CASE REPORT proctalgia
An Unusual Case of Proctalgia We report the case of a 21-year-old man with the sudden onset of severe proctalgia followed by headaches and fever. The patient had a lumbar puncture consistent with subarachnoid hemorrhage. After a normal fourvessel cerebral arteriogram, a myelogram and computed tomography scan of the spine were performed and revealed a mass lesion of the L1 level. Pathologic evaluation after resection of the mass resulted in a diagnosis of hemorrhage from a myxopapillary ependymoma. These relatively benign tumors of the cauda equina-filum terminale respond well to treatment. The differential diagnosis of proctalgia is reviewed, and the need for emergency physicians to consider spinal subarachnoid hemorrhage when a patient presents with the acute onset of rectal pain is emphasized. [Rappaport B, Emsellem HA, Shesser R, Millstein E: An unusual case of proctalgia. Ann Emerg Med February 1990;19:201-203.] INTRODUCTION Rectal pain or proctalgia is a relatively c o m m o n complaint in emergency practice and is rarely due to a life-threatening illness. Proctalgia is most often caused by hemorrhoids, obstipation, and anorectal fissures.1 Pain experienced as arising from the rectum m a y also be referred from distant structures. We present the case of a patient with an unusual cause of severe rectal pain that demonstrates the importance of fully considering all conditions that can cause proctalgia.
Bob Rappaport, MD* Helene A Emsellem, MD* Robert Shesser, MDt Washington, DC Ellen Millstein, MD:~ North Chicago, Illinois From the Depar.tments of Neurology* and Emergency Medicine,t The George Washington University Medical Center, Washington, DC; and Abbott Laboratories, North Chicago, IIlinois.¢ Received for publication April 13, 1989. Revision received July 13, 1989. Accepted for publication August 2, 1989. Address for reprints: Helene A Emsellem, MD, Department of Neurology, Room 7-407, 2150 Pennsylvania Avenue, NW, Washington, DC 20037.
CASE REPORT A 21-year-old m a n presented to the emergency department complaining of severe, deep peroneal and rectal pain. He had been in excellent health until four days earlier when he noted the sudden onset of pain deep in the rectal region. He had had a pilonidal cyst repair several m o n t h s earlier, and the pain was believed to be secondary to that procedure. Rectal examination, lumbosacral spinal radiography, and a CBC were normal, and the patient was discharged. The rectal discomfort intensified to the point that he was reluctant to move his legs for fear of precipitating the pain. He developed a bifrontal headache and returned to the ED two days later w h e n he became febrile. The patient's history was remarkable for only the pilonidal cyst repair, which was reportedly associated with exploration no deeper than the coccygeal-sacral fascia. He was not taking any medications, and the family history was unremarkable. Physical examination revealed a well-developed, ill-appearing man. His blood pressure was 120/70 m m Hg; temperature, 39.2 C; and pulse, 72. The neck was stiff. There was no spinal tenderness, but bilateral straight legraising produced low back pain. Rectal examination revealed no focal tenderness or mass. The remainder of the general medical examination was normal. The patient was alert and oriented with normal cognitive function. He complained of photophobia, but there were no focal cranial nerve abnormalities. The sensory examination was normal. Strength was normal in the upper and distal lower extremities. Hip flexion was limited to 5 degrees w i t h o u t resistance secondary to pain. The reflexes were 1 + throughout, and the plantar response was flexor. Cerebellar function was normal
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PROCTALGIA Rappaport et al
in the upper extremities and could not be evaluated in the lower extremities. The patient could not ambulate. A head computed tomography (CT) scan showed small ventricles, although sulci were seen clearly and there was no mass effect. A lumbar puncture revealed an opening pressure of 270 c m H20 and grossly bloody fluid with a xanthochromic s u p e r n a t a n t after c e n t r i f u g a t i o n . There were 90,000 R B C / m m 3, 300 WBC/mrn a (48% polymorphonuclear leukocytes, 52% lymphocytes), 270 mg/dL protein, and 29 mg/dL glucose with a simultaneous serum glucose of 107 mg/dL. The patient was placed on aneurysm precautions with a presumed diagnosis of subarachnoid hemorrhage (SAH). Four-vessel cerebral angiography performed the next morning was normal. A C T scan of the spine after myelography revealed a round lesion of the cauda equina at the L1 level. At thoracolumbar laminectorny the next day, a hemorrhagic 1.5 x 1 cm mass was resected from the L1 level of the cauda equina. P a t h o l o g i c e v a l u a t i o n revealed a hemorrhagic and necrotic ependymoma of the ilium terminale. DISCUSSION Rectal pain may be caused by local disease processes or referred from distant sources by irritation of t h e pudendal nerves, which innervate the rectum and anus. A careful physical examination is critical to exclude local pathology. Local causes of proctalgia include thrombosed or infected external hemorrhoids, anal fissures, anorectal abscesses, anal fistulas, infectious or i n f l a m m a t o r y proctitis, fecal impaction, anal foreign bodies, and neoplasm. ~ Although many of these conditions may be evident on inspection or digital rectal examination, further evaluation with anoscopy or rectosigmoidoscopy may be necessary. Detection of submucosal or supralevator abscess may require pelvic sonography. Referred rectal pain may be caused by pelvic inflammatory disease, uterine malposition or myomas, prostatitis, c o l o n i c e n d o m e t r i o s i s , or nephrolithiasis, z History and physical e x a m i n a t i o n directed t o w a r d these organ systems will guide in the choice of further diagnostic tests. Several rectal pain syndromes of 146/202
u n c l e a r p a t h o l o g y have been described in the literature. Proctalgia fugax is characterized by recurrent paroxysms of anorectal pain lasting less than ten minutes. This syndrome is benign and infrequently causes patients to seek medical attention. ~ Tension myalgia of the pelvic floor describes a group of pain s y n d r o m e s o r i g i n a t i n g f r o m the muscles of the pelvic floor or their insertions, including the pyriformis syndrome, levator ani spasm, diaphragma pelvis spastica, and coccygodynia. Diagnosis may be made on rectal examination with the use of special maneuvers. 3 A rare familial syndrome of rectal, ocular, and sub~ maxillary pain associated with autonomic dysfunction also has been described. 4 Rectal pain also may originate in the sacral segments of the spinal cord or sacral nerve roots that mediate rectal sensation. Neoplasm, abscess, or i n f l a m m a t o r y processes of the conus medullaris may present with saddle anesthesia or pain in the sacral dermatomes, including the rectum. Associated loss of bowel and bladder function frequently occurs. The sacral nerve roots of the cauda equina may become entrapped in inflammatory reactions in the cerebrospinal fluid or compressed by tumor, abscess, or midline lumbosacral disk herniation. 5 These same nerve roots intermingle against the posterior pelvic wall in the sacral plexus and may be compressed by t u m o r or enlarged l y m p h nodes. 6 Lumbar puncture and magnetic resonance imaging scan of the lumbosacral spine or pelvis may be necessary to rule out these processes. Spinal SAH is an extremely rare cause of rectal pain. SAH arises from a spinal source in less than 1% of cases. 7 Presenting symptoms of spinal SAH include the sudden onset of back pain at the spinal level of rupture, followed by prominent meningeal signs, headache, and fever, s The combined presence of sciatica, headache, and SAH has been termed the Fincher syndrome. 9 Spinal SAH is most commonly caused by rupture of a vascular malformation, although it also m a y be a s s o c i a t e d w i t h trauma, anticoagulant therapy, blood dyscrasias, and tumor. Tumor is responsible for less than 1% of all spinal SAH, and ependymomas of the cauda equina are the Annals of Emergency Medicine
source of bleeding in 64% of these cases. 8 E p e n d y m o m a s are slowgrowing tumors arising from nests of ependymal cells in the region of the fourth ventricle, the central canal of the spinal cord, and the cauda equina (filum terminale). N e a r l y half of ependymomas arise from the cauda equina and are circumscribed, slowgrowing tumors that rarely spread far or show c y t o p l a s m i c malignancy. Symptoms are generally due to direct compression of surrounding structures by tumor. 1o The myxopapillary variant of ependymoma is unique to the cauda equina, u The cauda equina e p e n d y m o m a most c o m m o n l y presents with the insidious onset of intractable low b a c k p a i n a s s o c i a t e d w i t h paresthesias. Pain is exacerbated by the recumbent position and may chronically interfere with sleep. Weakness is the presenting sign in less than 10% of cases. In children, the most important signs are painful spinal rigidity and progressive scoliosis with or without pain. Genitourinary disturbances such as sphincter abnormalities, impotence, and priapism also may occur. Trauma or pregnancy may unmask the tumor. 9 Rare and unusual presentations include spinal SAH, symptomatic increased intracranial pressure, and simulation of bacterial meningitis.8,12 14 Bleeding as a complication of spinal ependyrnoma is seen most frequently in young patients (average age, 20 years), often after exercise or inconsequential trauma. 8 The tendency of cauda equina ependymomas to bleed is believed to be due to the delicate and highly vascular nature of the tumor. 14 The accepted method of t r e a t m e n t of cauda equina ependymomas is radical excision followed by local irradiation. A five-year survival rate of more than 50% has been d o c u m e n t e d in patients with this method of therapy. 1°
SUMMARY The symptoms of rectal pain, headache, stiff neck, and fever experienced by our patient were typical of spinal SAH. An emergency physician should be alert to the possibility of spinal SAH in any patient presenting with atypical low back or rectal pain, particularly when there is no history of trauma or evidence of rectal pathology. Awareness of spinal SAH as a possible complication of ependy19:2 February 1990
m o m a s of the cauda equina will allow rapid diagnosis and treatment of these relatively benign tumors. Consideration of the less-common causes of proctalgia is essential to formulate an appropriate diagnostic plan when a patient presents with this c o m m o n complaint. REFERENCES 1. Davis SM, Odom MH: Disorders of the anorectum, in Rosen P, Baker FJ, Barkin RM, et al (eds): Emergency Medicine, ed 2. St Louis, CV Mosby Co, 1988, p 1525.
4. Hayden R, Grossman M: Rectal, ocular, and s u b m a x i l l a r y pain. A m J Dis Child 1959; 97:479-482. 5. Adams RD, Victor M: Disease of the spinal cord, in Principles of Neurology. New York, McGraw-Hill, 1989, p 743-746. 6. Emsellem HA: Metastatic disease of the spine: Diagnosis and management. South Med J 1986; 79:1405 -I412. 7. Maurice-Williams RS: Spinal subarachnoid haemorrhage and spinal vascular malformations, in Subarachnoid Haemorrhage: Aneurysms and Vascular Malformations of the Central Nervous System. Bristol, England, IOP Publishing Ltd, 1987, p 350-352.
Elsevier, 1976, p 353-387. 10. Rubenstein LJ: Tumors of neuroglial origin/ ependymomas, in Tumors of the Central Nervous System, AFIP Atlas of Tumor Pathology, 2nd series, fascicle 6. Washington, DC, Armed Forces Institute of Pathology, 1972, p 104-126. 11. Sonneland PRL, Scheithauer BW, Onofrio BM: Myxopapillary ependymoma: A clinicopathologic and immunocytochemical study of 77 cases. Cancer 1985;56:883-893. 12. Iob I, Andrioli GC, Rigobello L, et ah An unusual onset of a spinal cord tumour: Sub arachnoid bleeding and papilledema. Neurochir 1980;23:112-116.
2. Peery WH: Proctalgia fugax: A clinical enigma. South Med J 1988;81:621-623.
8. Djindjian M, Djindjian R, Houdart R, et al: Subaraehnoid hemorrhage due to intraspinal tumors. Surg Neuro] 1978;9:223-229.
13. Gibberd FB, Ngon H, Swann GF: Hydrocephalus, subarachnoid haemorrhage and epend y m o m a s of the cauda equina. Clin Radiol 1972;23:422-426,
3. Sinaki M, Merritt JL, Stillwell GK: Tension myalgia of the pelvic floor. Mayo Clin Proc 1977;52: 717-722.
9. Fischer G, Tommasi M: Spinal ependymomas, in Vinken PJ, Bmyn GW, DeJong JM (eds): Handbook of Clinical Neurology. New York,
14. Okawara S: Ruptured spinal ependymoma simulating bacterial meningitis. Arch Neurol 1983;40:54-55.
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An Unusual Case of Proctalgia We report the case of a 21-year-old man with the sudden onset of severe proctalgia followed by headaches and fever. The patient had a lumbar puncture consistent with subarachnoid hemorrhage. After a normal fourvessel cerebral arteriogram, a myelogram and computed tomography scan of the spine were performed and revealed a mass lesion of the L1 level. Pathologic evaluation after resection of the mass resulted in a diagnosis of hemorrhage from a myxopapillary ependymoma. These relatively benign tumors of the cauda equina-filum terminale respond well to treatment. The differential diagnosis of proctalgia is reviewed, and the need for emergency physicians to consider spinal subarachnoid hemorrhage when a patient presents with the acute onset of rectal pain is emphasized. [Rappaport B, Emsellem HA, Shesser R, Millstein E: An unusual case of proctalgia. Ann Emerg Med February 1990;19:201-203.] INTRODUCTION Rectal pain or proctalgia is a relatively c o m m o n complaint in emergency practice and is rarely due to a life-threatening illness. Proctalgia is most often caused by hemorrhoids, obstipation, and anorectal fissures.1 Pain experienced as arising from the rectum m a y also be referred from distant structures. We present the case of a patient with an unusual cause of severe rectal pain that demonstrates the importance of fully considering all conditions that can cause proctalgia.
Bob Rappaport, MD* Helene A Emsellem, MD* Robert Shesser, MDt Washington, DC Ellen Millstein, MD:~ North Chicago, Illinois From the Depar.tments of Neurology* and Emergency Medicine,t The George Washington University Medical Center, Washington, DC; and Abbott Laboratories, North Chicago, IIlinois.¢ Received for publication April 13, 1989. Revision received July 13, 1989. Accepted for publication August 2, 1989. Address for reprints: Helene A Emsellem, MD, Department of Neurology, Room 7-407, 2150 Pennsylvania Avenue, NW, Washington, DC 20037.
CASE REPORT A 21-year-old m a n presented to the emergency department complaining of severe, deep peroneal and rectal pain. He had been in excellent health until four days earlier when he noted the sudden onset of pain deep in the rectal region. He had had a pilonidal cyst repair several m o n t h s earlier, and the pain was believed to be secondary to that procedure. Rectal examination, lumbosacral spinal radiography, and a CBC were normal, and the patient was discharged. The rectal discomfort intensified to the point that he was reluctant to move his legs for fear of precipitating the pain. He developed a bifrontal headache and returned to the ED two days later w h e n he became febrile. The patient's history was remarkable for only the pilonidal cyst repair, which was reportedly associated with exploration no deeper than the coccygeal-sacral fascia. He was not taking any medications, and the family history was unremarkable. Physical examination revealed a well-developed, ill-appearing man. His blood pressure was 120/70 m m Hg; temperature, 39.2 C; and pulse, 72. The neck was stiff. There was no spinal tenderness, but bilateral straight legraising produced low back pain. Rectal examination revealed no focal tenderness or mass. The remainder of the general medical examination was normal. The patient was alert and oriented with normal cognitive function. He complained of photophobia, but there were no focal cranial nerve abnormalities. The sensory examination was normal. Strength was normal in the upper and distal lower extremities. Hip flexion was limited to 5 degrees w i t h o u t resistance secondary to pain. The reflexes were 1 + throughout, and the plantar response was flexor. Cerebellar function was normal
19:2 February 1990
Annals of Emergency Medicine
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PROCTALGIA Rappaport et al
in the upper extremities and could not be evaluated in the lower extremities. The patient could not ambulate. A head computed tomography (CT) scan showed small ventricles, although sulci were seen clearly and there was no mass effect. A lumbar puncture revealed an opening pressure of 270 c m H20 and grossly bloody fluid with a xanthochromic s u p e r n a t a n t after c e n t r i f u g a t i o n . There were 90,000 R B C / m m 3, 300 WBC/mrn a (48% polymorphonuclear leukocytes, 52% lymphocytes), 270 mg/dL protein, and 29 mg/dL glucose with a simultaneous serum glucose of 107 mg/dL. The patient was placed on aneurysm precautions with a presumed diagnosis of subarachnoid hemorrhage (SAH). Four-vessel cerebral angiography performed the next morning was normal. A C T scan of the spine after myelography revealed a round lesion of the cauda equina at the L1 level. At thoracolumbar laminectorny the next day, a hemorrhagic 1.5 x 1 cm mass was resected from the L1 level of the cauda equina. P a t h o l o g i c e v a l u a t i o n revealed a hemorrhagic and necrotic ependymoma of the ilium terminale. DISCUSSION Rectal pain may be caused by local disease processes or referred from distant sources by irritation of t h e pudendal nerves, which innervate the rectum and anus. A careful physical examination is critical to exclude local pathology. Local causes of proctalgia include thrombosed or infected external hemorrhoids, anal fissures, anorectal abscesses, anal fistulas, infectious or i n f l a m m a t o r y proctitis, fecal impaction, anal foreign bodies, and neoplasm. ~ Although many of these conditions may be evident on inspection or digital rectal examination, further evaluation with anoscopy or rectosigmoidoscopy may be necessary. Detection of submucosal or supralevator abscess may require pelvic sonography. Referred rectal pain may be caused by pelvic inflammatory disease, uterine malposition or myomas, prostatitis, c o l o n i c e n d o m e t r i o s i s , or nephrolithiasis, z History and physical e x a m i n a t i o n directed t o w a r d these organ systems will guide in the choice of further diagnostic tests. Several rectal pain syndromes of 146/202
u n c l e a r p a t h o l o g y have been described in the literature. Proctalgia fugax is characterized by recurrent paroxysms of anorectal pain lasting less than ten minutes. This syndrome is benign and infrequently causes patients to seek medical attention. ~ Tension myalgia of the pelvic floor describes a group of pain s y n d r o m e s o r i g i n a t i n g f r o m the muscles of the pelvic floor or their insertions, including the pyriformis syndrome, levator ani spasm, diaphragma pelvis spastica, and coccygodynia. Diagnosis may be made on rectal examination with the use of special maneuvers. 3 A rare familial syndrome of rectal, ocular, and sub~ maxillary pain associated with autonomic dysfunction also has been described. 4 Rectal pain also may originate in the sacral segments of the spinal cord or sacral nerve roots that mediate rectal sensation. Neoplasm, abscess, or i n f l a m m a t o r y processes of the conus medullaris may present with saddle anesthesia or pain in the sacral dermatomes, including the rectum. Associated loss of bowel and bladder function frequently occurs. The sacral nerve roots of the cauda equina may become entrapped in inflammatory reactions in the cerebrospinal fluid or compressed by tumor, abscess, or midline lumbosacral disk herniation. 5 These same nerve roots intermingle against the posterior pelvic wall in the sacral plexus and may be compressed by t u m o r or enlarged l y m p h nodes. 6 Lumbar puncture and magnetic resonance imaging scan of the lumbosacral spine or pelvis may be necessary to rule out these processes. Spinal SAH is an extremely rare cause of rectal pain. SAH arises from a spinal source in less than 1% of cases. 7 Presenting symptoms of spinal SAH include the sudden onset of back pain at the spinal level of rupture, followed by prominent meningeal signs, headache, and fever, s The combined presence of sciatica, headache, and SAH has been termed the Fincher syndrome. 9 Spinal SAH is most commonly caused by rupture of a vascular malformation, although it also m a y be a s s o c i a t e d w i t h trauma, anticoagulant therapy, blood dyscrasias, and tumor. Tumor is responsible for less than 1% of all spinal SAH, and ependymomas of the cauda equina are the Annals of Emergency Medicine
source of bleeding in 64% of these cases. 8 E p e n d y m o m a s are slowgrowing tumors arising from nests of ependymal cells in the region of the fourth ventricle, the central canal of the spinal cord, and the cauda equina (filum terminale). N e a r l y half of ependymomas arise from the cauda equina and are circumscribed, slowgrowing tumors that rarely spread far or show c y t o p l a s m i c malignancy. Symptoms are generally due to direct compression of surrounding structures by tumor. 1o The myxopapillary variant of ependymoma is unique to the cauda equina, u The cauda equina e p e n d y m o m a most c o m m o n l y presents with the insidious onset of intractable low b a c k p a i n a s s o c i a t e d w i t h paresthesias. Pain is exacerbated by the recumbent position and may chronically interfere with sleep. Weakness is the presenting sign in less than 10% of cases. In children, the most important signs are painful spinal rigidity and progressive scoliosis with or without pain. Genitourinary disturbances such as sphincter abnormalities, impotence, and priapism also may occur. Trauma or pregnancy may unmask the tumor. 9 Rare and unusual presentations include spinal SAH, symptomatic increased intracranial pressure, and simulation of bacterial meningitis.8,12 14 Bleeding as a complication of spinal ependyrnoma is seen most frequently in young patients (average age, 20 years), often after exercise or inconsequential trauma. 8 The tendency of cauda equina ependymomas to bleed is believed to be due to the delicate and highly vascular nature of the tumor. 14 The accepted method of t r e a t m e n t of cauda equina ependymomas is radical excision followed by local irradiation. A five-year survival rate of more than 50% has been d o c u m e n t e d in patients with this method of therapy. 1°
SUMMARY The symptoms of rectal pain, headache, stiff neck, and fever experienced by our patient were typical of spinal SAH. An emergency physician should be alert to the possibility of spinal SAH in any patient presenting with atypical low back or rectal pain, particularly when there is no history of trauma or evidence of rectal pathology. Awareness of spinal SAH as a possible complication of ependy19:2 February 1990
m o m a s of the cauda equina will allow rapid diagnosis and treatment of these relatively benign tumors. Consideration of the less-common causes of proctalgia is essential to formulate an appropriate diagnostic plan when a patient presents with this c o m m o n complaint. REFERENCES 1. Davis SM, Odom MH: Disorders of the anorectum, in Rosen P, Baker FJ, Barkin RM, et al (eds): Emergency Medicine, ed 2. St Louis, CV Mosby Co, 1988, p 1525.
4. Hayden R, Grossman M: Rectal, ocular, and s u b m a x i l l a r y pain. A m J Dis Child 1959; 97:479-482. 5. Adams RD, Victor M: Disease of the spinal cord, in Principles of Neurology. New York, McGraw-Hill, 1989, p 743-746. 6. Emsellem HA: Metastatic disease of the spine: Diagnosis and management. South Med J 1986; 79:1405 -I412. 7. Maurice-Williams RS: Spinal subarachnoid haemorrhage and spinal vascular malformations, in Subarachnoid Haemorrhage: Aneurysms and Vascular Malformations of the Central Nervous System. Bristol, England, IOP Publishing Ltd, 1987, p 350-352.
Elsevier, 1976, p 353-387. 10. Rubenstein LJ: Tumors of neuroglial origin/ ependymomas, in Tumors of the Central Nervous System, AFIP Atlas of Tumor Pathology, 2nd series, fascicle 6. Washington, DC, Armed Forces Institute of Pathology, 1972, p 104-126. 11. Sonneland PRL, Scheithauer BW, Onofrio BM: Myxopapillary ependymoma: A clinicopathologic and immunocytochemical study of 77 cases. Cancer 1985;56:883-893. 12. Iob I, Andrioli GC, Rigobello L, et ah An unusual onset of a spinal cord tumour: Sub arachnoid bleeding and papilledema. Neurochir 1980;23:112-116.
2. Peery WH: Proctalgia fugax: A clinical enigma. South Med J 1988;81:621-623.
8. Djindjian M, Djindjian R, Houdart R, et al: Subaraehnoid hemorrhage due to intraspinal tumors. Surg Neuro] 1978;9:223-229.
13. Gibberd FB, Ngon H, Swann GF: Hydrocephalus, subarachnoid haemorrhage and epend y m o m a s of the cauda equina. Clin Radiol 1972;23:422-426,
3. Sinaki M, Merritt JL, Stillwell GK: Tension myalgia of the pelvic floor. Mayo Clin Proc 1977;52: 717-722.
9. Fischer G, Tommasi M: Spinal ependymomas, in Vinken PJ, Bmyn GW, DeJong JM (eds): Handbook of Clinical Neurology. New York,
14. Okawara S: Ruptured spinal ependymoma simulating bacterial meningitis. Arch Neurol 1983;40:54-55.
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