Spontaneous haematoma in the head and neck By KNUT E. SCHR0DER and TAIN W. S. MAIR (Tromso, Norway) HAEMATOMAS commonly occur as the result of trauma, either accidental or iatrogenic, or secondary to an haemorrhagic diathesis. In the neck, the arrangement of fascial planes bordering potentially large, communicating spaces (Levitt, 1970) permits the development of considerable spaceoccupying lesions, which can compromise the integrity of the upper respiratory and alimentary passages by compression. Expanding haematomas in this region frequently complicate penetrating (May et al., 1975) or blunt (Olson and Miles, 1971) injuries and, in the past, have been a dreaded complication of deep cervical infections (Alexander et ah, 1968; Todman, 1960). Although such cases may occasion problems in management, additional diagnostic difficulties arise when a haematoma either develops spontaneously, particularly a chronic, slowly growing haematoma, which may mimic a neoplastic growth, or is situated at deeper anatomical locations which do not communicate with more superficial regions, and is therefore not accompanied by subcutaneous ecchymoses. The present report is based on our experiences with three cases of spontaneous haematoma in the head and neck encountered in the course of a six-month period. Case reports Case 1. A 6-year-old girl was first seen in 1968 on account of repeated minor haemorrhages from the mouth. Diffuse lingual haemangiomatosis was diagnosed, but the family declined treatment. Tn 1974 she again attended the clinic three weeks after the sudden, appearance of a swelling in the left submandibular region (Fig. 1). The swelling was soft, not fluctuant, relatively well denned, and readily palpable in the floor of the mouth where the linguo-gingival sulcus was obliterated, but neither subcutaneous nor submucosal ecchymosis was present. The tumour was punctured with a fine needle, and 7-8 ml of altered, liquefied blood aspirated, which resulted in restoration of normal facial contours, There have been no further recurrences in the subsequent three years, and the oral haemorrhages have also ceased following repeated electrocoagulation treatment of the angiomatous papillae. Case 2. A 51-year-old white male noticed gradually increasing irritation and tear secretion from the right eye, with subsequent protrusion and diplopia. He was admitted to the Department of Ophthalmology where a space-occupying lesion was found in the upper, lateral region of the orbit, with consequent downward displacement of the bulb and protrusion. Laboratory investigations were noncontributory, and carotid angiography revealed no abnormalities. Plain sinus 215
216
K. E. SCHR0DER AND I. W. S. MAIR
FIG. 1 Case 1 showing the soft tissue swelling in the left submandibular region (arrows).
X-rays and tomograms were inconclusive, although the possibility of a bony defect in the lateral extremity of the right frontal sinus could not be excluded. Following transfer to the Department of Otolaryngology, the roof of the orbit was approached through an eyebrow incision. In the upper, lateral corner of the orbit a cystic lesion was found, adherent to the underlying bone, which, however, was intact, and containing greenish-yellow fluid with obvious crystal inclusions. The macroscopic appearance was similar to that seen in the mastoid cells of cases with idiopathic haemotympanum. The cyst was excised, and histological examination revealed an organized haematoma with numerous cholesterol crystal spaces surrounded by foreign-body giant cells. The post-operative course was uneventful, the ocular symptoms and signs receding rapidly, and the patient was symptomless when last seen two years after surgery. Case 3. A 64-year-old female presented as an emergency with a three-day history of progressive difficulty with respiration and swallowing. The previous medical and family histories were unremarkable. On admission to hospital, she had moderate stridor which increased markedly in the recumbent position. There was a clearly visible, diffuse swelling of the neck, most pronounced on the left side, with subcutaneous ecchymosis which extended from the level of the mastoid processes to the upper part of the chest. A lateral X-ray of the neck revealed a large, soft-tissue retropharyngeal swelling, which extended from the base of the skull and continued down into the thorax (Fig. 2). The patient was afebrile, and physical examination was negative apart from indirect laryngoscopy which revealed the tip of the epiglottis sandwiched between the base of the tongue and the retropharyngeal swelling, where submucosal ecchymosis was pronounced. The laryngeal aditus could not be visualized. There was, however, little pooling of secretions in the throat, and swallowing appeared relatively unhindered. The
SPONTANEOUS HAEMATOMA
217
FIG. 2 Extensive retropharyngeal soft tissue swelling (arrows) as seen in lateral cervical X-ray.
patient was nursed in the sitting position, while a tracheotomy set was kept readily available. All haematological and laboratory investigations were negative. The patient denied the occurrence of trauma or sudden exertional activity, nor had she been using any form of medication. Barium studies confirmed the ventral displacement of the hypopharynx and oesophagus in the neck and upper mediastinum, but no other abnormalities were demonstrable, and oesophageal peristalsis was prac-
218
K. E. SCHR0DER AND I. W. S. MAIR
tically unhindered. The femoral artery was catheterized, and arcography and selective angiography of the carotid and vertebral arteries of both sides were performed without any abnormality being demonstrated. The patient was able to swallow fluids comparatively freely, and the stridor and respiratory difficulties gradually improved over the subsequent few days. The subcutaneous and submucosal ecchymoses migrated inferiorly, and one week later the former was concentrated over the root of the neck and upper thorax (Fig. 3). Lateral X-rays of the neck confirmed the spontaneous regression of the retropharyngeal swelling. Endoscopy was performed two weeks after admission to hospital, completely normal mucosa being found, without evidence of bruising or presence of retro-oesophageal swelling down to the level of the diaphragm. Repeated haematological investigations were consistently negative. The patient was discharged from hospital after two and a half weeks, and when last seen three years later was in good health, and had had no further evidence of increased bleeding tendencies. Discussion
Although spontaneous haematoma formation is apparently a comparatively infrequent occurrence in otorhinolaryngological practice, all three of our patients presented in the course of a six-month period. In none could a history of extrinsic or intrinsic trauma be elicited, and no evidence of an haemorrhagic diathesis was demonstrable. Whereas an obvious vascular anomaly was present in Case 1, repeated investigations, including angiography, failed to reveal the site of haemorrhage in the other two cases. Spontaneous haematoma of the orbit is an infrequently encountered condition, which usually presents acutely with sudden proptosis and diplopia (Wheeler, 1937; Law, 1971). More rarely, the lesion has increased slowly in size (Denig, 1902; Wheeler, 1937), which has led to diagnostic confusion with intra-orbital tumours and expansive processes from the frontal sinuses. In the former, acute cases, a history of exertion immediately preceding the sudden onset can usually be elicited, with subsequent development of a 'black-eye', and later spontaneous resolution (Law, 1971). The chronic, progressive cases are usually not accompanied by subcutaneous discoloration of the eyelids, and surgical removal is indicated. In a recent review of retropharyngeal haematoma, Owens et a!. (1975) were able to trace only 19 other cases in the literature in addition to their own patient who developed haematoma while receiving anticoagulant therapy for a long-standing polycythemia vera. External trauma has been the aetiological factor in four of these cases (Logan and Doby, 1962; Sandor and Cooke, 1964; Miller, 1970; Stein and Herschberg, 1970), in one patient the haematoma complicated an impacted foreign body (Clark and Monks, 1969), whilst a variety of intrinsic excrtional factors such as prolonged coughing, sneezing and vomiting, were implicated in a further four patients (Benedetti-Valentini et a!,, 1958; Epstein and Klassen, 1960; Sandor and Cooke, 1964). Four patients had either an haemorrhagic diathesis (Masuelli and Delia Beffa, 1953) or received anticoagulant therapy (Giroux et ul., 1967; Reussi et a!., 1969; Owens et al., 1975), while vascular
SPONTANEOUS HAEMATOMA
219
FIG. 3 Subcutaneous ecchymosis in Case 3 as seen two weeks after admission to hospital.
factors accounted for a further three cases (Weaver and Young, 1964; Field and DeSaussure, 1965; Miller, 1970). Parathyroid or thyroid pathology was present in two patients (Capps, 1934; Sandor and Cooke, 1964), and one fatal case of haemorrhage occurred as a complication of a retropharyngeal abscess secondary to tonsillitis (Hays, 1964). Only Hennessy and Martinez (1970) have reported a case of apparently spontaneous retropharyngeal haematoma which resolved completely after four weeks. An additional case of retropharyngeal haematoma secondary to blunt trauma has recently been reported (O'Neill et ah, 1977). Irrespective of the aetiology, the clinical picture of sudden retropharyngeal haematoma is characterized by a diagnostic triad of tracheal and oesophageal compression, ventral tracheal displacement on the lateral cervicai film, and the subsequent appearance of subcutaneous bruising in the anterior neck and upper thorax (Capps, 1934; Sandor and Cooke, 1964). On the basis of autopsy findings, Sandor and Cooke (1964) postulated that the source of haemorrhage was an aberrant retro-oesophageal
220
K. E. SCHR0DER AND I. W. S. MAIR
artery arising from the descending aorta and passing dorsal to the oesophagus. This hypothesis has, however, never been confirmed, and angiography was also normal in our case. The absence of angiographic abnormalities, the frequent association with raised intra-thoracic, and thus venous, pressure, and the usually benign course with spontaneous resolution would seem to implicate the retropharyngeal venous plexus as the probable source of bleeding in many of these cases. The outcome has terminated fatally in seven patients, but this has been due to the gravity of the associated pathology. Spontaneous resolution has occurred in five cases, and in a further two patients a similar result has been achieved following correction of anticoagulant therapy. Tracheotomy has been deemed necessary in only four of the cases reported in the literature. Spontaneous retropharyngeal haematoma formation in the absence of demonstrable pathology is a rare occurrence, but would appear to be a relatively benign condition, which can usually be managed expectantly, in anticipation of spontaneous resolution in the course of two to three weeks. Summary
Three cases of spontaneous haematoma in the head and neck are presented. Whereas one case was associated with lingual haemangiomatosis, no source of bleeding could be demonstrated in either a chronic, spontaneous orbital blood-cyst or an acute retropharyngeal haematoma. REFERENCES ALEXANDER, D. W., LEONARD, J. R., and TRAIL, M. L. (1968) Laryngoscope, 78, 361.
BENEDETTI-VALENTINI, S., CARRATU, M., and Rossi, F. (1958) Minerva Chirurgica, 13, 647. CAPPS, R. B. (1934) American Journal of the Medical Sciences, 188, 800. CLARK, W. G. B., and MONKS, C. J. (1969) Journal of Laryngology and Otology, 83, 1039. DENJG, R. (1902) Ophthalmological Records, 11, 187. EPSTEIN, A. M., and KLASSEK, D. P. (1960) Journal of Thoracic and Cardiovascular Surgery, 39, 740. FIELD, J. R., and DESAUSSURE, R. L. (1965) Journal of Neurosurgery, 22, 610. GIROUX, P., DUTAULT, C , and BERNIER, J. (1967) Canadian Medical Association Journal, 97, 185. HAYS, H. (1964) Texas Medicine, 60, 904. HENNESSY, T. P. J., and MARTINEZ, J. A. (1970) Journal of the Irish Medical Association, 63,298. LAW, F. W. (1971) British Journal of Ophthalmology, 55, 556. LEVITT, G. W. (1970) Laryngoscope, 80, 409. LOGAN, G. E. C, and DOBY, T. (1962) Journal of the Maine Medical Association, 53, 114. MASUELLI, L., and DELLA BFFFA, A. (1953) Haematoiogica, 37, 113. MAY, M., CHADARATANA, P., WEST, J. W., and OGURA, J. H. (1975) Laryngoscope, 85, 557.
MILLER, C. H. (1970) Minnesota Medicine, 53, 887. OLSON, N. R., and MILES, W. K. (1971) Annals of Otology, Rhinology and Laryngology, 80,704. O'NEILL, J. V., TOOMEY, J. M., and SNYDER, G. G. (1977) Journal of Otolaryngology, 6, 43. OWENS, D. E., CALCETERRA, T. C , and AARSTAD, R. A. (1975) Archives of Otolaryngology, 101,
565. REUSSI, C , SCHIAVI, J. E., ALTMAN, R., YUSSEM, E. E,,and ROUYIER, J. (1969) American Journal
of Medicine, 46, 460. SANDOR, F. t and COOKE, R. T. (1964) British Journal of Surgery, 51, 682. STEIN, H., and HERSCHBERG, S. (1970) Journal of the American Osteopathic Association, 70, 260. TODMAN, R. C. (1960) Journal of Laryngology and Otology, 74, 493.
SPONTANEOUS HAEMATOMA
221
WEAVER, D. F., and YOUNG, D. J. (1964) Annals of Otology, Rhinology and Laryngology, 73, 542. WHEELER, J. M. (1937) Archives of Ophthalmology, 18, 356. Address for reprints: I. W. S. Mair, Department of Otolaryngology, University of Tromw, 9000 Tromsa, Norway.
216
K. E. SCHR0DER AND I. W. S. MAIR
FIG. 1 Case 1 showing the soft tissue swelling in the left submandibular region (arrows).
X-rays and tomograms were inconclusive, although the possibility of a bony defect in the lateral extremity of the right frontal sinus could not be excluded. Following transfer to the Department of Otolaryngology, the roof of the orbit was approached through an eyebrow incision. In the upper, lateral corner of the orbit a cystic lesion was found, adherent to the underlying bone, which, however, was intact, and containing greenish-yellow fluid with obvious crystal inclusions. The macroscopic appearance was similar to that seen in the mastoid cells of cases with idiopathic haemotympanum. The cyst was excised, and histological examination revealed an organized haematoma with numerous cholesterol crystal spaces surrounded by foreign-body giant cells. The post-operative course was uneventful, the ocular symptoms and signs receding rapidly, and the patient was symptomless when last seen two years after surgery. Case 3. A 64-year-old female presented as an emergency with a three-day history of progressive difficulty with respiration and swallowing. The previous medical and family histories were unremarkable. On admission to hospital, she had moderate stridor which increased markedly in the recumbent position. There was a clearly visible, diffuse swelling of the neck, most pronounced on the left side, with subcutaneous ecchymosis which extended from the level of the mastoid processes to the upper part of the chest. A lateral X-ray of the neck revealed a large, soft-tissue retropharyngeal swelling, which extended from the base of the skull and continued down into the thorax (Fig. 2). The patient was afebrile, and physical examination was negative apart from indirect laryngoscopy which revealed the tip of the epiglottis sandwiched between the base of the tongue and the retropharyngeal swelling, where submucosal ecchymosis was pronounced. The laryngeal aditus could not be visualized. There was, however, little pooling of secretions in the throat, and swallowing appeared relatively unhindered. The
SPONTANEOUS HAEMATOMA
217
FIG. 2 Extensive retropharyngeal soft tissue swelling (arrows) as seen in lateral cervical X-ray.
patient was nursed in the sitting position, while a tracheotomy set was kept readily available. All haematological and laboratory investigations were negative. The patient denied the occurrence of trauma or sudden exertional activity, nor had she been using any form of medication. Barium studies confirmed the ventral displacement of the hypopharynx and oesophagus in the neck and upper mediastinum, but no other abnormalities were demonstrable, and oesophageal peristalsis was prac-
218
K. E. SCHR0DER AND I. W. S. MAIR
tically unhindered. The femoral artery was catheterized, and arcography and selective angiography of the carotid and vertebral arteries of both sides were performed without any abnormality being demonstrated. The patient was able to swallow fluids comparatively freely, and the stridor and respiratory difficulties gradually improved over the subsequent few days. The subcutaneous and submucosal ecchymoses migrated inferiorly, and one week later the former was concentrated over the root of the neck and upper thorax (Fig. 3). Lateral X-rays of the neck confirmed the spontaneous regression of the retropharyngeal swelling. Endoscopy was performed two weeks after admission to hospital, completely normal mucosa being found, without evidence of bruising or presence of retro-oesophageal swelling down to the level of the diaphragm. Repeated haematological investigations were consistently negative. The patient was discharged from hospital after two and a half weeks, and when last seen three years later was in good health, and had had no further evidence of increased bleeding tendencies. Discussion
Although spontaneous haematoma formation is apparently a comparatively infrequent occurrence in otorhinolaryngological practice, all three of our patients presented in the course of a six-month period. In none could a history of extrinsic or intrinsic trauma be elicited, and no evidence of an haemorrhagic diathesis was demonstrable. Whereas an obvious vascular anomaly was present in Case 1, repeated investigations, including angiography, failed to reveal the site of haemorrhage in the other two cases. Spontaneous haematoma of the orbit is an infrequently encountered condition, which usually presents acutely with sudden proptosis and diplopia (Wheeler, 1937; Law, 1971). More rarely, the lesion has increased slowly in size (Denig, 1902; Wheeler, 1937), which has led to diagnostic confusion with intra-orbital tumours and expansive processes from the frontal sinuses. In the former, acute cases, a history of exertion immediately preceding the sudden onset can usually be elicited, with subsequent development of a 'black-eye', and later spontaneous resolution (Law, 1971). The chronic, progressive cases are usually not accompanied by subcutaneous discoloration of the eyelids, and surgical removal is indicated. In a recent review of retropharyngeal haematoma, Owens et a!. (1975) were able to trace only 19 other cases in the literature in addition to their own patient who developed haematoma while receiving anticoagulant therapy for a long-standing polycythemia vera. External trauma has been the aetiological factor in four of these cases (Logan and Doby, 1962; Sandor and Cooke, 1964; Miller, 1970; Stein and Herschberg, 1970), in one patient the haematoma complicated an impacted foreign body (Clark and Monks, 1969), whilst a variety of intrinsic excrtional factors such as prolonged coughing, sneezing and vomiting, were implicated in a further four patients (Benedetti-Valentini et a!,, 1958; Epstein and Klassen, 1960; Sandor and Cooke, 1964). Four patients had either an haemorrhagic diathesis (Masuelli and Delia Beffa, 1953) or received anticoagulant therapy (Giroux et ul., 1967; Reussi et a!., 1969; Owens et al., 1975), while vascular
SPONTANEOUS HAEMATOMA
219
FIG. 3 Subcutaneous ecchymosis in Case 3 as seen two weeks after admission to hospital.
factors accounted for a further three cases (Weaver and Young, 1964; Field and DeSaussure, 1965; Miller, 1970). Parathyroid or thyroid pathology was present in two patients (Capps, 1934; Sandor and Cooke, 1964), and one fatal case of haemorrhage occurred as a complication of a retropharyngeal abscess secondary to tonsillitis (Hays, 1964). Only Hennessy and Martinez (1970) have reported a case of apparently spontaneous retropharyngeal haematoma which resolved completely after four weeks. An additional case of retropharyngeal haematoma secondary to blunt trauma has recently been reported (O'Neill et ah, 1977). Irrespective of the aetiology, the clinical picture of sudden retropharyngeal haematoma is characterized by a diagnostic triad of tracheal and oesophageal compression, ventral tracheal displacement on the lateral cervicai film, and the subsequent appearance of subcutaneous bruising in the anterior neck and upper thorax (Capps, 1934; Sandor and Cooke, 1964). On the basis of autopsy findings, Sandor and Cooke (1964) postulated that the source of haemorrhage was an aberrant retro-oesophageal
220
K. E. SCHR0DER AND I. W. S. MAIR
artery arising from the descending aorta and passing dorsal to the oesophagus. This hypothesis has, however, never been confirmed, and angiography was also normal in our case. The absence of angiographic abnormalities, the frequent association with raised intra-thoracic, and thus venous, pressure, and the usually benign course with spontaneous resolution would seem to implicate the retropharyngeal venous plexus as the probable source of bleeding in many of these cases. The outcome has terminated fatally in seven patients, but this has been due to the gravity of the associated pathology. Spontaneous resolution has occurred in five cases, and in a further two patients a similar result has been achieved following correction of anticoagulant therapy. Tracheotomy has been deemed necessary in only four of the cases reported in the literature. Spontaneous retropharyngeal haematoma formation in the absence of demonstrable pathology is a rare occurrence, but would appear to be a relatively benign condition, which can usually be managed expectantly, in anticipation of spontaneous resolution in the course of two to three weeks. Summary
Three cases of spontaneous haematoma in the head and neck are presented. Whereas one case was associated with lingual haemangiomatosis, no source of bleeding could be demonstrated in either a chronic, spontaneous orbital blood-cyst or an acute retropharyngeal haematoma. REFERENCES ALEXANDER, D. W., LEONARD, J. R., and TRAIL, M. L. (1968) Laryngoscope, 78, 361.
BENEDETTI-VALENTINI, S., CARRATU, M., and Rossi, F. (1958) Minerva Chirurgica, 13, 647. CAPPS, R. B. (1934) American Journal of the Medical Sciences, 188, 800. CLARK, W. G. B., and MONKS, C. J. (1969) Journal of Laryngology and Otology, 83, 1039. DENJG, R. (1902) Ophthalmological Records, 11, 187. EPSTEIN, A. M., and KLASSEK, D. P. (1960) Journal of Thoracic and Cardiovascular Surgery, 39, 740. FIELD, J. R., and DESAUSSURE, R. L. (1965) Journal of Neurosurgery, 22, 610. GIROUX, P., DUTAULT, C , and BERNIER, J. (1967) Canadian Medical Association Journal, 97, 185. HAYS, H. (1964) Texas Medicine, 60, 904. HENNESSY, T. P. J., and MARTINEZ, J. A. (1970) Journal of the Irish Medical Association, 63,298. LAW, F. W. (1971) British Journal of Ophthalmology, 55, 556. LEVITT, G. W. (1970) Laryngoscope, 80, 409. LOGAN, G. E. C, and DOBY, T. (1962) Journal of the Maine Medical Association, 53, 114. MASUELLI, L., and DELLA BFFFA, A. (1953) Haematoiogica, 37, 113. MAY, M., CHADARATANA, P., WEST, J. W., and OGURA, J. H. (1975) Laryngoscope, 85, 557.
MILLER, C. H. (1970) Minnesota Medicine, 53, 887. OLSON, N. R., and MILES, W. K. (1971) Annals of Otology, Rhinology and Laryngology, 80,704. O'NEILL, J. V., TOOMEY, J. M., and SNYDER, G. G. (1977) Journal of Otolaryngology, 6, 43. OWENS, D. E., CALCETERRA, T. C , and AARSTAD, R. A. (1975) Archives of Otolaryngology, 101,
565. REUSSI, C , SCHIAVI, J. E., ALTMAN, R., YUSSEM, E. E,,and ROUYIER, J. (1969) American Journal
of Medicine, 46, 460. SANDOR, F. t and COOKE, R. T. (1964) British Journal of Surgery, 51, 682. STEIN, H., and HERSCHBERG, S. (1970) Journal of the American Osteopathic Association, 70, 260. TODMAN, R. C. (1960) Journal of Laryngology and Otology, 74, 493.
SPONTANEOUS HAEMATOMA
221
WEAVER, D. F., and YOUNG, D. J. (1964) Annals of Otology, Rhinology and Laryngology, 73, 542. WHEELER, J. M. (1937) Archives of Ophthalmology, 18, 356. Address for reprints: I. W. S. Mair, Department of Otolaryngology, University of Tromw, 9000 Tromsa, Norway.
