Images in Pediatric Neurosurgery Pediatr Neurosurg 2013;49:193–194 DOI: 10.1159/000358093
Received: August 28, 2013 Accepted after revision: December 18, 2013 Published online: March 13, 2014
Occult Anterior Sacral Meningocele Abscess Silvia Gesheva b Clarence Greene a, b a
Department of Pediatric Neurosurgery, Children’s Hospital New Orleans, and b Department of Neurosurgery, Louisiana State University, New Orleans, La., USA
We report the case of a 3-year-old boy who presented to the hospital with induration and erythema of the right gluteal region (fig. 1), enlarging over 2 days prior to admission. He was known to have a rare genetic disorder – monosomy 7q and trisomy 10p, and multiple congenital anomalies (including cleft lip, microcephaly, hypospadias, mid-face hypoplasia). Past medical history included a prolonged stay at the neonatal intensive care unit, Nissen fundoplication and percutaneous endoscopic gastrostomy tube placement, diabetes insipidus, and atrial septal defect. On admission, the patient was hyponatremic, afebrile, with no leukocytosis. C-reactive protein was elevated at 2.0. Initial blood and urine cultures were negative. An echocardiogram showed small pericardial effusion, mitral valve mass and severe mitral valve insufficiency. However, compared to previous echocardiograms, the mass was stable, therefore endocarditis was ruled out. Contrasted CT and MRI demonstrated an anterior sacral meningocele (ASM), which enhanced with contrast that indicated abscess formation (fig. 2, 3). The patient was taken to the operating room for incision and drainage of the gluteal mass on hospital day 2, with ultimate plan to repair the ASM once the infection resolved. The purulent drainage was evacuated, the wound was copiously irrigated and a Jackson-Pratt drain left in place. Postoperative MRI showed significant decompression of the infected ASM with decreased peripheral enhancement of the lesion. No evidence of fistula was appreciated on the repeat MRI scan. © 2014 S. Karger AG, Basel 1016–2291/14/0493–0193$39.50/0 E-Mail [email protected] www.karger.com/pne
Intraoperative cultures grew Escherichia coli, Bacteroides thetaiotaomicron, Enterococcus faecalis, and Pseudomonas aeruginosa – consistent with gut flora. Intravenous Zosyn was administered, and he had an improving clinical course until sudden cardiorespiratory arrest ensued on postoperative day 9, associated with profound hypocalcemia and anemia. Resuscitation attempts were unsuccessful. It was hypothesized that a fistula existed between the gastrointestinal tract and the anterior meningocele. The sacral myelomeningocele was confirmed on autopsy, however no communication between the ASM and the gastrointestinal or genitourinary tract was identified. We were personally present during the autopsy and extensively searched for the culprit, however no evidence of fistula between the ASM and skin was discovered. The endocardium showed extensive fibrosis and dystrophic calcifications, however no vegetation was identified. There was no obvious evidence on autopsy as to the cause of the demise. Occult spinal dysraphism is a group of disorders caused by failure of the neuroectoderm to separate from the epithelial ectoderm during embryogenesis. Occult spinal dysraphism includes myelomeningoceles, diastematomyelia, split-cord malformations, dermal sinus tracts, filum terminale abnormalities, as well as cutaneous and subcutaneous anomalies like angioma, lipoma, dimples [1]. A study by Carter et al. [2] found that there Silvia Gesheva 2020 Gravier Street, Suite 749 New Orleans, LA 70112 (USA) E-Mail sgeshe @ lsuhsc.edu
a
Fig. 1. Preoperative image of the indurated right gluteal region.
Fig. 2. Coronal (a) and sagittal (b) CT with contrast showing the enhancing sacral meningocele abscess.
a
was a 4% incidence of myelomengocele in a study of 364 patients with all types of occult spinal dysraphism. ASM is a very rare form of spinal dysraphism, in which the meninges protrude through a defect in the anterior wall of the sacrum. There have been approximately 250 cases reported. This disorder may be asymptomatic but may present with symptoms of constipation, urinary problems or neurologic deficits [3]. Infected ASM is extremely rare. Several cases of ASM associated with bacterial meningitis have been reported [4]. One case reports a perirectal abscess communicating with the ASM causing bacterial meningitis [5]. Another report discusses an ASM with concomitant spinal epidural abscess [6]. To our knowledge, a frank purulent infection of the ASM itself, as in this case, has not been recorded in the literature.
b
b
Fig. 3. MRI with (b) and without contrast (a) showing sacral agen-
esis and intense enhancement of the peripheral margins of the anterior meningocele (bent arrow). Also notable are tethering of the spinal cord (broad arrow) with focal syrinx formation (thin arrow).
References 1 Harris HW, Miller OF: Midline cutaneous and spinal defects. Midline cutaneous abnormalities associated with occult spinal disorders. Arch Dermatol 1976;112:1724–1728. 2 Carter CO, Evans KA, Till K: Spinal dysraphism: genetic relation to neural tube malformations. J Med Genet 1976;13:343–350.
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3 Calihan RJ: Anterior sacral meningocele. Radiology 1952;58:104–108. 4 Miletic D, Poljak I, Eskinja N, Valkovic P, Sestan B, Troselj-Vukic B: Giant anterior sacral meningocele presenting as bacterial meningitis in a previously healthy adult. Orthopedics 2008;31:182.
Pediatr Neurosurg 2013;49:193–194 DOI: 10.1159/000358093
5 Antuña-Ramos A, García-Fructuoso G, Alamar-Abril M, Guillén-Quesada A, CostaClara JM: Occult anterior sacral meningocele (in Spanish). Neurocirugia (Astur) 2011; 22: 345–346. 6 Patnaik S: Anterior sacral meningocele with spinal epidural abscess: a case report with review of literature. J Pediatr Neurol 2013; 11: 141–145.
Gesheva /Greene
Copyright: S. Karger AG, Basel 2014. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.
Received: August 28, 2013 Accepted after revision: December 18, 2013 Published online: March 13, 2014
Occult Anterior Sacral Meningocele Abscess Silvia Gesheva b Clarence Greene a, b a
Department of Pediatric Neurosurgery, Children’s Hospital New Orleans, and b Department of Neurosurgery, Louisiana State University, New Orleans, La., USA
We report the case of a 3-year-old boy who presented to the hospital with induration and erythema of the right gluteal region (fig. 1), enlarging over 2 days prior to admission. He was known to have a rare genetic disorder – monosomy 7q and trisomy 10p, and multiple congenital anomalies (including cleft lip, microcephaly, hypospadias, mid-face hypoplasia). Past medical history included a prolonged stay at the neonatal intensive care unit, Nissen fundoplication and percutaneous endoscopic gastrostomy tube placement, diabetes insipidus, and atrial septal defect. On admission, the patient was hyponatremic, afebrile, with no leukocytosis. C-reactive protein was elevated at 2.0. Initial blood and urine cultures were negative. An echocardiogram showed small pericardial effusion, mitral valve mass and severe mitral valve insufficiency. However, compared to previous echocardiograms, the mass was stable, therefore endocarditis was ruled out. Contrasted CT and MRI demonstrated an anterior sacral meningocele (ASM), which enhanced with contrast that indicated abscess formation (fig. 2, 3). The patient was taken to the operating room for incision and drainage of the gluteal mass on hospital day 2, with ultimate plan to repair the ASM once the infection resolved. The purulent drainage was evacuated, the wound was copiously irrigated and a Jackson-Pratt drain left in place. Postoperative MRI showed significant decompression of the infected ASM with decreased peripheral enhancement of the lesion. No evidence of fistula was appreciated on the repeat MRI scan. © 2014 S. Karger AG, Basel 1016–2291/14/0493–0193$39.50/0 E-Mail [email protected] www.karger.com/pne
Intraoperative cultures grew Escherichia coli, Bacteroides thetaiotaomicron, Enterococcus faecalis, and Pseudomonas aeruginosa – consistent with gut flora. Intravenous Zosyn was administered, and he had an improving clinical course until sudden cardiorespiratory arrest ensued on postoperative day 9, associated with profound hypocalcemia and anemia. Resuscitation attempts were unsuccessful. It was hypothesized that a fistula existed between the gastrointestinal tract and the anterior meningocele. The sacral myelomeningocele was confirmed on autopsy, however no communication between the ASM and the gastrointestinal or genitourinary tract was identified. We were personally present during the autopsy and extensively searched for the culprit, however no evidence of fistula between the ASM and skin was discovered. The endocardium showed extensive fibrosis and dystrophic calcifications, however no vegetation was identified. There was no obvious evidence on autopsy as to the cause of the demise. Occult spinal dysraphism is a group of disorders caused by failure of the neuroectoderm to separate from the epithelial ectoderm during embryogenesis. Occult spinal dysraphism includes myelomeningoceles, diastematomyelia, split-cord malformations, dermal sinus tracts, filum terminale abnormalities, as well as cutaneous and subcutaneous anomalies like angioma, lipoma, dimples [1]. A study by Carter et al. [2] found that there Silvia Gesheva 2020 Gravier Street, Suite 749 New Orleans, LA 70112 (USA) E-Mail sgeshe @ lsuhsc.edu
a
Fig. 1. Preoperative image of the indurated right gluteal region.
Fig. 2. Coronal (a) and sagittal (b) CT with contrast showing the enhancing sacral meningocele abscess.
a
was a 4% incidence of myelomengocele in a study of 364 patients with all types of occult spinal dysraphism. ASM is a very rare form of spinal dysraphism, in which the meninges protrude through a defect in the anterior wall of the sacrum. There have been approximately 250 cases reported. This disorder may be asymptomatic but may present with symptoms of constipation, urinary problems or neurologic deficits [3]. Infected ASM is extremely rare. Several cases of ASM associated with bacterial meningitis have been reported [4]. One case reports a perirectal abscess communicating with the ASM causing bacterial meningitis [5]. Another report discusses an ASM with concomitant spinal epidural abscess [6]. To our knowledge, a frank purulent infection of the ASM itself, as in this case, has not been recorded in the literature.
b
b
Fig. 3. MRI with (b) and without contrast (a) showing sacral agen-
esis and intense enhancement of the peripheral margins of the anterior meningocele (bent arrow). Also notable are tethering of the spinal cord (broad arrow) with focal syrinx formation (thin arrow).
References 1 Harris HW, Miller OF: Midline cutaneous and spinal defects. Midline cutaneous abnormalities associated with occult spinal disorders. Arch Dermatol 1976;112:1724–1728. 2 Carter CO, Evans KA, Till K: Spinal dysraphism: genetic relation to neural tube malformations. J Med Genet 1976;13:343–350.
194
3 Calihan RJ: Anterior sacral meningocele. Radiology 1952;58:104–108. 4 Miletic D, Poljak I, Eskinja N, Valkovic P, Sestan B, Troselj-Vukic B: Giant anterior sacral meningocele presenting as bacterial meningitis in a previously healthy adult. Orthopedics 2008;31:182.
Pediatr Neurosurg 2013;49:193–194 DOI: 10.1159/000358093
5 Antuña-Ramos A, García-Fructuoso G, Alamar-Abril M, Guillén-Quesada A, CostaClara JM: Occult anterior sacral meningocele (in Spanish). Neurocirugia (Astur) 2011; 22: 345–346. 6 Patnaik S: Anterior sacral meningocele with spinal epidural abscess: a case report with review of literature. J Pediatr Neurol 2013; 11: 141–145.
Gesheva /Greene
Copyright: S. Karger AG, Basel 2014. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.
