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doi:10.1111/jpc.12707
INSTRUCTIVE CASE
Intramedullary spinal cord abscess: The result of a missed congenital dermal sinus Yoganathan Kanaheswari,1 CheeHoe Lai,1 Raja Juanita Raja Lope,1 Abu Bakar Azizi2 and Muhamed Annuar Zulfiqar3 Departments of 1Paediatrics, 2Neurosurgery, and 3Radiology, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia
Abstract: Congenital dermal sinus (CDS) and occult spinal dysraphism are suspected when a cutaneous marker overlies the spine of a newborn. CDS can have the appearance of a simple dimple and occur within the gluteal cleft without any skin markers. CDS are the commonest cause of intramedullary spinal cord abscess. Key words:
dermal sinus; occult spinal dysraphism; spinal cord abscess.
Hairy patches, pigmented naevi (other than Mongolian blue spots), haemangiomas, lipomas and sinuses found overlying the spine of a well neonate or infant may be a marker of underlying occult spinal dysraphism (OSD).These include lipomas, tethered cord, split cord malformations and spina bifida occulta. These asymptomatic children may develop lower limb neurological sequealae, urinary and bowel dysfunction as they grow as a result of distortion of the spinal cord and nerve roots.1 The presence of these midline skin markers should prompt early imaging of the underlying spine and surgical intervention to avoid irreversible neurological damage.2 What then of dimples, pits and sinuses without any of the skin markers described above? This report illustrates a child with a ‘simple’ pit, who developed disastrous consequences. We also review the literature and discuss the management of midline pits and sinuses. The aim of this article is to clarify and differentiate the lesions that require further investigation from those that can be safely ignored. Key Points 1 Sacral dimples and pits lying more than 25 mm above the anus (in the superior gluteal cleft region) should be imaged to rule out congenital dermal sinus and occult spinal dysraphism (OSD) even if there are no other associated cutaneous markers. 2 Cutaneous lesions over the spine, above the gluteal cleft, can be associated with occult spinal dysraphism. 3 The leading cause of intramedullary spinal cord abscesses is congenital dermal sinus with OSD. Correspondence: Associate Professor Yoganathan Kanaheswari, Paediatrics, Universiti Kebangsaan Malaysia, Jalan Yaakob Latiff, Cheras, Kuala Lumpur 56000, Malaysia. Fax: 603-91456637; email: kanahes@ ppukm.ukm.edu.my Conflict of interest: None declared. Accepted for publication 11 July 2014.
Case Report A previously well 2-year- and 3-month-old boy presented to our hospital with an 8-day history of fever and cough and a 1-day history of lethargy with poor oral intake. He was fully immunised, and developmental milestones were normal. On examination, he was alert, febrile, mildly dehydrated and had erythematous tonsils with exudates. There was no meningism, and all other system examinations were normal. A diagnosis of acute exudative tonsillo-pharyngitis was made, and he was treated with intravenous (IV) antibiotics and fluid rehydration. The following day he developed meningism and a positive Kernig sign. Fundoscopy was normal, and Glasgow Coma Scale was 15/15. Upper and lower limb muscle power was estimated at grade 3/5. There was hypotonia and hyporeflexia of both lower limbs. An urgent computed tomography brain scan showed mild ventriculomegaly without meningeal enhancement or cerebral oedema. Lumbar puncture revealed cloudy cerebrospinal fluid (CSF) with protein level of 2905 mg/L; glucose of 1.2 mmol/L (random blood glucose: 6 mmol/L); polymorpholeucocytes of 55/mm3, lymphocytes of 320/mm3 and red blood cells of 600/mm3. Latex agglutination test was negative. He was treated for meningitis with IV crystalline penicillin, cefotaxime and dexamethsone. CSF culture grew Enterobacter sakazakii sensitive to the cephalosporins. However, the blood culture was negative. On the third day, he developed acute urinary retention. Lower limbs muscle power deteriorated to grade 0/5 with areflexia and loss of sensation. A magnetic resonance imaging (MRI) of brain and spine revealed an intradural abscess with septations extending from T9 to S2 levels (Fig. 1) and absent posterior elements of the sacral vertebrae. A sinus tract was seen extending from the skin of the right gluteal region and communicating with the epidural space at S3 level. A diagnosis of intramedullary spinal cord abscess (ISCA) with congenital dermal sinus (CDS) and underlying spina bifida occulta was made.
Journal of Paediatrics and Child Health 51 (2015) 223–225 © 2014 The Authors Journal of Paediatrics and Child Health © 2014 Paediatrics and Child Health Division (Royal Australasian College of Physicians).
223
Spinal cord abscess and dermal sinus
a
b
Y Kanaheswari et al.
c
Fig. 1 Sagittal T1W post-gadolinium images. (a) The abscess extends from T9 to S2 levels (white arrows). (b and c) The sinus tract extends caudally from the skin towards the spinal canal (black arrows).
On further detailed clinical examination, a tiny pin-point (less than 5 mm width) pit was seen at the sacral region a few millimetres to the right of the midline within the gluteal cleft and more than 25 mm cephalad to the anus. The overlying skin was normal. During surgery, a sinus tract, with tufts of hair within, was seen extending from the skin. This was resected along with copius amounts of pus. Histology was consistent with a dermal sinus and an inflamed epidermoid cyst wall consisting of keratinised squamous epithelium. His fever persisted and repeat MRI (day 11) showed further cephalad extension of the abscess to the T2 level. Repeat blood cultures were negative. Antibiotics were changed to a combination of meropenem, metronidazole and teicoplanin with clinical improvement. He required clean intermittent catheterisation for neurogenic bladder. He completed 8 weeks of antibiotics and was afebrile and well after discharge. On review 6 months later, he remained wheelchair bound with sensory loss below T2 level. He required clean intermittent catheterisations five times daily as well as manual bowel evacuations.
Discussion CDS is the leading cause of ISCA in children.3 This extremely rare infection may present at any age but is most common in children aged under 5 years. Diagnosis is difficult and often delayed until significant neurological deficit occurs. Progressive sensory and motor loss is the most common presentation. Signs of sepsis and pain are often absent.3 Prognosis is often unfavourable with consequences such as paraparesis and neurogenic bladder and bowel. CDSs are cutaneous depressions or tracts that result from abnormal separation of the cutaneous and neural ectoderm 224
during early intra-uterine life. They are lined by stratified squamous epithelium and surrounding dermal tissues.4–6 They can occur anywhere along the cranio-spinal axis but are most common (35%) in the lumbosacral region above the gluteal cleft.7 The sinus is cephalically oriented and often associated with OSD. Hairy tufts, lipomas, vascular or pigmented patches are commonly seen with CDS. Presence of these markers prompts further evaluation in a newborn. On occasion, sacral CDS occur within the gluteal cleft and appear as tiny pits or dimples without obvious skin markers. These are often dismissed as innocuous lesions that can lead to adverse outcomes. Imaging with spinal ultrasound is possible in the first 3–6 months of life when the posterior spinal elements are still cartilaginous.1 MRI of the spine is the gold standard and is required beyond this age. Early excision of the sinus tract is recommended. Other lesions that appear within the gluteal cleft are coccygeal pits and sacral dimples. These are more common, caudally oriented and not associated with any underlying pathology. Those that are less than 5 mm wide and less than 25 mm from the anus do not require any further evaluation.1
Conclusion CDS and OSD are suspected when a cutaneous marker overlies the spine of a newborn, particularly when positioned above the gluteal cleft. Although rare, CDS can have the appearance of a simple dimple and occur within the gluteal cleft without any skin markers. CDS are the commonest cause of ISCA. All medical trainees should be taught to identify the lesions that require further evaluation. We recommend a low threshold for imaging any lesion greater than 25 mm from the anus. Early surgical intervention is recommended when OSD is found.
Journal of Paediatrics and Child Health 51 (2015) 223–225 © 2014 The Authors Journal of Paediatrics and Child Health © 2014 Paediatrics and Child Health Division (Royal Australasian College of Physicians)
Y Kanaheswari et al.
MCQ Q1. The following statements are true of intramedullary spinal cord abscesses (ISCA) except A. The commonest age of presentation is in children under 5 years of age. B. The most common lesion leading to ISCA is a congenital dermal sinus. C. Pain is an uncommon feature of this infection. D. Lower limb motor and sensory deficits are usually present. E. Prognosis is excellent. Answer: E. Explanation: CDSs have a cephalically oriented tract that ends in the intradural space hence allowing organisms entry into the spinal canal. Abscess formation in the intradural space involves nerve roots leading to neurological deficits. The majority of cases present late because (i) pain is not a symptom; and (ii) the motor and/or sensory deficit is often missed by parents especially in young children. Q2. Which of the following is true of occult spinal dysraphism (OSD)? A. Mongolian blue spots are common markers of OSD. B. Hydrocephalus is a common association. C. Formation of intradural abscesses are common. D. It is commonest in the thoraco-lumbar spine. E. It should be included in the differential diagnosis of a child with bowel and bladder dysfunction. Answer: E. Explanation: Mongolian blue spots are not associated with any serious pathology. Hydrocephalus is common in spina bifida aperta but not OSD. Intradural abscesses occur when there is a direct connection from skin to the intradural space, and this is uncommon except in presence of CDS. The commonest site for OSD is the lumbo-sacral spine. OSD can present later in childhood. This is because the spinal cord is adhered to the conus medullaris and as the child grows, the cord is stretched and distorted. This leads to damage to the sacral nerve roots that supply the bladder and bowel. Hence a
Spinal cord abscess and dermal sinus
previously asymptomatic child can develop bowel and bladder dysfunction symptoms. Q3. Which of the following features of a lesion overlying the spine of a newborn infant suggests a congenital dermal sinus? A. A midline pit seen in the lumbosacral region with a tuft of hair. B. A midline depression with a soft cystic mass inferiorly. C. A cephalic-oriented sinus tract from the skin to the spinal canal on ultrasound evaluation. D. An isolated pit within the gluteal cleft that is more than 25 mm above the superior anal margin. E. All of the above. Answer: E. Explanation: Cutaneous markers such as tufts of hair (A), lipomas (B), hyperpigmentation, vascular naevi are associated with CDS. The tract in a CDS is usually oriented cephalically rather than caudally as in coccygeal pits. Isolated pits if lying high in the gluteal cleft and overlying the sacral vertebrae may have a sinus tract leading to the intradural space, that is a CDS.
References 1 Williams H. Spinal sinuses, dimples, pits and patches: what lies beneath? Arch. Dis. Child. Educ. Pract. Ed. 2006; 91: ep75–80. 2 Ackerman LL, Menzes AH. Spinal congenital dermal sinuses: a 30 year experience. Pediatrics 2003; 112: 641–7. 3 Volkan ETUS. Intramedullary spinal cord abscesses in children: a review. J. Neurol. Sci. Turk. 2011; 28: 651–61. http://jns.dergisi.org/text.php3?id=477. 4 Hung P-C, Wang H-S, Wu C-T, Lui TN, Wong AM-C. Spinal intramedullary abscess with an epidermoid secondary to a dermal sinus. Pediatr. Neurol. 2007; 37: 144–7 [accessed 11 September 2013]. 5 Barbarawi MA, Khriesat W, Qudsieh S, Sudsieh H, Loai AA. Management of intramedullary spinal cord abscess: experience with four cases, pathophysiology and outcomes. Eur. Spine J. 2009; 18: 710–17. 6 Weprin BE, Oakes WJ. Coccygeal pits. Pediatrics 2000; 105: E69. 7 French BN. Midline fusion defects and defects of formation. In: You-mans JR, ed. Neurological Survey. Philadelphia, PA: WB Saunders Company, 1990; 1081–235.
Journal of Paediatrics and Child Health 51 (2015) 223–225 © 2014 The Authors Journal of Paediatrics and Child Health © 2014 Paediatrics and Child Health Division (Royal Australasian College of Physicians)
225
doi:10.1111/jpc.12707
INSTRUCTIVE CASE
Intramedullary spinal cord abscess: The result of a missed congenital dermal sinus Yoganathan Kanaheswari,1 CheeHoe Lai,1 Raja Juanita Raja Lope,1 Abu Bakar Azizi2 and Muhamed Annuar Zulfiqar3 Departments of 1Paediatrics, 2Neurosurgery, and 3Radiology, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia
Abstract: Congenital dermal sinus (CDS) and occult spinal dysraphism are suspected when a cutaneous marker overlies the spine of a newborn. CDS can have the appearance of a simple dimple and occur within the gluteal cleft without any skin markers. CDS are the commonest cause of intramedullary spinal cord abscess. Key words:
dermal sinus; occult spinal dysraphism; spinal cord abscess.
Hairy patches, pigmented naevi (other than Mongolian blue spots), haemangiomas, lipomas and sinuses found overlying the spine of a well neonate or infant may be a marker of underlying occult spinal dysraphism (OSD).These include lipomas, tethered cord, split cord malformations and spina bifida occulta. These asymptomatic children may develop lower limb neurological sequealae, urinary and bowel dysfunction as they grow as a result of distortion of the spinal cord and nerve roots.1 The presence of these midline skin markers should prompt early imaging of the underlying spine and surgical intervention to avoid irreversible neurological damage.2 What then of dimples, pits and sinuses without any of the skin markers described above? This report illustrates a child with a ‘simple’ pit, who developed disastrous consequences. We also review the literature and discuss the management of midline pits and sinuses. The aim of this article is to clarify and differentiate the lesions that require further investigation from those that can be safely ignored. Key Points 1 Sacral dimples and pits lying more than 25 mm above the anus (in the superior gluteal cleft region) should be imaged to rule out congenital dermal sinus and occult spinal dysraphism (OSD) even if there are no other associated cutaneous markers. 2 Cutaneous lesions over the spine, above the gluteal cleft, can be associated with occult spinal dysraphism. 3 The leading cause of intramedullary spinal cord abscesses is congenital dermal sinus with OSD. Correspondence: Associate Professor Yoganathan Kanaheswari, Paediatrics, Universiti Kebangsaan Malaysia, Jalan Yaakob Latiff, Cheras, Kuala Lumpur 56000, Malaysia. Fax: 603-91456637; email: kanahes@ ppukm.ukm.edu.my Conflict of interest: None declared. Accepted for publication 11 July 2014.
Case Report A previously well 2-year- and 3-month-old boy presented to our hospital with an 8-day history of fever and cough and a 1-day history of lethargy with poor oral intake. He was fully immunised, and developmental milestones were normal. On examination, he was alert, febrile, mildly dehydrated and had erythematous tonsils with exudates. There was no meningism, and all other system examinations were normal. A diagnosis of acute exudative tonsillo-pharyngitis was made, and he was treated with intravenous (IV) antibiotics and fluid rehydration. The following day he developed meningism and a positive Kernig sign. Fundoscopy was normal, and Glasgow Coma Scale was 15/15. Upper and lower limb muscle power was estimated at grade 3/5. There was hypotonia and hyporeflexia of both lower limbs. An urgent computed tomography brain scan showed mild ventriculomegaly without meningeal enhancement or cerebral oedema. Lumbar puncture revealed cloudy cerebrospinal fluid (CSF) with protein level of 2905 mg/L; glucose of 1.2 mmol/L (random blood glucose: 6 mmol/L); polymorpholeucocytes of 55/mm3, lymphocytes of 320/mm3 and red blood cells of 600/mm3. Latex agglutination test was negative. He was treated for meningitis with IV crystalline penicillin, cefotaxime and dexamethsone. CSF culture grew Enterobacter sakazakii sensitive to the cephalosporins. However, the blood culture was negative. On the third day, he developed acute urinary retention. Lower limbs muscle power deteriorated to grade 0/5 with areflexia and loss of sensation. A magnetic resonance imaging (MRI) of brain and spine revealed an intradural abscess with septations extending from T9 to S2 levels (Fig. 1) and absent posterior elements of the sacral vertebrae. A sinus tract was seen extending from the skin of the right gluteal region and communicating with the epidural space at S3 level. A diagnosis of intramedullary spinal cord abscess (ISCA) with congenital dermal sinus (CDS) and underlying spina bifida occulta was made.
Journal of Paediatrics and Child Health 51 (2015) 223–225 © 2014 The Authors Journal of Paediatrics and Child Health © 2014 Paediatrics and Child Health Division (Royal Australasian College of Physicians).
223
Spinal cord abscess and dermal sinus
a
b
Y Kanaheswari et al.
c
Fig. 1 Sagittal T1W post-gadolinium images. (a) The abscess extends from T9 to S2 levels (white arrows). (b and c) The sinus tract extends caudally from the skin towards the spinal canal (black arrows).
On further detailed clinical examination, a tiny pin-point (less than 5 mm width) pit was seen at the sacral region a few millimetres to the right of the midline within the gluteal cleft and more than 25 mm cephalad to the anus. The overlying skin was normal. During surgery, a sinus tract, with tufts of hair within, was seen extending from the skin. This was resected along with copius amounts of pus. Histology was consistent with a dermal sinus and an inflamed epidermoid cyst wall consisting of keratinised squamous epithelium. His fever persisted and repeat MRI (day 11) showed further cephalad extension of the abscess to the T2 level. Repeat blood cultures were negative. Antibiotics were changed to a combination of meropenem, metronidazole and teicoplanin with clinical improvement. He required clean intermittent catheterisation for neurogenic bladder. He completed 8 weeks of antibiotics and was afebrile and well after discharge. On review 6 months later, he remained wheelchair bound with sensory loss below T2 level. He required clean intermittent catheterisations five times daily as well as manual bowel evacuations.
Discussion CDS is the leading cause of ISCA in children.3 This extremely rare infection may present at any age but is most common in children aged under 5 years. Diagnosis is difficult and often delayed until significant neurological deficit occurs. Progressive sensory and motor loss is the most common presentation. Signs of sepsis and pain are often absent.3 Prognosis is often unfavourable with consequences such as paraparesis and neurogenic bladder and bowel. CDSs are cutaneous depressions or tracts that result from abnormal separation of the cutaneous and neural ectoderm 224
during early intra-uterine life. They are lined by stratified squamous epithelium and surrounding dermal tissues.4–6 They can occur anywhere along the cranio-spinal axis but are most common (35%) in the lumbosacral region above the gluteal cleft.7 The sinus is cephalically oriented and often associated with OSD. Hairy tufts, lipomas, vascular or pigmented patches are commonly seen with CDS. Presence of these markers prompts further evaluation in a newborn. On occasion, sacral CDS occur within the gluteal cleft and appear as tiny pits or dimples without obvious skin markers. These are often dismissed as innocuous lesions that can lead to adverse outcomes. Imaging with spinal ultrasound is possible in the first 3–6 months of life when the posterior spinal elements are still cartilaginous.1 MRI of the spine is the gold standard and is required beyond this age. Early excision of the sinus tract is recommended. Other lesions that appear within the gluteal cleft are coccygeal pits and sacral dimples. These are more common, caudally oriented and not associated with any underlying pathology. Those that are less than 5 mm wide and less than 25 mm from the anus do not require any further evaluation.1
Conclusion CDS and OSD are suspected when a cutaneous marker overlies the spine of a newborn, particularly when positioned above the gluteal cleft. Although rare, CDS can have the appearance of a simple dimple and occur within the gluteal cleft without any skin markers. CDS are the commonest cause of ISCA. All medical trainees should be taught to identify the lesions that require further evaluation. We recommend a low threshold for imaging any lesion greater than 25 mm from the anus. Early surgical intervention is recommended when OSD is found.
Journal of Paediatrics and Child Health 51 (2015) 223–225 © 2014 The Authors Journal of Paediatrics and Child Health © 2014 Paediatrics and Child Health Division (Royal Australasian College of Physicians)
Y Kanaheswari et al.
MCQ Q1. The following statements are true of intramedullary spinal cord abscesses (ISCA) except A. The commonest age of presentation is in children under 5 years of age. B. The most common lesion leading to ISCA is a congenital dermal sinus. C. Pain is an uncommon feature of this infection. D. Lower limb motor and sensory deficits are usually present. E. Prognosis is excellent. Answer: E. Explanation: CDSs have a cephalically oriented tract that ends in the intradural space hence allowing organisms entry into the spinal canal. Abscess formation in the intradural space involves nerve roots leading to neurological deficits. The majority of cases present late because (i) pain is not a symptom; and (ii) the motor and/or sensory deficit is often missed by parents especially in young children. Q2. Which of the following is true of occult spinal dysraphism (OSD)? A. Mongolian blue spots are common markers of OSD. B. Hydrocephalus is a common association. C. Formation of intradural abscesses are common. D. It is commonest in the thoraco-lumbar spine. E. It should be included in the differential diagnosis of a child with bowel and bladder dysfunction. Answer: E. Explanation: Mongolian blue spots are not associated with any serious pathology. Hydrocephalus is common in spina bifida aperta but not OSD. Intradural abscesses occur when there is a direct connection from skin to the intradural space, and this is uncommon except in presence of CDS. The commonest site for OSD is the lumbo-sacral spine. OSD can present later in childhood. This is because the spinal cord is adhered to the conus medullaris and as the child grows, the cord is stretched and distorted. This leads to damage to the sacral nerve roots that supply the bladder and bowel. Hence a
Spinal cord abscess and dermal sinus
previously asymptomatic child can develop bowel and bladder dysfunction symptoms. Q3. Which of the following features of a lesion overlying the spine of a newborn infant suggests a congenital dermal sinus? A. A midline pit seen in the lumbosacral region with a tuft of hair. B. A midline depression with a soft cystic mass inferiorly. C. A cephalic-oriented sinus tract from the skin to the spinal canal on ultrasound evaluation. D. An isolated pit within the gluteal cleft that is more than 25 mm above the superior anal margin. E. All of the above. Answer: E. Explanation: Cutaneous markers such as tufts of hair (A), lipomas (B), hyperpigmentation, vascular naevi are associated with CDS. The tract in a CDS is usually oriented cephalically rather than caudally as in coccygeal pits. Isolated pits if lying high in the gluteal cleft and overlying the sacral vertebrae may have a sinus tract leading to the intradural space, that is a CDS.
References 1 Williams H. Spinal sinuses, dimples, pits and patches: what lies beneath? Arch. Dis. Child. Educ. Pract. Ed. 2006; 91: ep75–80. 2 Ackerman LL, Menzes AH. Spinal congenital dermal sinuses: a 30 year experience. Pediatrics 2003; 112: 641–7. 3 Volkan ETUS. Intramedullary spinal cord abscesses in children: a review. J. Neurol. Sci. Turk. 2011; 28: 651–61. http://jns.dergisi.org/text.php3?id=477. 4 Hung P-C, Wang H-S, Wu C-T, Lui TN, Wong AM-C. Spinal intramedullary abscess with an epidermoid secondary to a dermal sinus. Pediatr. Neurol. 2007; 37: 144–7 [accessed 11 September 2013]. 5 Barbarawi MA, Khriesat W, Qudsieh S, Sudsieh H, Loai AA. Management of intramedullary spinal cord abscess: experience with four cases, pathophysiology and outcomes. Eur. Spine J. 2009; 18: 710–17. 6 Weprin BE, Oakes WJ. Coccygeal pits. Pediatrics 2000; 105: E69. 7 French BN. Midline fusion defects and defects of formation. In: You-mans JR, ed. Neurological Survey. Philadelphia, PA: WB Saunders Company, 1990; 1081–235.
Journal of Paediatrics and Child Health 51 (2015) 223–225 © 2014 The Authors Journal of Paediatrics and Child Health © 2014 Paediatrics and Child Health Division (Royal Australasian College of Physicians)
225
