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Clinical Image
1 (
1
); 38-39
doi:
10.25259/WJWCH_2022_13

PHACE syndrome

Division of Neonatology, Department of Paediatric Medicine, Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India
Corresponding author: Nidhi Chauhan, Division of Neonatology, Department of Paediatric Medicine, Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India. nidschauhan.10@gmail.com
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This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Rakshpaul S, Chauhan N. PHACE syndrome. Wadia J Women Child Health 2022;1(1):38-9.

Twenty two-days-old female, second child of a non-consanguineous marriage born at 38 week, 3500 g weight, appropriate for Gestational age with no significant Antenatal/intrapartum/ postnatal period, was brought to High-Risk OPD with complaints of gradually increasing rash with excoriations on the left side of the face, including upper lip, nostril, temple, and neck [Figure 1a] noticed after a few days of birth.

Figure 1:: (a) Haemangioma, (b) fundus showing enlarged disc with vasculature, (c) like morning glory.

For a complete PHACE survey, -(P)osterior fossa abnormality; (H)emangioma of the face, neck and scalp; anatomical anomalies of cerebral or cervical (A)rteries; (C)ardiac anomalies/ (C)oarctation of aorta; (E)ye abnormalities. If (S)ternal anomalies are present then it is termed as “PHACES” syndrome.[1] MRA brain was done which revealed left midbrain hypoplasia, prominent and tortuous basilar artery, small caliber fetal origin of a right posterior cerebral artery, prominent left supraclinoid internal carotid artery, and proximal, middle cerebral artery. Fundus examination was done which is as shown in [Figure 1b]. The lesion has got its name from the flower morning glory in [Figure 1c]. Echocardiogram done was normal.

Presently, baby is on regular follow-up on propranolol showing normal growth parameters and normal INFANIB at 9 months.

Acknowledgments

Neonatology Team, B. J. Wadia Hospital.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

  1. , , , , , , et al. Consensus statement on diagnostic criteria for PHACE syndrome. Pediatrics. 2009;124:1447-56.
    [CrossRef] [PubMed] [Google Scholar]

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