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A previously healthy 2-year-old African American female with braids presented to our hospital with 2 days of right-sided face and head swelling. What's the diagnosis?
A previously healthy 2-year-old African American female with braids presented to our hospital with 2 days of right-sided face and head swelling. The swelling was initially noted to be around the right eyelid and nasal bridge. The mother stated that her head size was growing over the 2 days. The patient also had had a week of cold symptoms and 4 days earlier had complained about headaches and had 1 episode of vomiting. Due to the headaches, the mother took her braids down. Of note, a month ago, the patient sustained blunt head trauma after falling on her midface and developed swelling and bruising of the eyelids and face. She went to another emergency department and a computerized tomography (CT) of her head that was reported as negative. The swelling and bruising from this traumatic event resolved and the child returned to her baseline. Per the mother, the child did not sustain any new traumatic event.
History and exam
The patient has no history of easy bleeding or bruising and had an incision and drainage of an abscess in the past without any perfuse bleeding. On physical examination, the child was active and playful. Her vital signs were within normal limits. She had swelling over her nasal bridge and right upper eyelid and a boggy and tender scalp. There were no patches of hair loss or bruising. Her neurological exam was normal. Her head circumference was 19.5 inches, which is at the 90% percentile. There were no signs of trauma.
The differential diagnosis at this time included a skull fracture, preseptal cellulitis, orbital cellulitis, accidental trauma and non-accidental trauma (NAT) (Table 1). An infectious cause such as preseptal and orbital cellulitis were low on the differential due to the physical exam and lack of infectious symptoms in the history such as fever. A skull fracture was also considered, but also less likely due to the lack of tenderness. Highest on the differential for this unexplained evolving swelling were an unwitnessed NAT. Imaging and laboratory studies would help rule these diagnoses in or out.
Her laboratory studies were significant for a hemoglobin of 7.9 g/dL. All other laboratory values were within normal limits including PTT at 25.9 seconds and PT at 13.5 seconds. A CT head showed an acute on subacute subgaleal bleed without a skull fracture or any acute intracranial bleeding (Figure 1). The patient was discussed with neurosurgery and neuroradiology and no acute intervention was done. Due to an unclear etiology of this acute bleeding, child protective team and social work were consulted. A skeletal survey was negative. The child was admitted for further evaluation.
During her admission, the surgical consultants determined that no operation was needed and recommended the patient’s care be guided by her laboratory values and clinical presentation. Hematology was consulted to evaluate the low hemoglobin and for a possible undiagnosed underlying bleeding disorder. A bleeding evaluation demonstrated a normal von Willebrand factor (vWF) antigen level, vWF ristocetin cofactor activity, vWF collagen-binding activity, ratio of each of the cofactor functional assays to von Willebrand antigen, and hemoglobin electrophoresis. The factor VIII assay was elevated at 265%, but was not clinically significant. The low hemoglobin was due to extensive bleeding.
The child’s hemoglobin was followed and had stabilized. The patient was discharged with a hemoglobin of 8.8 g/dL without any interventions. The patient was sent home 2 days after admission and with daily iron. The child protection team performed an extensive social evaluation and were not concerned about NAT. It was at this time, that the etiology was determined to be due to the release of hair braids.
Five pediatric (non-neonatal) cases have been described in the literature where a subgaleal bleed developed after hair braiding due to shearing forces on emissary veins in the scalp.1-3 This case, although connected through hair braids, did not develop through braiding, but instead through takedown of the patient’s hair. We believe that it was the movement of her hair when the braids were taken down that caused the acute bleed.
The scalp is made of multiple layers which can be summarized with the acronym SCALP. The skin is the outer most part, then the connective tissue, aponeuroses, loose alveolar tissue and lastly the periosteum. Subgaleal hematomas (SGH) occur between the periosteum and galea aponeuroses and occurs due to rupture of emissary veins that travel through this space through tangential or radial forces.1-3 The bleed is contained by the galea aponeuroses and periosteum, superiorly and inferiorly, the orbital ridge and nuchal ridge anteriorly and posteriorly and lastly the lateral temporal fascia.1
Subgaleal hematomas occur most commonly in neonates and is reported in only a handful of case reports outside of the neonatal period. In children, the described causes of SGH include minor trauma, lymphoma or malignant tumors, and bleeding disorders.4,5 In this case, the finding of subacute bleeding was suspected by radiology to be the result of the initial trauma and then subsequent acute bleeding was due to the take down and movement of the braids. The initial subacute SGH, although not seen on the initial head CT, per mom’s report, was likely due to her recent injury as it can take several weeks for resorption of blood from her initial trauma. Subgaleal hematoma can re-bleed when the scalp has additional physical stress. The hematoma can cross suture lines and can extend subperiosteally into the orbital ridge.1,3 When this occurs, the patient can experience proptosis, vision loss, and pain to the eye, which our patient did not experience.2
Whereas SGH can be diagnosed clinically, it is important to investigate other causes of a swelling around the scalp, forehead, and eye. After the diagnosis is made, there needs to be consideration for a bleeding disorder and NAT. For this patient, given the clinical picture and age, abdominal trauma labs and a skeletal survey were completed as part of the NAT work-up and were negative. Our child protection team evaluated the patient and her mother and were not concerned about NAT. Additionally, hematology was consulted and the patient was tested for vW disease as well as factor XIII deficiency. Hematology also recommended platelet functioning testing via PFA-100 given the patient’s normal platelet count. The elevation in Factor VIII can cause thrombosis and not bleeding, and so was not considered significant given that the patient did not have hypercoagulable issues. In the end, the anemia was attributed to iron deficiency anemia.
Treatment of SGH is conservative as the blood will self-resolve in a few weeks. It is not recommended to drain the SGH through needle aspiration or surgery.1-3
Braids can be culturally important and so we would not recommend parents to not braid their child’s hair at all. Given this case, we recommend families to not weave braids tightly as there is a risk of bleeding and SGH development. We would recommend pediatricians to evaluate for underlying coagulopathies and hereditary bleeding disorders in non-neonatal patients with SGH.
1. Kichari JR, Gielkens H. Massive traumatic subgaleal haematoma. Emerg Med J. 2013;30(4):344.
2. Edmondson SJ, Ramman S, Hachach-Haram N, et al. Hair today; scalped tomorrow: Massive subgaleal haematoma following sudden hair pulling in an adolescent in the absence of haematological abnormality or skull fracture. J Craniofac Surg. 2016;27(5):1261-1262.
3. Meyer S, Gortner L, Abdul-Khaliq H, Papanagiotou P. Subgaleal hematoma. Indian J Pediatr. 2014;81(3):321.
4. Natarajan MS, Prabhu K, Braganza A, Chacko AG. Posttraumatic subgaleal and orbital hematoma due to factor XIII deficiency. J Neurosurg Pediatr. 2011;7(2):213-217.
5. Nittby HR, Bjartmarz H. A Subgaleal Hematoma That Turned Out to Be a B-Cell Lymphoma. World Neurosurg. 2016;93:489.e7-9.