Childs Nerv Syst DOI 10.1007/s00381-015-2688-4
CASE REPORT
Spinal extradural arachnoid cyst presenting as recurrent abdominal pain John M. Kerr 1,2,3 & Henry Ukpeh 4 & Paul Steinbok 1,2
Received: 9 March 2015 / Accepted: 16 March 2015 # Springer-Verlag Berlin Heidelberg 2015
Abstract Introduction Spinal arachnoid cysts are an infrequently reported cause of abdominal pain in children. In this report, we describe the unusual case of an extradural arachnoid cyst presenting as recurrent abdominal pain in a pediatric patient without any signs of cord or nerve root compression. Case report A 14-year-old girl with recurrent abdominal pain as the only symptom of a spinal extradural arachnoid cyst is reported. The patient was incidentally diagnosed with an intraspinal mass on abdominal computed tomography (CT) during the course of investigating her abdominal pain. Magnetic resonance (MR) imaging confirmed the diagnosis of a T11–L2 extradural arachnoid cyst. After resection of the T11–L2 arachnoid cyst, the patient’s abdominal pain resolved. To our knowledge, this is the first report describing abdominal pain as the sole presenting feature of a spinal arachnoid cyst in the pediatric population. Conclusion This case illustrates that abdominal pain may be the first and only presentation of spinal arachnoid cysts in children. Spinal pathology should be considered in the differential
* Paul Steinbok [email protected] 1
Division of Pediatric Neurosurgery, Department of Surgery, University of British Columbia, 4480 Oak St, #K3-159, Vancouver, BC V6H 3V4, Canada
2
British Columbia Children’s Hospital, Vancouver, Canada
3
Medical Undergraduate Program, Faculty of Medicine, University of British Columbia, Vancouver, Canada
4
Department of Pediatrics, University of British Columbia, Trail, Canada
diagnosis of unexplained abdominal pain, even when there are no other symptoms of spinal disease.
Keywords Spinal arachnoid cyst . Abdominal pain . Diagnosis . Treatment . Pediatric . Symptoms
Introduction Spinal extradural arachnoid cysts (EDACs) are rare and benign intraspinal lesions. These cysts are thought to develop from congenital defects in the spinal dura mater [6, 10] which give rise to the formation of arachnoid diverticula. These defects are commonly located along the posterolateral region of the spinal canal in association with the nerve root sleeve [6, 15]. Progressive expansion of the cyst wall by cerebrospinal fluid (CSF) flow may lead to compression of the spinal cord and nerve roots and vertebral remodeling [2, 6, 10, 12, 15]. In general, the clinical presentation of EDAC is related to the location of the cyst and degree of spinal cord and/or nerve root compression [10]. EDACs have a predilection for the thoracic spine but can develop anywhere along the spinal canal. Thoracic lesions typically produce symptoms of spastic paraparesis [2, 6, 10, 12, 15], whereas back pain, radiculopathy, and bowel and bladder dysfunction are more common in lumbo-sacral cysts [6, 12]. Cervical lesions can present with symptoms of Horner’s syndrome, spastic quadriparesis, and sensory disturbances of the upper limbs [6]. EDACs are diagnosed primarily by magnetic resonance (MR) imaging. These lesions have a characteristic CSF-like appearance on T2-weighted imaging that aids in diagnosis [9]. EDACs are often incidental findings, discovered during the course of investigating back pain, radiculopathy, or myelopathy [5]. In this report, we describe a rare case of a spinal
Childs Nerv Syst
extradural arachnoid cyst presenting as recurrent abdominal pain without other findings in a pediatric patient.
Case presentation History This 14-year-old girl had a 5-year history of intermittent abdominal pain that started before menarche. The pain was sharp in character and poorly localized but typically occurred below the umbilicus. She described the pain as non-radiating, with no clear precipitating or relieving factors, and the pain was severe enough to limit activities in sports and school. The episodes would last 20–30 min and were usually quite severe (8.5/10 pain scale). There was no associated vomiting or changes in bowel function (no constipation or diarrhea). There was no history of weight loss, morning stiffness, bloody stools, joint pains, or unexplained fevers. Menarche was at age 12 and regular. Bowel and bladder functions were both normal. She denied having any back pain and did not complain of any numbness, stiffness, or weakness in the lower limbs. Past medical history included only a prior Achilles tendon injury that left her with some residual ankle weakness (grade 4/5). She had no known allergies, but did have intolerance to wheat. Her primary care physician had previously investigated her abdominal pain. Vital signs and physical exam were unremarkable except for some mild tenderness to deep palpation of the lumbar region. Laboratory investigations included complete blood count, electrolytes, urinalysis, liver function tests, BUN, creatinine, calcium, phosphorous, ESR, and anti-tissue transglutaminase IgA levels, all of which were normal. Several imaging studies were performed to rule out an organic cause of abdominal pain. Abdominal and pelvic ultrasound was unremarkable except for borderline hepatosplenomegaly which was not appreciated on physical examination. A CT scan of the abdomen and pelvis revealed no intra-abdominal cause for abdominal pain; however, there was an incidental finding of scalloping of the T11–L1 vertebrae and distension of the spinal canal which was consistent with an intra-spinal lesion. She was subsequently referred to our institution for further evaluation and management. Examination On examination, the only detectable deficit was minimal weakness in the right ankle and toe dorsiflexor and inability to heel walk (related to previous injury). There was normal tone, strength, reflexes, and muscle bulk in the lower limbs. Plantar responses were down going bilaterally. Sensory examination was normal. Motor and reflex testing in the upper limbs was normal. Gait was normal. The remainder of the neurological examination was unremarkable. Palpation of the back did not elicit any spinal tenderness.
Neuroimaging MR imaging of the spine showed a large, dorsally located EDAC extending from the T10/11 border to the L2 vertebral body (Fig. 1a, c). The lesion terminated at the level of the conus medullaris, above the cauda equina. It extended bilaterally and symmetrically through the T11, T12, and L1 intervertebral foramina. Marked anterior flattening and compression of the spinal cord was seen from T11 to L1, along with thinning of the posterior vertebral elements and expansion of the lumbar spinal canal. No definite signal abnormality was seen in the compressed cord. Operation After much discussion as to whether or not the cyst was responsible for the abdominal pain, the patient underwent T11–T12 laminoplasty. Superior reflection of the T11 and T12 laminae revealed a thin-walled cyst in the extradural space. The anterior cyst wall had a communicating pedicle emerging from the left infero-lateral margin of T11 through a defect in the dura. Clear CSF-like fluid was aspirated and sent for laboratory analysis. Dissection of the cyst wall was then carried out inferiorly from the origin of the pedicle to the lower border of T12 where the distinction between the arachnoid and dura became clear. The cyst wall was then peeled away from the dura. The posterior dural wall, including the defect through which the cyst had originated, was sutured closed in a water-tight fashion and overlaid with a graft of the excised cyst wall. The laminae were returned to their original position and sutured in place. Histological examination Histopathological analysis showed a single layer of epithelial membrane antigen (EMA)-positive arachnoid cells lining dense collagenous connective tissue, consistent with the diagnosis of an EDAC (Fig. 1e, f). No hemosiderin-laden macrophages were present. Post-operative course At 3-month follow-up, the patient’s abdominal pain had completely resolved with no more episodes since her surgery. MR imaging completed 10 months post-op showed complete resolution of the cyst and reconstitution of the lower spinal canal structure (Fig. 1b, d). As of her last follow-up (18 months post-op), the patient remained symptom free.
Discussion Recurrent abdominal pain (RAP) is the most common chronic pain disorder in the pediatric population [13], affecting
Childs Nerv Syst Fig. 1 Pre-operative T2weighted axial (a) and sagittal (c) MR imaging of the spine showing an extradural arachnoid cyst extending from the T10/T11 border to the mid-L2 vertebral body. b, d Spinal MR imaging at 10 months follow-up. T2-weighted axial (b) and sagittal (d) images show complete resolution of the arachnoid cyst and reconstitution of the spinal cord anatomy. Photomicrographs of the excised cyst wall showing dense collagenous connective tissue (asterisks, e) underlying a single layer of arachnoid meningothelial cells (arrows, f). Hematoxylin and eosin (e) and epithelial membrane antigen (EMA) stain (f) (×100 magnification)
approximately 10–15 % of all children. Spinal cord lesions, like the one seen in this case, are a rare but important cause of RAP. In reviewing the literature, we found no case of EDAC presenting with abdominal pain. We did find one report describing abdominal pain as a symptom of an intramedullary cyst by Aithala et al. [1]. In their report, they described a 7-year-old male presenting with sudden onset of lower limb weakness and severe abdominal pain secondary to a thoracic intramedullary cyst. The cyst was successfully treated with fenestration, and the abdominal pain and associated neurological deficit had completely subsided by the second day post-operatively. An unusual feature of our case was the absence of any signs or symptoms of myelopathy, radiculopathy, or localized spinal pain. Patients with symptomatic spinal EDAC typically present with deficits related to compression of the spinal long tracts and/or nerve roots [6, 15]. The most common presenting features are spastic paraparesis, back pain, radiculopathy, sensory deficits, gait disturbance, and bowel and bladder dysfunction [2, 15]. Asymptomatic cases of EDAC are relatively
rare. At least 68 % of pediatric patients with spinal arachnoid cysts will present with one sign of radiculopathy or myelopathy, with only 10 % of patients being asymptomatic at the time of diagnosis [2]. During our examination of this child, we could not identify any objective signs or symptoms directly related to the spine. Careful neurological examination elicited no sensory deficits, weakness, or reflex abnormalities. On questioning, the patient denied ever having any back pain or incontinence. The only detectable deficit was right ankle and toe dorsiflexor weakness that was related to a previous Achilles tendon injury. It is not surprising then, in the absence of any neurological symptoms, that the cause of abdominal pain went undiagnosed for so long. Surgery is the mainstay of treatment of symptomatic spinal arachnoid cysts, but conservative management is preferred in asymptomatic cases [9, 10]. As such, atypical presentations of EDAC can pose a significant dilemma. Because abdominal pain was the sole presenting feature of our patient’s EDAC, it was not clear whether surgery would resolve her symptoms
Childs Nerv Syst
and this was discussed preoperatively. However, following resection, our patient experienced immediate resolution of her symptoms and has had no further recurrences following resection of her cyst. In light of these results, we concluded that the cyst was the cause of her symptoms. Similar cases have been reported in the literature where abdominal pain was the sole presenting feature of thoracic cord tumors in children [3, 7, 8, 14]. In these cases, recurrent abdominal pain was initially misdiagnosed as abdominal epilepsy [7], functional abdominal pain [3], psychogenic abdominal pain [8], and irritable bowel syndrome [14]. All patients experienced worsening of symptoms over time, and two developed a new scoliosis or neurological deficit before the correct diagnosis was made. In all cases, there was immediate and complete resolution of the abdominal pain following surgical decompression or resection of the lesion. The abdominal pain in our case was likely due to compression of the nerve roots or spinothalamic tracts innervating the abdominal wall and viscera [17]. Radicular or nerve root pain is caused by tethering or compression of the dorsal root ganglion and is a common manifestation in patients with spinal EDAC. Given the dermatomal distribution of the affected spinal segments (T11–L2), it is possible that her pain could be radiculopathic in origin. Irritation of these nerve roots would produce abdominal pain below the umbilicus, which is in keeping with the patient’s history. Funicular or central pain is another possibility. Funicular pain is a poorly localized pain thought to arise from irritation of spinothalamic tracts or posterior columns. Funicular pain from a lesion anywhere in the spinal cord up to the foramen magnum can result in abdominal pain. It typically presents as a burning quality with a superficial sharp, stabbing sensation in patients with intramedullary tumors. This type of pain is less likely, however, since it is usually associated with intramedullary lesions and is only rarely seen in extramedullary lesions [4]. Several factors likely contributed to the delay in this patient’s diagnosis. First, there were no red flags in the patient’s history or physical examination to suggest an organic cause of abdominal pain. Organic causes of RAP tend to be well localized away from the umbilicus (Apley’s Law) and present with vomiting or associated changes in bowel function [11]. This patient’s pain was poorly localized and had no temporal association. All physical examination findings and investigations performed prior to abdominal CT imaging were normal. Secondly, in the absence of neurological symptoms, a spinal etiology is less likely to be pursued. Finally, there are only a small number of case reports in the literature that have found an association between recurrent abdominal pain and spinal cord lesions in children. Because of this, much of the pediatric literature does not include spinal disease in the differential
diagnosis of recurrent abdominal pain [11, 13, 16]. Therefore, pediatricians and other primary care providers, whose patient population includes children with RAP, may not be aware of this association.
Conclusion In summary, this case demonstrates that abdominal pain may be the sole presenting feature of a spinal EDAC in children. In this case, excision of the cyst resulted in complete and long-term resolution of the patient’s abdominal pain, indicating that surgical intervention was appropriate. From the pediatrician’s perspective, the possibility of intraspinal disorders should be considered in children with unexplained RAP, even in the absence of neurological signs or symptoms. Acknowledgments The authors wish to thank Mr. Ross Hengel for his assistance with manuscript editing, Dr. Paul Thiessen for his guidance with submission of the manuscript, and Dr. Glenda Hendson from the Department of Anatomical Pathology at BC Children’s Hospital for preparing the photomicrographs for this manuscript. Conflict of interest The authors have no conflict of interest to disclose.
References 1.
2.
3.
4. 5.
6. 7.
8.
9.
10.
Aithala GR, Sztriha L, Amirlak I, Devadas K, Ohlsson I (1999) Spinal arachnoid cyst with weakness in the limbs and abdominal pain. Pediatr Neurol 20:155–156 Bond AE, Zada G, Bowen I, McComb JG, Krieger MD (2012) Spinal arachnoid cysts in the pediatric population: report of 31 cases and a review of the literature. J Neurosurg Pediatr 9: 432–441 Buck E, Bodensteiner J (1981) Thoracic cord tumor appearing as recurrent abdominal pain. Am J Dis Child 135:574–575 Byrne TN, Benzel EC, Waxman SG (2000) Diseases of the spine and spinal cord. Oxford University Press, New York Chang IC, Chou MC, Bell WR, Lin ZI (2004) Spinal cord compression caused by extradural arachnoid cysts. Clinical examples and review. Pediatr Neurosurg 40:70–74 Cloward RB (1968) Congenital spinal extradural cysts: case report with review of literature. Ann Surg 168:851–864 Drexler DL, Grill BB, Ashwal S (1989) Spinal cord tumorassociated syrinx mimicking abdominal epilepsy: a rare cause of childhood abdominal pain. J Pediatr Gastroenterol Nutr 9: 524–527 Eeg-Olofsson O, Carlsson E, Jeppsson S (1981) Recurrent abdominal pains as the first symptom of a spinal cord tumor. Acta Paediatr Scand 70:595–597 Liu JK, Cole CD, Kan P, Schmidt MH (2007) Spinal extradural arachnoid cysts: clinical, radiological, and surgical features. Neurosurg Focus 22:E6 Myles ST, Hamilton MG (2008) Intraspinal congenital cysts: neurenteric, arachnoid, dermoid, epidermoid. In: Albright AL, Pollack IF, Adelson PD (eds) Principles and practice of
Childs Nerv Syst
11. 12.
13. 14.
pediatric neurosurgery, 2nd edn. Thieme Medical Publishers, New York Oberlander TF, Rappaport LA (1993) Recurrent abdominal pain during childhood. Pediatr Rev 14:313–319 Oh JK, Lee DY, Kim TY, Yi S, Ha Y, Kim KN, Shin H, Kim DS, Yoon DH D2012] Thoracolumbar extradural arachnoid cysts: a study of 14 consecutive cases. Acta Neurochir DWien] 154:341– 348, discussion 348 Olson A (1987) Recurrent abdominal pain: an approach to diagnosis and management. Pediatr Ann 16:834–836, 838–840, 842 Robertson PL (1992) Atypical presentations of spinal cord tumors in children. J Child Neurol 7:360–363
15.
16.
17.
Santos de Oliveira R, Amato MC, Santos MV, Simao GN, Machado HR (2007) Extradural arachnoid cysts in children. Childs Nerv Syst 23:1233–1238 Sreedharan R, Liacouras CA (2011) Major symptoms and signs of digestive tract disorders. In: Kliegman RM, Stanton BF, Geme JWS, Schor NF, Behrman RE (eds) Nelson textbook of pediatrics, 19th edn. Elsevier Saunders, Philadelphia Woolsey RM, Martin DS (2003) Acute nontraumatic myelopathies. In: Lin VW, Cardenas DD, Cutter NC, Frost FS, Hammond MC, Lindblom LB, Perkash I, Waters R, Woolsey RM (eds) Spinal cord medicine: principles and practice. Demos Medical Publishing, New York
CASE REPORT
Spinal extradural arachnoid cyst presenting as recurrent abdominal pain John M. Kerr 1,2,3 & Henry Ukpeh 4 & Paul Steinbok 1,2
Received: 9 March 2015 / Accepted: 16 March 2015 # Springer-Verlag Berlin Heidelberg 2015
Abstract Introduction Spinal arachnoid cysts are an infrequently reported cause of abdominal pain in children. In this report, we describe the unusual case of an extradural arachnoid cyst presenting as recurrent abdominal pain in a pediatric patient without any signs of cord or nerve root compression. Case report A 14-year-old girl with recurrent abdominal pain as the only symptom of a spinal extradural arachnoid cyst is reported. The patient was incidentally diagnosed with an intraspinal mass on abdominal computed tomography (CT) during the course of investigating her abdominal pain. Magnetic resonance (MR) imaging confirmed the diagnosis of a T11–L2 extradural arachnoid cyst. After resection of the T11–L2 arachnoid cyst, the patient’s abdominal pain resolved. To our knowledge, this is the first report describing abdominal pain as the sole presenting feature of a spinal arachnoid cyst in the pediatric population. Conclusion This case illustrates that abdominal pain may be the first and only presentation of spinal arachnoid cysts in children. Spinal pathology should be considered in the differential
* Paul Steinbok [email protected] 1
Division of Pediatric Neurosurgery, Department of Surgery, University of British Columbia, 4480 Oak St, #K3-159, Vancouver, BC V6H 3V4, Canada
2
British Columbia Children’s Hospital, Vancouver, Canada
3
Medical Undergraduate Program, Faculty of Medicine, University of British Columbia, Vancouver, Canada
4
Department of Pediatrics, University of British Columbia, Trail, Canada
diagnosis of unexplained abdominal pain, even when there are no other symptoms of spinal disease.
Keywords Spinal arachnoid cyst . Abdominal pain . Diagnosis . Treatment . Pediatric . Symptoms
Introduction Spinal extradural arachnoid cysts (EDACs) are rare and benign intraspinal lesions. These cysts are thought to develop from congenital defects in the spinal dura mater [6, 10] which give rise to the formation of arachnoid diverticula. These defects are commonly located along the posterolateral region of the spinal canal in association with the nerve root sleeve [6, 15]. Progressive expansion of the cyst wall by cerebrospinal fluid (CSF) flow may lead to compression of the spinal cord and nerve roots and vertebral remodeling [2, 6, 10, 12, 15]. In general, the clinical presentation of EDAC is related to the location of the cyst and degree of spinal cord and/or nerve root compression [10]. EDACs have a predilection for the thoracic spine but can develop anywhere along the spinal canal. Thoracic lesions typically produce symptoms of spastic paraparesis [2, 6, 10, 12, 15], whereas back pain, radiculopathy, and bowel and bladder dysfunction are more common in lumbo-sacral cysts [6, 12]. Cervical lesions can present with symptoms of Horner’s syndrome, spastic quadriparesis, and sensory disturbances of the upper limbs [6]. EDACs are diagnosed primarily by magnetic resonance (MR) imaging. These lesions have a characteristic CSF-like appearance on T2-weighted imaging that aids in diagnosis [9]. EDACs are often incidental findings, discovered during the course of investigating back pain, radiculopathy, or myelopathy [5]. In this report, we describe a rare case of a spinal
Childs Nerv Syst
extradural arachnoid cyst presenting as recurrent abdominal pain without other findings in a pediatric patient.
Case presentation History This 14-year-old girl had a 5-year history of intermittent abdominal pain that started before menarche. The pain was sharp in character and poorly localized but typically occurred below the umbilicus. She described the pain as non-radiating, with no clear precipitating or relieving factors, and the pain was severe enough to limit activities in sports and school. The episodes would last 20–30 min and were usually quite severe (8.5/10 pain scale). There was no associated vomiting or changes in bowel function (no constipation or diarrhea). There was no history of weight loss, morning stiffness, bloody stools, joint pains, or unexplained fevers. Menarche was at age 12 and regular. Bowel and bladder functions were both normal. She denied having any back pain and did not complain of any numbness, stiffness, or weakness in the lower limbs. Past medical history included only a prior Achilles tendon injury that left her with some residual ankle weakness (grade 4/5). She had no known allergies, but did have intolerance to wheat. Her primary care physician had previously investigated her abdominal pain. Vital signs and physical exam were unremarkable except for some mild tenderness to deep palpation of the lumbar region. Laboratory investigations included complete blood count, electrolytes, urinalysis, liver function tests, BUN, creatinine, calcium, phosphorous, ESR, and anti-tissue transglutaminase IgA levels, all of which were normal. Several imaging studies were performed to rule out an organic cause of abdominal pain. Abdominal and pelvic ultrasound was unremarkable except for borderline hepatosplenomegaly which was not appreciated on physical examination. A CT scan of the abdomen and pelvis revealed no intra-abdominal cause for abdominal pain; however, there was an incidental finding of scalloping of the T11–L1 vertebrae and distension of the spinal canal which was consistent with an intra-spinal lesion. She was subsequently referred to our institution for further evaluation and management. Examination On examination, the only detectable deficit was minimal weakness in the right ankle and toe dorsiflexor and inability to heel walk (related to previous injury). There was normal tone, strength, reflexes, and muscle bulk in the lower limbs. Plantar responses were down going bilaterally. Sensory examination was normal. Motor and reflex testing in the upper limbs was normal. Gait was normal. The remainder of the neurological examination was unremarkable. Palpation of the back did not elicit any spinal tenderness.
Neuroimaging MR imaging of the spine showed a large, dorsally located EDAC extending from the T10/11 border to the L2 vertebral body (Fig. 1a, c). The lesion terminated at the level of the conus medullaris, above the cauda equina. It extended bilaterally and symmetrically through the T11, T12, and L1 intervertebral foramina. Marked anterior flattening and compression of the spinal cord was seen from T11 to L1, along with thinning of the posterior vertebral elements and expansion of the lumbar spinal canal. No definite signal abnormality was seen in the compressed cord. Operation After much discussion as to whether or not the cyst was responsible for the abdominal pain, the patient underwent T11–T12 laminoplasty. Superior reflection of the T11 and T12 laminae revealed a thin-walled cyst in the extradural space. The anterior cyst wall had a communicating pedicle emerging from the left infero-lateral margin of T11 through a defect in the dura. Clear CSF-like fluid was aspirated and sent for laboratory analysis. Dissection of the cyst wall was then carried out inferiorly from the origin of the pedicle to the lower border of T12 where the distinction between the arachnoid and dura became clear. The cyst wall was then peeled away from the dura. The posterior dural wall, including the defect through which the cyst had originated, was sutured closed in a water-tight fashion and overlaid with a graft of the excised cyst wall. The laminae were returned to their original position and sutured in place. Histological examination Histopathological analysis showed a single layer of epithelial membrane antigen (EMA)-positive arachnoid cells lining dense collagenous connective tissue, consistent with the diagnosis of an EDAC (Fig. 1e, f). No hemosiderin-laden macrophages were present. Post-operative course At 3-month follow-up, the patient’s abdominal pain had completely resolved with no more episodes since her surgery. MR imaging completed 10 months post-op showed complete resolution of the cyst and reconstitution of the lower spinal canal structure (Fig. 1b, d). As of her last follow-up (18 months post-op), the patient remained symptom free.
Discussion Recurrent abdominal pain (RAP) is the most common chronic pain disorder in the pediatric population [13], affecting
Childs Nerv Syst Fig. 1 Pre-operative T2weighted axial (a) and sagittal (c) MR imaging of the spine showing an extradural arachnoid cyst extending from the T10/T11 border to the mid-L2 vertebral body. b, d Spinal MR imaging at 10 months follow-up. T2-weighted axial (b) and sagittal (d) images show complete resolution of the arachnoid cyst and reconstitution of the spinal cord anatomy. Photomicrographs of the excised cyst wall showing dense collagenous connective tissue (asterisks, e) underlying a single layer of arachnoid meningothelial cells (arrows, f). Hematoxylin and eosin (e) and epithelial membrane antigen (EMA) stain (f) (×100 magnification)
approximately 10–15 % of all children. Spinal cord lesions, like the one seen in this case, are a rare but important cause of RAP. In reviewing the literature, we found no case of EDAC presenting with abdominal pain. We did find one report describing abdominal pain as a symptom of an intramedullary cyst by Aithala et al. [1]. In their report, they described a 7-year-old male presenting with sudden onset of lower limb weakness and severe abdominal pain secondary to a thoracic intramedullary cyst. The cyst was successfully treated with fenestration, and the abdominal pain and associated neurological deficit had completely subsided by the second day post-operatively. An unusual feature of our case was the absence of any signs or symptoms of myelopathy, radiculopathy, or localized spinal pain. Patients with symptomatic spinal EDAC typically present with deficits related to compression of the spinal long tracts and/or nerve roots [6, 15]. The most common presenting features are spastic paraparesis, back pain, radiculopathy, sensory deficits, gait disturbance, and bowel and bladder dysfunction [2, 15]. Asymptomatic cases of EDAC are relatively
rare. At least 68 % of pediatric patients with spinal arachnoid cysts will present with one sign of radiculopathy or myelopathy, with only 10 % of patients being asymptomatic at the time of diagnosis [2]. During our examination of this child, we could not identify any objective signs or symptoms directly related to the spine. Careful neurological examination elicited no sensory deficits, weakness, or reflex abnormalities. On questioning, the patient denied ever having any back pain or incontinence. The only detectable deficit was right ankle and toe dorsiflexor weakness that was related to a previous Achilles tendon injury. It is not surprising then, in the absence of any neurological symptoms, that the cause of abdominal pain went undiagnosed for so long. Surgery is the mainstay of treatment of symptomatic spinal arachnoid cysts, but conservative management is preferred in asymptomatic cases [9, 10]. As such, atypical presentations of EDAC can pose a significant dilemma. Because abdominal pain was the sole presenting feature of our patient’s EDAC, it was not clear whether surgery would resolve her symptoms
Childs Nerv Syst
and this was discussed preoperatively. However, following resection, our patient experienced immediate resolution of her symptoms and has had no further recurrences following resection of her cyst. In light of these results, we concluded that the cyst was the cause of her symptoms. Similar cases have been reported in the literature where abdominal pain was the sole presenting feature of thoracic cord tumors in children [3, 7, 8, 14]. In these cases, recurrent abdominal pain was initially misdiagnosed as abdominal epilepsy [7], functional abdominal pain [3], psychogenic abdominal pain [8], and irritable bowel syndrome [14]. All patients experienced worsening of symptoms over time, and two developed a new scoliosis or neurological deficit before the correct diagnosis was made. In all cases, there was immediate and complete resolution of the abdominal pain following surgical decompression or resection of the lesion. The abdominal pain in our case was likely due to compression of the nerve roots or spinothalamic tracts innervating the abdominal wall and viscera [17]. Radicular or nerve root pain is caused by tethering or compression of the dorsal root ganglion and is a common manifestation in patients with spinal EDAC. Given the dermatomal distribution of the affected spinal segments (T11–L2), it is possible that her pain could be radiculopathic in origin. Irritation of these nerve roots would produce abdominal pain below the umbilicus, which is in keeping with the patient’s history. Funicular or central pain is another possibility. Funicular pain is a poorly localized pain thought to arise from irritation of spinothalamic tracts or posterior columns. Funicular pain from a lesion anywhere in the spinal cord up to the foramen magnum can result in abdominal pain. It typically presents as a burning quality with a superficial sharp, stabbing sensation in patients with intramedullary tumors. This type of pain is less likely, however, since it is usually associated with intramedullary lesions and is only rarely seen in extramedullary lesions [4]. Several factors likely contributed to the delay in this patient’s diagnosis. First, there were no red flags in the patient’s history or physical examination to suggest an organic cause of abdominal pain. Organic causes of RAP tend to be well localized away from the umbilicus (Apley’s Law) and present with vomiting or associated changes in bowel function [11]. This patient’s pain was poorly localized and had no temporal association. All physical examination findings and investigations performed prior to abdominal CT imaging were normal. Secondly, in the absence of neurological symptoms, a spinal etiology is less likely to be pursued. Finally, there are only a small number of case reports in the literature that have found an association between recurrent abdominal pain and spinal cord lesions in children. Because of this, much of the pediatric literature does not include spinal disease in the differential
diagnosis of recurrent abdominal pain [11, 13, 16]. Therefore, pediatricians and other primary care providers, whose patient population includes children with RAP, may not be aware of this association.
Conclusion In summary, this case demonstrates that abdominal pain may be the sole presenting feature of a spinal EDAC in children. In this case, excision of the cyst resulted in complete and long-term resolution of the patient’s abdominal pain, indicating that surgical intervention was appropriate. From the pediatrician’s perspective, the possibility of intraspinal disorders should be considered in children with unexplained RAP, even in the absence of neurological signs or symptoms. Acknowledgments The authors wish to thank Mr. Ross Hengel for his assistance with manuscript editing, Dr. Paul Thiessen for his guidance with submission of the manuscript, and Dr. Glenda Hendson from the Department of Anatomical Pathology at BC Children’s Hospital for preparing the photomicrographs for this manuscript. Conflict of interest The authors have no conflict of interest to disclose.
References 1.
2.
3.
4. 5.
6. 7.
8.
9.
10.
Aithala GR, Sztriha L, Amirlak I, Devadas K, Ohlsson I (1999) Spinal arachnoid cyst with weakness in the limbs and abdominal pain. Pediatr Neurol 20:155–156 Bond AE, Zada G, Bowen I, McComb JG, Krieger MD (2012) Spinal arachnoid cysts in the pediatric population: report of 31 cases and a review of the literature. J Neurosurg Pediatr 9: 432–441 Buck E, Bodensteiner J (1981) Thoracic cord tumor appearing as recurrent abdominal pain. Am J Dis Child 135:574–575 Byrne TN, Benzel EC, Waxman SG (2000) Diseases of the spine and spinal cord. Oxford University Press, New York Chang IC, Chou MC, Bell WR, Lin ZI (2004) Spinal cord compression caused by extradural arachnoid cysts. Clinical examples and review. Pediatr Neurosurg 40:70–74 Cloward RB (1968) Congenital spinal extradural cysts: case report with review of literature. Ann Surg 168:851–864 Drexler DL, Grill BB, Ashwal S (1989) Spinal cord tumorassociated syrinx mimicking abdominal epilepsy: a rare cause of childhood abdominal pain. J Pediatr Gastroenterol Nutr 9: 524–527 Eeg-Olofsson O, Carlsson E, Jeppsson S (1981) Recurrent abdominal pains as the first symptom of a spinal cord tumor. Acta Paediatr Scand 70:595–597 Liu JK, Cole CD, Kan P, Schmidt MH (2007) Spinal extradural arachnoid cysts: clinical, radiological, and surgical features. Neurosurg Focus 22:E6 Myles ST, Hamilton MG (2008) Intraspinal congenital cysts: neurenteric, arachnoid, dermoid, epidermoid. In: Albright AL, Pollack IF, Adelson PD (eds) Principles and practice of
Childs Nerv Syst
11. 12.
13. 14.
pediatric neurosurgery, 2nd edn. Thieme Medical Publishers, New York Oberlander TF, Rappaport LA (1993) Recurrent abdominal pain during childhood. Pediatr Rev 14:313–319 Oh JK, Lee DY, Kim TY, Yi S, Ha Y, Kim KN, Shin H, Kim DS, Yoon DH D2012] Thoracolumbar extradural arachnoid cysts: a study of 14 consecutive cases. Acta Neurochir DWien] 154:341– 348, discussion 348 Olson A (1987) Recurrent abdominal pain: an approach to diagnosis and management. Pediatr Ann 16:834–836, 838–840, 842 Robertson PL (1992) Atypical presentations of spinal cord tumors in children. J Child Neurol 7:360–363
15.
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