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This infant presented to the emergency department with respiratory distress and failure to thrive. Examination revealed biphasic stridor with subcostal recession, tracheal tug, nasal flaring as well as a 5.5 cm cutaneous haemangioma on the patient’s upper back (figure 1). Following discussion with otorhinolaryngology, a diagnostic laryngotracheobronscopy was performed. This demonstrated a large subglottic haemangioma (SGH) occluding 80% of the airway (figure 2). She was subsequently intubated and sedated, admitted to paediatric intensive care unit for careful monitoring and given propranolol therapy. Dexamethasone was prescribed concurrently while the propranolol was titrated upwards over 3 days to 3 mg/kg/day. Blood glucose and electrolyte levels were monitored closely without any significant derangement seen. Following 1 week of treatment, her cutaneous haemangioma and SGH reduced in size (figures 3 and 4). She was extubated and stepped down to a paediatric ward for further monitoring and investigation for a possible atrial haemangioma.
Cutaneous haemangioma located on the patient’s upper back.
Annotated image of subglottic haemangioma (*) occluding over 80% of airway identified on emergency laryngotracehobronscopy. VC, vocal cord. Dashed orange line represents the glottic aperture.
Reduced size of cutaneous haemangioma following propranolol treatment.
Repeat laryngotracheobronscopy following propranolol treatment showing an improvement in patient’s airway due to reduction of subglottic haemangioma.
This patient had been seen four times in the community and twice in the paediatric emergency department before her admission. She had developed a biphasic wheeze by 6 weeks of age and was given multiple diagnoses by different clinicians, including new-born wheeze and chest infection. There was no familial history of atopy or asthma. It was not until her second attendance at the emergency department that a thorough examination was conducted by a paediatrician who associated the haemangioma on her upper back with her respiratory symptoms.
Infantile haemangiomas (IHs) are one of the most common benign vascular tumours of infancy affecting up to 10% of children.1 They usually develop in the head and neck region as cutaneous haemangiomas but can manifest as a paediatric upper airway infantile haemangioma (UAIH), typically in the subglottic region.2 Our case is particularly noteworthy as the cutaneous involvement was found on the patient’s upper back. IH occurring outside of the face is rare (<19%) in the context of UAIH, and the back specifically is yet more uncommon.3 For example, only one case in a 15-patient series by Marianowski et al was found to involve the patient’s back4 and similarly 2 patients in the 38-patient series by Uthurriagua et al.3
Due to the rarity of the UAIH, it has been difficult to draw conclusions between cutaneous haemangiomas and synchronous lesions in the upper airway. However, when there is an association between cutaneous haemangioma and UAIH, the suspicion is almost always raised with cutaneous lesions in the classical ‘beard’ distribution or with PHACES syndrome. The most high-risk skin patterns for UAIH are large segmental mandibular IH, as well as segmental IH of the lower lip and neck.3 This case is a clear demonstration that UAIH can occur alongside non-segmental cutaneous involvement in an atypical distribution, in this case, the patient’s upper back.
Despite the rarity of the disease, it is important for clinicians to recognise the association between cutaneous haemangiomas and UAIH as untreated mortality rates approach 50%.2 There are multiple treatment options for IH; propranolol (beta blocker) is now widely accepted as first-line treatment with research supporting its overall efficacy and safety.5
Patient’s perspective
We noticed our daughter developed a slight wheeze, at first we thought she had just caught a common cold; however, it turns out this was the first symptom of our daughter’s condition. Over the course of the 2 weeks leading up to our daughter’s diagnosis the wheeze gradually got worse and began to sound like a honking noise. Our daughter’s behaviour also changed drastically during that 2 week period, she went from being a content happy baby to screaming most of the day unless she was asleep. She was visibly working hard to breathe and became increasingly lethargic as a result. 5 days prior to her admission into PICU she also halved her daily milk intake, she went from taking 6 oz bottles to only taking 1 oz at a time. During this period, our daughter’s visible haemangioma on her back also grew significantly as I began to notice I could now feel it through her clothes as it became increasingly raised. As a mother I knew something wasn’t right and thankfully I persisted with seeking medical help until our daughter was finally diagnosed a full 2 weeks after her wheeze first appeared. We are so grateful that our daughter is recovering so well.
Learning points
While clinicians must be able to recognise the high-risk ‘beard’ distribution of cutaneous haemangioma with upper airway infantile haemangioma (UAIH), our case demonstrates this can occur when the cutaneous distribution is not classical.
The presence of stridor and/or respiratory distress in infants with any form of cutaneous haemangioma should prompt clinicians to consider UAIH.
In these cases, swift referral to an otolaryngologist and initiation of propranolol under close observation is crucial and can be life-saving.
Ethics statements
Patient consent for publication
Footnotes
Contributors ZS and ML co-first authored the manuscript. MD reviewed and supervised.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.