trace their clinical history [Table 2]. They usually begin as latent lesions in childhood and develop into a pulsatile warm pink-bluish skin lesion as puberty starts. Dystrophic skin changes, bleeding, ulceration, and tissue necrosis occur as the lesion grows. Excessive venous flow, if left untreated, can cause volume and pressure overload in the heart and may cause cardiac failure.[7] All three cases, including ours, presented during the neonatal period without any dystrophic skin changes or necrosis. The newborn had signs of cardiac failure in the case reported by Mariana et al.,[6] whereas in the case reported by Ali Shabbir et al.,[5] the newborn had spontaneous bleeding from the lesion. In our case, the baby neither had cardiac failure nor bleeding.{Table 2}

Radioimaging is diagnostic as well as therapeutic in AVMs. USG and MR imaging (MRI) help with flow pattern and size detection of such malformations. MRI is particularly effective for determining the extent of AVMs as it often reveals multiple flow voids and hyperintense signals in the absence of a visible mass. On MRI, AVMs appear as a tangled mesh of dilated arteries and veins connected by linear or localized shunts.[5] MR angiography (MRA) is an effective modality in assessing AVMs before treatment. Due to its high temporal resolution, MRA can show arterial feeders, shunting volume, and the position and size of the nidus.[5],[8] CT is helpful in AVMs which are in close vicinity to bony structures. The contrast-enhanced helical CT allows structural evaluation of arteries, veins, and nidus, as well as flow analysis for preprocedural planning. Before any surgical intervention, angiography can be used to define the feeding and draining arteries.[9] CT or MRI in all three studies helped in reaching the diagnosis.

The goal of treatment is to eliminate the nidus, which attracts new vessels from nearby areas. Sclerotherapy and embolization are the first-line treatments because they cause less blood loss.[10],[11] In the cases reported by Abreu et al.,[6] the newborn manifested signs of heart failure, warranting intervention and embolization. However, in the study by Hussain et al.,[5] spontaneous bleeding from the lesion was the rationale for embolization. Ligation promotes collaterals' formation and hence not used. Cases with pain, bleed, ulcers, and cardiac failure need intervention. Resection of lesions leaves enormous defects that must be covered or repaired by flaps; thus, it should only be done if the advantages outweigh the dangers.[4],[7],[8]

On follow-ups, the size of the lesion regressed and only supportive care was provided to the child. All three cases revealed a favorable prognosis with minimal or no neurological impairment.

 Conclusion



The diagnosis of scalp AVM in a baby can be difficult because it is frequently mistaken for a hemangioma. As a result, thorough diagnosis and management are required. To confirm the diagnosis and identify the extent of the lesion, an MRI or CT angiography should be done. Embolization is usually the first line of treatment for a symptomatic lesion, and it is only recommended if the benefit justifies the risks.

Patient consent

Written informed consent was provided by the father.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's father has given his consent for images and other clinical information to be reported in the journal. The patient's father understands that names and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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