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Spontaneous brain rupture: a complication of untreated hydrocephalus
Clinical Neurology and Neurosurgery 108 (2005) 48–51
Case report
Spontaneous brain rupture: a complication of untreated hydrocephalus Ali Moghtaderia,∗ , Vafa Rahimi-Movagharb , Mohammad Safdarib b
a Neurology Department, Zahedan University of Medical Sciences, Zahedan, Iran Department of Neurological Surgery, Zahedan University of Medical Sciences, Zahedan, Iran
Received 18 July 2004; received in revised form 1 October 2004; accepted 16 October 2004
Abstract A case of infantile hydrocephalus with secondary spontaneous brain rupture is reported. As far as the authors’ knowledge, this is the first case of spontaneous brain and ventricular rupture secondary to high-pressure hydrocephalus. It is a case of infantile hydrocephalus occurring due to the expansility of an infantile skull, which is normally not seen these days, and is a fatal sequela of untreated hydrocephalus. This rare complication occurs with the rupture of thinnest part of the ependymal layer of the ventricle, cerebral tissue, meningeal membranes, bone and scalp. © 2004 Elsevier B.V. All rights reserved. Keywords: Infantile hydrocephalus; Untreated hydrocephalus; Brain rupture
1. Objective and importance
2. Clinical presentation
Marked tension hydrocephalus can occasionally cause ventricular rupture, producing either a ventricular diverticulum or a channel between the ventricles and the subarachnoid space (spontaneous ventriculostomy) [1–5]. The literature contains reports of two cases of congenital atresia of the foramen Monro with a parieto-occipital diverticulum, spontaneous ventriculostomy and formation of a cyst in the supracerebellar region [6], three cases of spontaneous ventriculostomy of the posterior wall of the third ventricle associated with a diverticulum [7] and five cases of ventricular diverticula in obstructive hydrocephalus secondary to tumor growth [8]. Spontaneous brain rupture, which is a complication of untreated hydrocephalus, refers to the extrusion of cerebral tissue and fluid through the extracranial tissues and scalp. As far as we know, this has not been reported in the literature.
An 8-month-old boy had been born after a full-term pregnancy. His head circumference and weight at birth were unknown. At 3 months of age, he was admitted to our hospital because of macrocephaly. At that time, his head circumference was 45 cm (above the 95th percentile). His scalp was normal, and no congenital defects, skin atrophy, ulceration or infection were present. The anterior fontanel was wide and tense, even in the upright position. A brain CT scan revealed the presence of hydrocephalus, but magnetic resonance imaging was not carried out. Despite the hydrocephalus, the patient’s parents refused to have a ventriculo-peritoneal (VP) shunt inserted. Five months later, the patient was brought into the emergency room due to scalp rupture and CSF leakage. His parents reported that after changing his head position from one side to the other on the previous day, fluid had started to leak out through his scalp, which was tense and red, in the left posterior parietal area. On the day of admission, after a sudden spontaneous scalp rupture, the brain tissue was squeezed forcefully out (Fig. 1). On examination, the patient’s temperature was 38.4 ◦ C. The occipital and right parietal bones were depressed, and
∗
Corresponding author. Tel.: +98 541 3234551; fax: +98 541 3218848. E-mail addresses: [email protected], [email protected] (A. Moghtaderi). 0303-8467/$ – see front matter © 2004 Elsevier B.V. All rights reserved. doi:10.1016/j.clineuro.2004.10.015
A. Moghtaderi et al. / Clinical Neurology and Neurosurgery 108 (2005) 48–51
49
Fig. 1. Severe hydrocephalus with hemorrhage and brain extrusion through the left lambdoid suture, viewed from the base of the skull to the top (A–D).
the scalp was torn and deformed. A neurological examination revealed the child to be comatose. His pupils were miotic and responsive to light. The plantar reflexes were absent. A CT scan revealed extrusion of the parieto-occipital lobes through the left lambdoid suture. A thin rim of cortical mantle and marked ventriculomegaly were seen intracranially. A small occipital extra-axial hemorrhage was present. Extensive subdural and subarachnoidal bleeding was observed, which covered the entire extracerebral spaces in the frontal and parietal lobe areas. The right lateral ventricle remained dilated. Multiple foci of pneumocephalus were present. An emergency reconstructive operation was performed on the scalp and skull to prevent meningitis. During the operation, the ventricles were irrigated, excessive bone was resected and a medium-pressure VP shunt was inserted. Empiric treatment with wide-spectrum antibiotics had begun to treat the presumed CNS infection. The patient had a complicated postoperative course, but at the time of
discharge from the hospital 15 days later, he was conscious, and capable of normal sucking and movement. He was subsequently lost to follow-up.
3. Discussion Hydrocephalus rarely remains untreated in modern medicine, so cases similar as this one are seldom seen. Historically, spontaneous leakage of CSF through the scalp was described in the early days of hydrocephalus treatment in the 18th century (the pre-shunt era), when the heads of infant patients were compressed using dressings or plasters in order to prevent macrocephaly. In infants whose cranial sutures have not closed, increased ventriculomegaly is manifested by enlargement of the head due to separation of the sutures. The ventricles enlarge at the expense of the thinning cerebral mantle. Periventricular stress concentrations tend to appear
50
A. Moghtaderi et al. / Clinical Neurology and Neurosurgery 108 (2005) 48–51
around the ventricular horns, which seem to be a direct consequence of the geometry of the ventricular wall [9]. In the lateral ventricles, the frontal and occipital horns enlarge preferentially because of the limited resistance of the surrounding white matter [10–12]. Spontaneous brain rupture might only appear in infantile hydrocephalus if the sutures are not closed and the skull is able to expand. The appearance of CSF leakage into the parieto-occipital area and through the lambdoid suture was not unexpected in this case, due to the factors discussed below. An important factor is the biomechanics of ventricular expansion in the presence of increased intraventricular pressure, and the characteristic pattern of brain distortion in infantile hydrocephalus with a selective thinning of the occipital lobes. This last symptom is partly due to the yielding nature of the cranial vault in these areas, as well as the presence of more rigid nuclear masses in relation to the frontal horns [13]. According to a computer simulation designed to study the biomechanics of acute hydrocephalus, ependymal rupture at the side of both the frontal and occipital horns is predicted to be a result of an expansive (tensile) stress concentration, which initiates the development of tissue (ependyma and brain) rupture and is a direct consequence of the convex/concave geometry of the ventricular wall [9]. The changes in cortical mantel thickness might be explained by shrinkage of the cells [14]. Another, possibly more important factor during the later stages of untreated hydrocephalus is the difficulty for the parents of maintaining the growing skull. Due to the weight of the head and problems with its positioning, skin atrophy or even ulcers and local infection might be caused by local disturbances of the blood circulation and skin metabolism on the side on which the head is usually placed (that is, the parietal or occipital area). This could be the principal external mechanical factor weakening the skin and promoting CSF leakage in cases of untreated ventricular distension in infants. In addition, skin infection and external mechanical irritation might also lead to nutritive problems of the underlying dura, causing dural tears. In infants, the dura is attached to the bone at the suture side more tightly than in adults. It is therefore exposed to dural tears at the suture side, as well as ruptures caused by bone movements or distension of the bone components. With further increased pressure, CSF can ooze through the tense thinned scalp. Under these circumstances, minor scalp trauma might cause lacerations and facilitate the outward migration of the CSF and/or the brain. Thus, scalp defects, brain rupture through the dura and CSF leakage in an untreated infantile hydrocephalus are caused by a combination of internal biomechanical forces as a result of ventricular expansion and external mechanical irritation, which is partly due to problems with positioning the head and nutritive disturbances to the skin while the skull is expanding. The authors would like to state that in our country, physicians can act autonomously to save the life of an infant patient, even if the parents are not available or do not give consent,
only in a life-threatening emergency situation. In all other circumstances, the parents’ permission is mandatory in order to perform a surgical procedure.
4. Conclusion It is important to document the sequels of untreated infantile hydrocephalus. Few doctors have experienced this situation, and this report might encourage physicians to persuade parents to have their children treated in such cases. In rare cases of untreated severe hydrocephalus, a spontaneous ventriculostomy normalizes the intracranial pressure. Otherwise, progressive hydrocephalus might cause the spontaneous extrusion of the brain extracranially. A matter of considerable practical, as well as theoretical, interest is the capacity of the dura to protect against extracranial cerebral herniation. Acute exacerbation of intracranial pressure might induce rupture of both the dura and the scalp. The resulting defect can be repaired by reconstruction of the bone and closure of the scalp. In this case, the residual hydrocephalus was treated with a VP shunt following reconstructive surgery.
Acknowledgement We thank Dr. Leland Albright, Professor of Pediatric Neurosurgery at University of Pittsburg Medical Center (UPMC), USA, for critically reviewing this manuscript.
References [1] Fujita K, Enomoto T, Yanaka K, Nose T. Ventricular diverticulum at the posterior horn of the lateral ventricle presenting as aplasia cutis congenita. Childs Nerv Syst 2001;17:750–3. [2] Rovira A, Capellades J, Grive E, Poca MA, Pedraza S, Sahuquillo J, et al. Spontaneous ventriculostomy: report of three cases revealed by flow-sensitive phase-contrast cine MR imaging. AJNR Am J Neuroradiol 1999;20:1647–52. [3] Ohsato T, Isu T, Tashiro K, Abe H, Takei H, Miyasaka K. A case of subtentorial ventricular diverticulum accompanied with choroid plexus papilloma in the lateral ventricle. No To Shinkei 1987;39: 257–62. [4] Mahapatra AK. Spontaneous ventriculocisternostomy. Indian J Pediatr 1985;52:93–5. [5] Zilkha A. Spontaneous ventriculostomy. Report of two cases demonstrated by Pantopaque ventriculography. Radiology 1974;111: 633–7. [6] Taboada D, Alonso A, Alvarez JA, Paramo C, Vila J. Congenital atresia of the foramen of Monro. Neuroradiology 1979;17: 161–4. [7] Mracek Z. Spontaneous ventriculostomy of the posterior wall of the 3rd ventricle associated with a diverticulum in the infratentorial space and stenosis of the aqueduct. Clinical and radiological study in 3 patients. Cesk Neurol Neurochir 1984;47:377–82. [8] Abe M, Uchino A, Tsuji T, Tabuchi K. Ventricular diverticula in obstructive hydrocephalus secondary to tumor growth. Neurosurgery 2003;52:65–70 [discussion 70–61].
A. Moghtaderi et al. / Clinical Neurology and Neurosurgery 108 (2005) 48–51 [9] Pena A, Bolton MD, Whitehouse H, Pickard JD. Effects of brain ventricular shape on periventricular biomechanics: a finite-element analysis. Neurosurgery 1999;45:107–16 [discussion 116–108]. [10] Milhorat TH. Experimental hydrocephalus 1. A technique for producing obstructive hydrocephalus in the monkey. J Neurosurg 1970;32:385–9. [11] Milhorat TH, Clark RG, Hammock MK. Experimental hydrocephalus 2. Gross pathological findings in acute and subacute obstructive hydrocephalus in the dog and monkey. J Neurosurg 1970;32: 390–9.
51
[12] Clark RG, Milhorat TH. Experimental hydrocephalus 3. Light microscopic findings in acute and subacute obstructive hydrocephalus in the monkey. J Neurosurg 1970;32:400–13. [13] Chapman PH. Hydrocephalus in childhood. In: Youmans JR, editor. Neurological surgery: A Comprehensive Reference Guide to the Diagnosis and Management of Neurosurgical Problems. 3rd ed. Philadelphia: W.B. Saunders Company; 1990. pp. 1237–1240. [14] Bluml S, McComb JG, Ross BD. Differentiation between cortical atrophy and hydrocephalus using 1H MRS. Magn Reson Med 1997;37:395–403.
Case report
Spontaneous brain rupture: a complication of untreated hydrocephalus Ali Moghtaderia,∗ , Vafa Rahimi-Movagharb , Mohammad Safdarib b
a Neurology Department, Zahedan University of Medical Sciences, Zahedan, Iran Department of Neurological Surgery, Zahedan University of Medical Sciences, Zahedan, Iran
Received 18 July 2004; received in revised form 1 October 2004; accepted 16 October 2004
Abstract A case of infantile hydrocephalus with secondary spontaneous brain rupture is reported. As far as the authors’ knowledge, this is the first case of spontaneous brain and ventricular rupture secondary to high-pressure hydrocephalus. It is a case of infantile hydrocephalus occurring due to the expansility of an infantile skull, which is normally not seen these days, and is a fatal sequela of untreated hydrocephalus. This rare complication occurs with the rupture of thinnest part of the ependymal layer of the ventricle, cerebral tissue, meningeal membranes, bone and scalp. © 2004 Elsevier B.V. All rights reserved. Keywords: Infantile hydrocephalus; Untreated hydrocephalus; Brain rupture
1. Objective and importance
2. Clinical presentation
Marked tension hydrocephalus can occasionally cause ventricular rupture, producing either a ventricular diverticulum or a channel between the ventricles and the subarachnoid space (spontaneous ventriculostomy) [1–5]. The literature contains reports of two cases of congenital atresia of the foramen Monro with a parieto-occipital diverticulum, spontaneous ventriculostomy and formation of a cyst in the supracerebellar region [6], three cases of spontaneous ventriculostomy of the posterior wall of the third ventricle associated with a diverticulum [7] and five cases of ventricular diverticula in obstructive hydrocephalus secondary to tumor growth [8]. Spontaneous brain rupture, which is a complication of untreated hydrocephalus, refers to the extrusion of cerebral tissue and fluid through the extracranial tissues and scalp. As far as we know, this has not been reported in the literature.
An 8-month-old boy had been born after a full-term pregnancy. His head circumference and weight at birth were unknown. At 3 months of age, he was admitted to our hospital because of macrocephaly. At that time, his head circumference was 45 cm (above the 95th percentile). His scalp was normal, and no congenital defects, skin atrophy, ulceration or infection were present. The anterior fontanel was wide and tense, even in the upright position. A brain CT scan revealed the presence of hydrocephalus, but magnetic resonance imaging was not carried out. Despite the hydrocephalus, the patient’s parents refused to have a ventriculo-peritoneal (VP) shunt inserted. Five months later, the patient was brought into the emergency room due to scalp rupture and CSF leakage. His parents reported that after changing his head position from one side to the other on the previous day, fluid had started to leak out through his scalp, which was tense and red, in the left posterior parietal area. On the day of admission, after a sudden spontaneous scalp rupture, the brain tissue was squeezed forcefully out (Fig. 1). On examination, the patient’s temperature was 38.4 ◦ C. The occipital and right parietal bones were depressed, and
∗
Corresponding author. Tel.: +98 541 3234551; fax: +98 541 3218848. E-mail addresses: [email protected], [email protected] (A. Moghtaderi). 0303-8467/$ – see front matter © 2004 Elsevier B.V. All rights reserved. doi:10.1016/j.clineuro.2004.10.015
A. Moghtaderi et al. / Clinical Neurology and Neurosurgery 108 (2005) 48–51
49
Fig. 1. Severe hydrocephalus with hemorrhage and brain extrusion through the left lambdoid suture, viewed from the base of the skull to the top (A–D).
the scalp was torn and deformed. A neurological examination revealed the child to be comatose. His pupils were miotic and responsive to light. The plantar reflexes were absent. A CT scan revealed extrusion of the parieto-occipital lobes through the left lambdoid suture. A thin rim of cortical mantle and marked ventriculomegaly were seen intracranially. A small occipital extra-axial hemorrhage was present. Extensive subdural and subarachnoidal bleeding was observed, which covered the entire extracerebral spaces in the frontal and parietal lobe areas. The right lateral ventricle remained dilated. Multiple foci of pneumocephalus were present. An emergency reconstructive operation was performed on the scalp and skull to prevent meningitis. During the operation, the ventricles were irrigated, excessive bone was resected and a medium-pressure VP shunt was inserted. Empiric treatment with wide-spectrum antibiotics had begun to treat the presumed CNS infection. The patient had a complicated postoperative course, but at the time of
discharge from the hospital 15 days later, he was conscious, and capable of normal sucking and movement. He was subsequently lost to follow-up.
3. Discussion Hydrocephalus rarely remains untreated in modern medicine, so cases similar as this one are seldom seen. Historically, spontaneous leakage of CSF through the scalp was described in the early days of hydrocephalus treatment in the 18th century (the pre-shunt era), when the heads of infant patients were compressed using dressings or plasters in order to prevent macrocephaly. In infants whose cranial sutures have not closed, increased ventriculomegaly is manifested by enlargement of the head due to separation of the sutures. The ventricles enlarge at the expense of the thinning cerebral mantle. Periventricular stress concentrations tend to appear
50
A. Moghtaderi et al. / Clinical Neurology and Neurosurgery 108 (2005) 48–51
around the ventricular horns, which seem to be a direct consequence of the geometry of the ventricular wall [9]. In the lateral ventricles, the frontal and occipital horns enlarge preferentially because of the limited resistance of the surrounding white matter [10–12]. Spontaneous brain rupture might only appear in infantile hydrocephalus if the sutures are not closed and the skull is able to expand. The appearance of CSF leakage into the parieto-occipital area and through the lambdoid suture was not unexpected in this case, due to the factors discussed below. An important factor is the biomechanics of ventricular expansion in the presence of increased intraventricular pressure, and the characteristic pattern of brain distortion in infantile hydrocephalus with a selective thinning of the occipital lobes. This last symptom is partly due to the yielding nature of the cranial vault in these areas, as well as the presence of more rigid nuclear masses in relation to the frontal horns [13]. According to a computer simulation designed to study the biomechanics of acute hydrocephalus, ependymal rupture at the side of both the frontal and occipital horns is predicted to be a result of an expansive (tensile) stress concentration, which initiates the development of tissue (ependyma and brain) rupture and is a direct consequence of the convex/concave geometry of the ventricular wall [9]. The changes in cortical mantel thickness might be explained by shrinkage of the cells [14]. Another, possibly more important factor during the later stages of untreated hydrocephalus is the difficulty for the parents of maintaining the growing skull. Due to the weight of the head and problems with its positioning, skin atrophy or even ulcers and local infection might be caused by local disturbances of the blood circulation and skin metabolism on the side on which the head is usually placed (that is, the parietal or occipital area). This could be the principal external mechanical factor weakening the skin and promoting CSF leakage in cases of untreated ventricular distension in infants. In addition, skin infection and external mechanical irritation might also lead to nutritive problems of the underlying dura, causing dural tears. In infants, the dura is attached to the bone at the suture side more tightly than in adults. It is therefore exposed to dural tears at the suture side, as well as ruptures caused by bone movements or distension of the bone components. With further increased pressure, CSF can ooze through the tense thinned scalp. Under these circumstances, minor scalp trauma might cause lacerations and facilitate the outward migration of the CSF and/or the brain. Thus, scalp defects, brain rupture through the dura and CSF leakage in an untreated infantile hydrocephalus are caused by a combination of internal biomechanical forces as a result of ventricular expansion and external mechanical irritation, which is partly due to problems with positioning the head and nutritive disturbances to the skin while the skull is expanding. The authors would like to state that in our country, physicians can act autonomously to save the life of an infant patient, even if the parents are not available or do not give consent,
only in a life-threatening emergency situation. In all other circumstances, the parents’ permission is mandatory in order to perform a surgical procedure.
4. Conclusion It is important to document the sequels of untreated infantile hydrocephalus. Few doctors have experienced this situation, and this report might encourage physicians to persuade parents to have their children treated in such cases. In rare cases of untreated severe hydrocephalus, a spontaneous ventriculostomy normalizes the intracranial pressure. Otherwise, progressive hydrocephalus might cause the spontaneous extrusion of the brain extracranially. A matter of considerable practical, as well as theoretical, interest is the capacity of the dura to protect against extracranial cerebral herniation. Acute exacerbation of intracranial pressure might induce rupture of both the dura and the scalp. The resulting defect can be repaired by reconstruction of the bone and closure of the scalp. In this case, the residual hydrocephalus was treated with a VP shunt following reconstructive surgery.
Acknowledgement We thank Dr. Leland Albright, Professor of Pediatric Neurosurgery at University of Pittsburg Medical Center (UPMC), USA, for critically reviewing this manuscript.
References [1] Fujita K, Enomoto T, Yanaka K, Nose T. Ventricular diverticulum at the posterior horn of the lateral ventricle presenting as aplasia cutis congenita. Childs Nerv Syst 2001;17:750–3. [2] Rovira A, Capellades J, Grive E, Poca MA, Pedraza S, Sahuquillo J, et al. Spontaneous ventriculostomy: report of three cases revealed by flow-sensitive phase-contrast cine MR imaging. AJNR Am J Neuroradiol 1999;20:1647–52. [3] Ohsato T, Isu T, Tashiro K, Abe H, Takei H, Miyasaka K. A case of subtentorial ventricular diverticulum accompanied with choroid plexus papilloma in the lateral ventricle. No To Shinkei 1987;39: 257–62. [4] Mahapatra AK. Spontaneous ventriculocisternostomy. Indian J Pediatr 1985;52:93–5. [5] Zilkha A. Spontaneous ventriculostomy. Report of two cases demonstrated by Pantopaque ventriculography. Radiology 1974;111: 633–7. [6] Taboada D, Alonso A, Alvarez JA, Paramo C, Vila J. Congenital atresia of the foramen of Monro. Neuroradiology 1979;17: 161–4. [7] Mracek Z. Spontaneous ventriculostomy of the posterior wall of the 3rd ventricle associated with a diverticulum in the infratentorial space and stenosis of the aqueduct. Clinical and radiological study in 3 patients. Cesk Neurol Neurochir 1984;47:377–82. [8] Abe M, Uchino A, Tsuji T, Tabuchi K. Ventricular diverticula in obstructive hydrocephalus secondary to tumor growth. Neurosurgery 2003;52:65–70 [discussion 70–61].
A. Moghtaderi et al. / Clinical Neurology and Neurosurgery 108 (2005) 48–51 [9] Pena A, Bolton MD, Whitehouse H, Pickard JD. Effects of brain ventricular shape on periventricular biomechanics: a finite-element analysis. Neurosurgery 1999;45:107–16 [discussion 116–108]. [10] Milhorat TH. Experimental hydrocephalus 1. A technique for producing obstructive hydrocephalus in the monkey. J Neurosurg 1970;32:385–9. [11] Milhorat TH, Clark RG, Hammock MK. Experimental hydrocephalus 2. Gross pathological findings in acute and subacute obstructive hydrocephalus in the dog and monkey. J Neurosurg 1970;32: 390–9.
51
[12] Clark RG, Milhorat TH. Experimental hydrocephalus 3. Light microscopic findings in acute and subacute obstructive hydrocephalus in the monkey. J Neurosurg 1970;32:400–13. [13] Chapman PH. Hydrocephalus in childhood. In: Youmans JR, editor. Neurological surgery: A Comprehensive Reference Guide to the Diagnosis and Management of Neurosurgical Problems. 3rd ed. Philadelphia: W.B. Saunders Company; 1990. pp. 1237–1240. [14] Bluml S, McComb JG, Ross BD. Differentiation between cortical atrophy and hydrocephalus using 1H MRS. Magn Reson Med 1997;37:395–403.