Eur Arch Otorhinolaryngol DOI 10.1007/s00405-014-2960-3
Case Report
Recurrent cerebrospinal fluid leaks and bacterial meningitis in complex posttraumatic dural‑lymphatic skull‑base malformation after craniocerebral injury in childhood Carmela Koch · Anne Lorenz · Patrick Thamm · Werner G. Hosemann · Achim G. Beule
Received: 5 November 2013 / Accepted: 18 February 2014 © Springer-Verlag Berlin Heidelberg 2014
Introduction Recurrent bacterial meningitis is rare but severe disease and its origins should be well investigated. One reason could be anatomical problems mainly localized in the head and neck region [1] such as occult skull base malformations with or without cerebrospinal fluid (CSF) leaks which have no external manifestation. These malformations can be divided into congenital [1–3] and mostly acquired forms with inflammatory, e.g. osteomyelitis [4], iatrogenic [5] or traumatic genesis [1]. They can involve several vessels, skull base and brain structures in the lateral [6, 7], anterior [4], or less often of the medial skull base [4, 8]. Posttraumatic skull base malformation can become symptomatic immediately with persistent CSF fluid fistulas after a head injury which can lead to posture-related headaches and pneumocephalus or meningitis [1]. Furthermore, delayed onsets of symptoms in occult malformations like recurrent meningitis or CSF leaks after years or decades are possible [1, 4]. One variant of these occult skull base malformations can be dural-lymphatic fistulas where the cerebral fluid interspaces anastomose with regional lymphatic vessels and also nerve sheaths so that they are filled with liquor. Durallymphatic fistulas are a very rare vascular malformation of the skull base. That is why they are not well described yet.
C. Koch (*) · A. Lorenz · W. G. Hosemann · A. G. Beule Department of ENT, Head and Neck Surgery, University Medicine Greifswald, Greifswald, Germany e-mail: carmela.koch@uni‑greifswald.de P. Thamm Institute of Diagnostic Radiology and Neuroradiology, University Medicine Greifswald, Greifswald, Germany
For that reason we want to demonstrate one of these unusual cases and its complications.
Case report A 45-year-old woman was presented in our hospital after at least ten episodes of bacterial meningitis with and without simultaneous CSF leaks. Previously, she has had 12 operations involving the nose, paranasal sinuses and frontal skull base due to a known posttraumatic skull base malformation located in the sphenoid sinus and cavernous sinus after head trauma in early childhood (overview of the available information in Fig. 1). Five years before visiting our hospital, two large parasellar and temporobasal liquor accumulations as well as several smaller osseous CSF leaks in the posterior wall and ground of the left sphenoid and maxillary sinus had been described. The liquor seemed to be transported longitudinally along the nerve sheaths which could be observed via intrathecal fluorescein imaging. Following the last neurosurgical operation, there was no doubt that the left cavernous sinus was filled with arachnoidea and liquor and was surrounded by very filmy, perforated bone with diaphanous trigeminal branches. In further histopathological analysis of mucosa, untypically distended lymphatic vessels without direct detection of liquor were provided, so that a complex dural-lymphatic fistula of the skull base was probable. After the tenth meningitis, following a 5-year meningitis-free period, our patient had another recurrent meningitis and accordingly a need for restaging to repair damage and avoid recurrence of meningitis. We began by CT- and MRimaging of the head focusing on the skull base. After this proved ineffective, we conducted an interdisciplinary radiological conference to find additional strategies. We started
13
Eur Arch Otorhinolaryngol Head trauma in childhood (nine years) with the suspicion of skull base malformation of the left sphenoid sinus and cavernous sinus 1 year later 1. meningitis 6 months later CSF leak 3 months later
1. Sinus surgery via eye brow incision
2. meningitis, CSF leak
8 years later
3. meningitis, CSF leak
2. Sinus surgery via eye brow incision 3. Exploration of skull base and covering of the skull base defect with fascia lata
3 months later
CSF leak?
4. Exploration of skull base and covering of the defect with fascia lata
4 months later
CSF leak ?
5. Sinus surgery via transfacial approach
16 years later
4. meningitis, CSF leak
1 year later
5. meningitis, CSF leak, sympt. focal epilepsy
6. Trepanation and covering of the frontal CSF leak with an autologous transplant
1 year later 7. Transsphenoidal covering of an skull base defect on the floor of the sella turcica with transposition of muscle, fat and fascia lata
6. meningitis
7 months later
epidural hematoma
8. Left frontal recraniotomy and decompression of an epidural hematoma
1 year later 9. Surgery of the left rhinobasis, covering of the left CSF leak with fascia lata and exploration of the right frontal sinus 7. meningitis 9 months later 8. meningitis
10. Osteoplastic surgery of the right frontal sinus via coronal or bi-temporal approach and its obliteration with umbilical fat, endonasal endoscopic surgery of the left skull base and sphenoid sinus and duraplastic after intrathecal fluorescein 9 months later 11. Sinus surgery of the left maxillary sinus and obliteration with abdominal fat for occluding several CSF leaks after intrathecal fluorescein application
9. meningitis, CSF leak
4 months later 12. Occlusion of the cystic distended left cavernous sinus and covering of several CFS leaks temporal left with fascia temporalis after recraniotomy and decompression of a left pterional epidural hematoma
5 years later 10. meningitis 13. Reconstruction of skull base via transpterydoidal approuch after intrathecal gadolinium Imaging and after intrathecal fluorescein application and endoskopy of the nose, sinuses and epipharynx preoperative
Fig. 1 Time bar of the patient’s medical history especially concerning symptoms of occult skull base malformation as well as following complications and their surgery
13
Eur Arch Otorhinolaryngol
Fig. 2 MR-imaging of skull base a axial T2 b–d axial, coronal and sagittal T1 after the application of intrathecal contrast agent (gadolinium). asterisk left trigeminal nerve plus liquor-filled defect of the
left cavum meckeli with baggy swelling in the left cavernous sinus arrow contrast agent leakage
with MR-imaging after applying intrathecal gadolinium revealing a defect of the left cavum meckeli with baggy swelling in the left cavernous sinus which seemed to communicate directly with cerebral fluid interspaces. A contrast
agent passage from the cavum meckeli along the trigeminal branches with a maximum in the left posterior ethmoidal cells (Fig. 2) was detected. Two days later, we applied intrathecal fluorescein and performed endoscopy of the
13
Eur Arch Otorhinolaryngol
a
b
right tubal protuberance left tubal protuberance posterior epipharyngeal wall submucosal fluorescein pathways
Fig. 3 Endoscopic view (blue light) in right a and left b epipharynx. Visible are submucosal fluorescein pathways (yellow) and no proof for an active CSF leak
nose, paranasal sinuses and epipharynx in general anaesthesia. This revealed submucosal fluorescein pathways in the epipharynx and near the left sphenopalatine foramen as the probable cause of the recurrent meningitis (Fig. 3). A CSF leak could not be proved. In consequence of these findings, we performed another skull base reconstruction via a transpterygoidal approach and covered the defect as central as possible with fascia lata. Intraoperatively, there were two CSF leaks in the region of the anterior sphenoid wall through the foramen rotundum as well as following the maxillary artery dorsal of the posterior wall of the sphenoid sinus. Afterwards, CSF fluid was no longer visible along the intramucosal nerve sheaths. The postoperative course was free of complications. Currently, with another follow-up of 6 months the patient is free of any disorders and under frequent clinical surveillance. The next re-evaluation is planned in 6 months with an endoscopy in general anaesthesia after an intrathecal fluorescein application.
Discussion Posttraumatic malformations of skull base vessels are rare [1] but can have severe consequences as seen in our case. There are reported cases where adult patients were symptomatic with recurrent meningitis or CSF leaks associated with posttraumatic herniation of subarachnoid space into diploe, paranasal sinuses or orbit following injury having caused a dura tear in the past [9]. This case seems to be similar where arachnoidea and liquor were seen in the cavernous sinus and several CSF leaks in the region of the left sphenoid sinus were observed. Nevertheless, nontraumatic skull base defects leading to spontaneous CSF rhinorrhea
13
could have analogous radiological findings [10], so the patient history is the only chance of discriminating between a traumatic and a nontraumatic origin. Additionally, our patient had a malformation that communicated with regional lymphatic vessels, which transported the CSF in submucosal structures primarily of the left paranasal sinuses and epipharynx, as could be shown in several diagnostic results at different points in time. Bektas et al. described similar findings in a patient with same medical history as our patient. During their investigation, they could also prove intact septal nasal mucosa with submucosal nasoseptal collection of CSF without CSF leaks or rhinoliquorrhea [11] which caused recurrent meningitis. All types of occult malformations causing CSF leaks or recurrent meningitis should be rapidly treated surgically immediately after controlling infections [4] as in our case to avoid serious consequences. However, in spite of sufficient therapy recurrence of skull base malformation including lymphatic-dural fistulas is possible as observed in our case. This shows that there is a need for local and radiological diagnostic findings due to the possibility of long asymptomatic period lasting years or even decades. This is another reason why one should think of skull base malformations in recurrent meningitis or CSF leaks. Exact patient anamnesis and adequate diagnostics are essential.
Conclusion Dural-lymphatic fistulas are rare but severe complications after traumatic craniocerebral injuries can lead to CSF leaks and recurrent meningitis with potentially life-threatening consequences. Correct diagnose-finding can be difficult due
Eur Arch Otorhinolaryngol
to the need of delicate and invasive diagnostic tools which are often unavailable. In spite of high operational expertise and interdisciplinary collaboration successful therapy can be challenging due to the high relapsing rate. Hence, these patients should be sent immediately to specialised medical interdisciplinary skull base centres. They should also stick to permanent and lifelong surveillance for early recognition of recurrence in order and to avoid permanent individual health consequences like persistent impairment or death.
References 1. Tebruegge M, Curtis N (2008) Epidemiology, etiology, pathogenesis, and diagnosis of recurrent bacterial meningitis. Clin Microbiol Rev 21(3):519–537 2. Schick B, Prescher A, Hofmann E, Steigerwald C, Draf W (2003) Two occult skull base malformations causing recurrent meningitis in a child: a case report. Eur Arch Otorhinolaryngol 260:518–521 3. Prescher A (2009) Embryology of skull base malformations. Meeting contribution ESBS 2005: skull base surgery: an interdisciplinary challenge. 7th Congress of the European Skull Base Society held in association with 13th Congress of the German Society of Skull Base Surgery, Fulda, 18–21/05/2005. German Medical Science GMS Publishing House 2009
4. Wang H-S, Kuo M-F, Huang S-C (2005) Diagnostic approach to recurrent bacterial meningitis in children. Chang Gung Med J 28:441–452 5. Bumm K, Heupel J, Bozzato A, Iro H, Hornung J (2009) Localization and infliction pattern of iatrogenic skull base defects following endoscopic sinus surgery at a teaching hospital. Auris Nasus Larynx 36:671–676 6. Windfuhr JP, Sesterhenn K (2002) Spontane Defekte der seitlichen Schädelbasis, Teil 2, Ätiologie und Literaturübersicht. HNO 50:441–463 7. Bryson E, Draf W, Hofmann E, Bockmühl U (2005) Versorgung okkulter Missbildungen der lateralen Schädelbasis. LaryngoRhino-Otol 84:921–928 8. Mulcahy MM, McMenomey SO, Talbot JM, Delashaw JB Jr (1997) Congenital encephalocele of the medial skull base. Laryngoscope 107:910–914 9. Byrne JV, Britten JA, Kaar G (1992) Chronic posttraumatic erosion of skull base. Neuroradiology 34:528–531 10. Schuknecht B, Simen D, Briner HR, Holzmann D (2008) Nontraumatic skull base defects with spontaneous rhinorrhea and arachnoid herniation: imaging findings and correlation with endoscopic sinus surgery in 27 patients. Am J Neuroradiol 29:542–549 11. Bektas D, Caylan R, Bahadir O, Caylan R (2007) Occult anterior skull base defect without rhinorrhea as a cause of recurrent meningitis. Surg Neurol 68:50–52
13
Case Report
Recurrent cerebrospinal fluid leaks and bacterial meningitis in complex posttraumatic dural‑lymphatic skull‑base malformation after craniocerebral injury in childhood Carmela Koch · Anne Lorenz · Patrick Thamm · Werner G. Hosemann · Achim G. Beule
Received: 5 November 2013 / Accepted: 18 February 2014 © Springer-Verlag Berlin Heidelberg 2014
Introduction Recurrent bacterial meningitis is rare but severe disease and its origins should be well investigated. One reason could be anatomical problems mainly localized in the head and neck region [1] such as occult skull base malformations with or without cerebrospinal fluid (CSF) leaks which have no external manifestation. These malformations can be divided into congenital [1–3] and mostly acquired forms with inflammatory, e.g. osteomyelitis [4], iatrogenic [5] or traumatic genesis [1]. They can involve several vessels, skull base and brain structures in the lateral [6, 7], anterior [4], or less often of the medial skull base [4, 8]. Posttraumatic skull base malformation can become symptomatic immediately with persistent CSF fluid fistulas after a head injury which can lead to posture-related headaches and pneumocephalus or meningitis [1]. Furthermore, delayed onsets of symptoms in occult malformations like recurrent meningitis or CSF leaks after years or decades are possible [1, 4]. One variant of these occult skull base malformations can be dural-lymphatic fistulas where the cerebral fluid interspaces anastomose with regional lymphatic vessels and also nerve sheaths so that they are filled with liquor. Durallymphatic fistulas are a very rare vascular malformation of the skull base. That is why they are not well described yet.
C. Koch (*) · A. Lorenz · W. G. Hosemann · A. G. Beule Department of ENT, Head and Neck Surgery, University Medicine Greifswald, Greifswald, Germany e-mail: carmela.koch@uni‑greifswald.de P. Thamm Institute of Diagnostic Radiology and Neuroradiology, University Medicine Greifswald, Greifswald, Germany
For that reason we want to demonstrate one of these unusual cases and its complications.
Case report A 45-year-old woman was presented in our hospital after at least ten episodes of bacterial meningitis with and without simultaneous CSF leaks. Previously, she has had 12 operations involving the nose, paranasal sinuses and frontal skull base due to a known posttraumatic skull base malformation located in the sphenoid sinus and cavernous sinus after head trauma in early childhood (overview of the available information in Fig. 1). Five years before visiting our hospital, two large parasellar and temporobasal liquor accumulations as well as several smaller osseous CSF leaks in the posterior wall and ground of the left sphenoid and maxillary sinus had been described. The liquor seemed to be transported longitudinally along the nerve sheaths which could be observed via intrathecal fluorescein imaging. Following the last neurosurgical operation, there was no doubt that the left cavernous sinus was filled with arachnoidea and liquor and was surrounded by very filmy, perforated bone with diaphanous trigeminal branches. In further histopathological analysis of mucosa, untypically distended lymphatic vessels without direct detection of liquor were provided, so that a complex dural-lymphatic fistula of the skull base was probable. After the tenth meningitis, following a 5-year meningitis-free period, our patient had another recurrent meningitis and accordingly a need for restaging to repair damage and avoid recurrence of meningitis. We began by CT- and MRimaging of the head focusing on the skull base. After this proved ineffective, we conducted an interdisciplinary radiological conference to find additional strategies. We started
13
Eur Arch Otorhinolaryngol Head trauma in childhood (nine years) with the suspicion of skull base malformation of the left sphenoid sinus and cavernous sinus 1 year later 1. meningitis 6 months later CSF leak 3 months later
1. Sinus surgery via eye brow incision
2. meningitis, CSF leak
8 years later
3. meningitis, CSF leak
2. Sinus surgery via eye brow incision 3. Exploration of skull base and covering of the skull base defect with fascia lata
3 months later
CSF leak?
4. Exploration of skull base and covering of the defect with fascia lata
4 months later
CSF leak ?
5. Sinus surgery via transfacial approach
16 years later
4. meningitis, CSF leak
1 year later
5. meningitis, CSF leak, sympt. focal epilepsy
6. Trepanation and covering of the frontal CSF leak with an autologous transplant
1 year later 7. Transsphenoidal covering of an skull base defect on the floor of the sella turcica with transposition of muscle, fat and fascia lata
6. meningitis
7 months later
epidural hematoma
8. Left frontal recraniotomy and decompression of an epidural hematoma
1 year later 9. Surgery of the left rhinobasis, covering of the left CSF leak with fascia lata and exploration of the right frontal sinus 7. meningitis 9 months later 8. meningitis
10. Osteoplastic surgery of the right frontal sinus via coronal or bi-temporal approach and its obliteration with umbilical fat, endonasal endoscopic surgery of the left skull base and sphenoid sinus and duraplastic after intrathecal fluorescein 9 months later 11. Sinus surgery of the left maxillary sinus and obliteration with abdominal fat for occluding several CSF leaks after intrathecal fluorescein application
9. meningitis, CSF leak
4 months later 12. Occlusion of the cystic distended left cavernous sinus and covering of several CFS leaks temporal left with fascia temporalis after recraniotomy and decompression of a left pterional epidural hematoma
5 years later 10. meningitis 13. Reconstruction of skull base via transpterydoidal approuch after intrathecal gadolinium Imaging and after intrathecal fluorescein application and endoskopy of the nose, sinuses and epipharynx preoperative
Fig. 1 Time bar of the patient’s medical history especially concerning symptoms of occult skull base malformation as well as following complications and their surgery
13
Eur Arch Otorhinolaryngol
Fig. 2 MR-imaging of skull base a axial T2 b–d axial, coronal and sagittal T1 after the application of intrathecal contrast agent (gadolinium). asterisk left trigeminal nerve plus liquor-filled defect of the
left cavum meckeli with baggy swelling in the left cavernous sinus arrow contrast agent leakage
with MR-imaging after applying intrathecal gadolinium revealing a defect of the left cavum meckeli with baggy swelling in the left cavernous sinus which seemed to communicate directly with cerebral fluid interspaces. A contrast
agent passage from the cavum meckeli along the trigeminal branches with a maximum in the left posterior ethmoidal cells (Fig. 2) was detected. Two days later, we applied intrathecal fluorescein and performed endoscopy of the
13
Eur Arch Otorhinolaryngol
a
b
right tubal protuberance left tubal protuberance posterior epipharyngeal wall submucosal fluorescein pathways
Fig. 3 Endoscopic view (blue light) in right a and left b epipharynx. Visible are submucosal fluorescein pathways (yellow) and no proof for an active CSF leak
nose, paranasal sinuses and epipharynx in general anaesthesia. This revealed submucosal fluorescein pathways in the epipharynx and near the left sphenopalatine foramen as the probable cause of the recurrent meningitis (Fig. 3). A CSF leak could not be proved. In consequence of these findings, we performed another skull base reconstruction via a transpterygoidal approach and covered the defect as central as possible with fascia lata. Intraoperatively, there were two CSF leaks in the region of the anterior sphenoid wall through the foramen rotundum as well as following the maxillary artery dorsal of the posterior wall of the sphenoid sinus. Afterwards, CSF fluid was no longer visible along the intramucosal nerve sheaths. The postoperative course was free of complications. Currently, with another follow-up of 6 months the patient is free of any disorders and under frequent clinical surveillance. The next re-evaluation is planned in 6 months with an endoscopy in general anaesthesia after an intrathecal fluorescein application.
Discussion Posttraumatic malformations of skull base vessels are rare [1] but can have severe consequences as seen in our case. There are reported cases where adult patients were symptomatic with recurrent meningitis or CSF leaks associated with posttraumatic herniation of subarachnoid space into diploe, paranasal sinuses or orbit following injury having caused a dura tear in the past [9]. This case seems to be similar where arachnoidea and liquor were seen in the cavernous sinus and several CSF leaks in the region of the left sphenoid sinus were observed. Nevertheless, nontraumatic skull base defects leading to spontaneous CSF rhinorrhea
13
could have analogous radiological findings [10], so the patient history is the only chance of discriminating between a traumatic and a nontraumatic origin. Additionally, our patient had a malformation that communicated with regional lymphatic vessels, which transported the CSF in submucosal structures primarily of the left paranasal sinuses and epipharynx, as could be shown in several diagnostic results at different points in time. Bektas et al. described similar findings in a patient with same medical history as our patient. During their investigation, they could also prove intact septal nasal mucosa with submucosal nasoseptal collection of CSF without CSF leaks or rhinoliquorrhea [11] which caused recurrent meningitis. All types of occult malformations causing CSF leaks or recurrent meningitis should be rapidly treated surgically immediately after controlling infections [4] as in our case to avoid serious consequences. However, in spite of sufficient therapy recurrence of skull base malformation including lymphatic-dural fistulas is possible as observed in our case. This shows that there is a need for local and radiological diagnostic findings due to the possibility of long asymptomatic period lasting years or even decades. This is another reason why one should think of skull base malformations in recurrent meningitis or CSF leaks. Exact patient anamnesis and adequate diagnostics are essential.
Conclusion Dural-lymphatic fistulas are rare but severe complications after traumatic craniocerebral injuries can lead to CSF leaks and recurrent meningitis with potentially life-threatening consequences. Correct diagnose-finding can be difficult due
Eur Arch Otorhinolaryngol
to the need of delicate and invasive diagnostic tools which are often unavailable. In spite of high operational expertise and interdisciplinary collaboration successful therapy can be challenging due to the high relapsing rate. Hence, these patients should be sent immediately to specialised medical interdisciplinary skull base centres. They should also stick to permanent and lifelong surveillance for early recognition of recurrence in order and to avoid permanent individual health consequences like persistent impairment or death.
References 1. Tebruegge M, Curtis N (2008) Epidemiology, etiology, pathogenesis, and diagnosis of recurrent bacterial meningitis. Clin Microbiol Rev 21(3):519–537 2. Schick B, Prescher A, Hofmann E, Steigerwald C, Draf W (2003) Two occult skull base malformations causing recurrent meningitis in a child: a case report. Eur Arch Otorhinolaryngol 260:518–521 3. Prescher A (2009) Embryology of skull base malformations. Meeting contribution ESBS 2005: skull base surgery: an interdisciplinary challenge. 7th Congress of the European Skull Base Society held in association with 13th Congress of the German Society of Skull Base Surgery, Fulda, 18–21/05/2005. German Medical Science GMS Publishing House 2009
4. Wang H-S, Kuo M-F, Huang S-C (2005) Diagnostic approach to recurrent bacterial meningitis in children. Chang Gung Med J 28:441–452 5. Bumm K, Heupel J, Bozzato A, Iro H, Hornung J (2009) Localization and infliction pattern of iatrogenic skull base defects following endoscopic sinus surgery at a teaching hospital. Auris Nasus Larynx 36:671–676 6. Windfuhr JP, Sesterhenn K (2002) Spontane Defekte der seitlichen Schädelbasis, Teil 2, Ätiologie und Literaturübersicht. HNO 50:441–463 7. Bryson E, Draf W, Hofmann E, Bockmühl U (2005) Versorgung okkulter Missbildungen der lateralen Schädelbasis. LaryngoRhino-Otol 84:921–928 8. Mulcahy MM, McMenomey SO, Talbot JM, Delashaw JB Jr (1997) Congenital encephalocele of the medial skull base. Laryngoscope 107:910–914 9. Byrne JV, Britten JA, Kaar G (1992) Chronic posttraumatic erosion of skull base. Neuroradiology 34:528–531 10. Schuknecht B, Simen D, Briner HR, Holzmann D (2008) Nontraumatic skull base defects with spontaneous rhinorrhea and arachnoid herniation: imaging findings and correlation with endoscopic sinus surgery in 27 patients. Am J Neuroradiol 29:542–549 11. Bektas D, Caylan R, Bahadir O, Caylan R (2007) Occult anterior skull base defect without rhinorrhea as a cause of recurrent meningitis. Surg Neurol 68:50–52
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