Journal of Postgraduate Medicine
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Year : 2014  |  Volume : 60  |  Issue : 3  |  Page : 349-350  

Scalp hematoma: An atypical presentation of neurofibromatosis and a cause of hemorrhagic shock

T Samra 
 Department of Anesthesia, Postgraduate Institute of Medical Education and Research, Chandigarh, Punjab and Haryana, India

Correspondence Address:
Dr. T Samra
Department of Anesthesia, Postgraduate Institute of Medical Education and Research, Chandigarh, Punjab and Haryana
India




How to cite this article:
Samra T. Scalp hematoma: An atypical presentation of neurofibromatosis and a cause of hemorrhagic shock.J Postgrad Med 2014;60:349-350


How to cite this URL:
Samra T. Scalp hematoma: An atypical presentation of neurofibromatosis and a cause of hemorrhagic shock. J Postgrad Med [serial online] 2014 [cited 2020 Dec 3 ];60:349-350
Available from: https://www.jpgmonline.com/text.asp?2014/60/3/349/138836


Full Text

Sir,

A 25-year-old male was shifted to the Neurosurgery Operation Theater (OT) for evacuation of a large scalp hematoma (25 cm × 20 cm). He was hypotensive and anemic (Hb = 5 gm%) and his head computed tomography (CT) scan revealed a subcutaneous soft tissue density overlying the left frontotemporoparietal and occipital area of the head with massive intratumoral hemorrhage. He had multiple neurofibromas and café-au-lait spots on his body.

Resuscitation with one liter of crystalloids and two units of whole blood was done and standard monitors (electrocardiography, non-invasive blood pressure, pulse oximetry) were attached in the OT. Etomidate (0.3 mg/kg) was used for induction; fentanyl (2μ/kg) for analgesia, and O2/N2O/isoflurane for maintenance. The right subclavian vein and right radial artery were cannulated and the central venous pressure and invasive blood pressure were monitored.

Uncontrolled bleeding complicated the surgical dissection; 2.5 liters of clotted blood and 2 liters of fresh blood were lost. Crystalloids (6 liters), colloids (1 liter), whole blood (6 units), and fresh frozen plasma (4 units) were transfused. Dopamine, at the rate of 10 μg/kg/minute, was administered to maintain hemodynamic stability. Hypotensive anesthesia and balanced resuscitation was adopted and mean arterial pressure was maintained at 50 mmHg. Inhalational anesthetics were stopped and opioid-based (fentanyl 6 μg/kg) anesthesia was given. Further surgical dissection was abandoned and the patient was shifted to the Intensive Care Unit (ICU) for stabilization and postoperative mechanical ventilation. He received an additional 6 units of blood and 4 units of fresh frozen plasma in the next 24 hours. The patient, however, did not develop complications of massive transfusion, like, transfusion-related acute lung injury (TRALI), infection, systemic inflammatory response syndrome (SIRS) or multiple organ failure. Inotropic support was withdrawn and the trachea extubated on the second postoperative day. He subsequently underwent further resections after ligation of the external carotid artery and is being followed up on an outpatient basis for tumor recurrence.

The diagnosis of neurofibromatosis was made on the basis of histopathology, which reported multiple areas of compact spindle cells with elongated nuclei, with vascular proliferation and invasion of the tumor in the sweat glands, blood vessels, and hair follicles.

Neurofibromatosis is a rare autosomal-dominant disorder characterized by the presence of benign soft-tissue tumors (neurofibromas) arising within the peripheral nerves. [1] Case reports of massive scalp hematomas leading to hemodynamic instability, secondary to spontaneous hemorrhage of the neurofibromas are limited. [2] They are difficult to manage surgically, as they are extensively infiltrative and highly vascular. Preliminary suturing around the lesion, ligation of the feeding vessels, hypotensive anesthesia, external compression, and preoperative intravascular embolization are some measures adopted to minimize intraoperative blood loss. [3] Neurofibromatosis is also complicated by the possibility of coexisting coagulopathies. [4]

A commonly used definition of massive transfusion is >10 units of RBCs in 24 hours. [5] An increased ratio of plasma and platelet to RBCs is thought to improve the outcome in patients with hemorrhage. Unanticipated intraoperative blood loss can complicate the anesthetic management of any surgical patient. Thus, each hospital should have a massive transfusion protocol for hemorrhagic shock.

Spontaneously developing massive scalp hematomas are always atypical presentations of an underlying tumor of the skin, subcutaneous tissue or neurovascular bundle. Thus, a thorough preoperative workup in the form of CT, magnetic resonance imaging (MRI), and cerebral angiography is mandatory.

References

1Jett K, Friedman JM. Clinical and genetic aspects of neurofibromatosis 1. Genet Med 2010;12:1-11.
2Renshaw A, Borsetti M, Nelson RJ, Orlando A. Massive plexiform neurofibroma with associated meningo-encephalocoele and occipital bone defect presenting as a cervical mass. Br J Plast Surg 2003:56;514-7.
3Jones RG, Kiatisevi P, Morris DC, Munk PL, Clarkson PW, Masri BA. Intravascular embolisation and surgical resection of a giant neurofibroma with intratumoural haemorrhage. Br J Radiol 2010;83:e225-9.
4White N, Gwanmesia I, Akhtar N, Withey SJ. Severe haemorrhage in neurofibromatoma: A lesson. Br J Plast Surg 2004;57;456-7.
5Repine TB, Perkins JG, Kauvar DS, Blackborne L. The use of fresh whole blood in massive transfusion. J Trauma 2006;60(Suppl 6):S59-69.

 
Thursday, December 3, 2020
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