Remote intracranial hemorrhage following surgery for giant orbitofrontal growing skull fracture: A lesson learnt.

Growing skull fracture is an extremely rare complication of pediatric head injury, especially in infants. Repair of the dural tear early in the course of development of growing skull fracture has been suggested for a better outcome. Surgical repair of large, tense growing skull fractures, especially those in the communication of the ventricles can lead to potentially life-threatening complications. The author reports a rare case of remote intracranial hemorrhage following surgery for large, tense growing skull fracture in a 12-year-old girl and discusses the likely pathogenesis and possible ways to avoid this life-threatening complication.

Introduction

Growing skull fracture occurs following head injury in the first 3 years of life, particularly in infancy.[1] Progressive diastasis of the fracture line with underlying dural tear results in herniation of arachnoid through the fracture line and formation of a cyst.[2] The watertight dural closure is standard treatment recommended early in the course of fracture development.[3] Remote site intracranial hemorrhage following surgery for growing skull fracture is a serious, life-threatening complication which has not yet reported in the literature.

Case Report

A 12-year-old girl presented with progressively increasing swelling over the right forehead since the age of 4 years. She had suffered a minor head injury few months before the onset of swelling. She had received symptomatic treatment for the head injury and had made an uneventful recovery. Six months after the head injury, she had repeated seizures which were not very well controlled on antiepileptic drugs. Her scholastic performance was poor. On examination, she had a right axial proptosis with a large frontal tense cystic swelling. Fundus and neurological examination were normal. Magnetic resonance imaging of the brain demonstrated the right frontal leptomeningeal cyst which was similar to cerebrospinal fluid (CSF) intensity on all sequences [Figure 1a–c]. The cyst was in communication with the right lateral ventricle with septations within it. The cyst was also seen extending into right orbit through a defect in orbital roof [Figure 1d]. The lateral and third ventricles were disproportionately dilated as compared to the fourth ventricle. There was no evidence of periventricular lucency. Computed tomography (CT) of the brain demonstrated right frontal bone defect with the expansion of the outer and inner table at the edge of bone defect [Figure 1e and f]. CSF from the cyst was aspirated on the night before surgery. The next day she underwent right frontal craniectomy, and the dural defect was identified. The dural edges were defined, and the dural defect was sutured using pericranium. Cranioplasty was done using polymethyl methacrylate bone cement (DePuy International Limited, Johnson and Johnson, United Kingdom). As the patient did not wake up from anesthesia, emergency CT scan was performed which revealed remote site right temporoparietal hematoma with intraventricular extension [Figure 2a]. The external ventricular drain was placed in right lateral ventricle and craniotomy was done to evacuate the hematoma. She was managed with best supportive care. Follow-up scan 3 weeks later revealed panventriculomegaly [Figure 2b]. Medium pressure Chabbra ventriculoperitoneal shunt was inserted. At the end of 6 weeks, during discharge, her glasgow outcome scale was 3 which remained unchanged on follow-up at 3 months.

Figure 1

Magnetic resonance imaging of the brain demonstrates a right frontal leptomeningeal cyst similar to cerebrospinal fluid intensity on all sequences (a-c). The cyst is in communication with the right lateral ventricle with septations within it (c). The cyst is seen extending into right orbit through defect in orbital roof (d, arrow). Computed tomography of the brain shows right frontal bone defect with expansion of the outer and inner table at the edge of bone defect (e and f)

Figure 2

Computed tomography scan of the brain performed after surgical repair of growing skull fracture reveals remote site right temporoparietal hematoma with intraventricular extension (a). Follow-up scan 3 weeks later shows dilated temporal horn (b)

Discussion

A growing skull fracture is an extremely unusual but known neurological complication of pediatric head injuries.[4] It is also known as a leptomeningeal cyst or craniocerebral erosion.[34] It commonly manifests as progressively enlarging scalp swelling following skull bone fracture involving dural tear.[13] It may also result in seizures, cranial asymmetry, and neurological deficit.[23] The early surgical intervention involves performing craniectomy, repair of the dural defect, and closure of the bone.[4]

Surgical repair for growing skull fracture rarely results in life-threatening complications. The occurrence of remote site hemorrhage, i.e., hemorrhage occurring at a distant site from the neurosurgical site, either on ipsilateral or contralateral site, is an extremely unusual complication resulting in significant morbidity or mortality.[25] Remote site intracerebral hemorrhage following surgical repair of growing skull fracture has not been reported in literature until date. Remote cerebellar hemorrhage following supratentorial neurosurgical procedures has been described.[125] The most likely explanation is an extensive loss of CSF from basal cisterns during supratentorial surgery results in sagging of the cerebellum with stretch on superior vermian vein causing hemorrhage.[125] Another possible theory is the occurrence of craniocephalic disproportion due to hydrocephalus.[6] In the presence of hydrocephalus, the ratio of brain parenchyma to intracranial volume is relatively small. Greater is the craniocephalic disproportion; larger is the potential for development of intracranial hemorrhage as in the present case.[6]

In this case, the growing skull fracture was seen to be in communication with the lateral ventricle. Sudden decompression of the ventricle following drainage of the cyst resulted in intracranial hypotension. Remote site intracranial hemorrhage occurred due to sudden, rapid as well as substantial loss of CSF from the lateral ventricles. This lead to sagging of the ipsilateral cerebral hemisphere and stretching of the ipsilateral bridging veins, i.e., vein of labbe with resultant tear leading to hemorrhagic venous infarct.[12] The occurrence of greater craniocephalic disproportion due to excess CSF in the ventricles and the cyst could also be a contributing factor.[6]

The only probable way to reduce the risk of occurrence of this life-threatening complication in large, tense growing skull fracture communicating with the ventricle is to prevent acute decompression of the ventricles and the cyst. Insertion of ventriculoperitoneal shunt would inadvertently, result in a gradual reduction in intracranial pressure. This would prevent rapid, sudden brain shift thereby avoiding avulsion of the bridging veins. Placement of the shunt should then be followed by dural repair and bone closure. Delayed diagnosis, large bone defect, delayed repair, and occurrence of life-threatening complication lead to a poor outcome in the present case.

Conclusion

Remote site hemorrhage is a potentially fatal complication in large, tense growing skull fractures which communicate with the ventricles. CT of the brain needs to be performed in patients who do not recover from anesthesia or those who deteriorate in the postoperative period. Slow drainage of CSF by placement of ventriculoperitoneal shunt is likely to cause a gradual reduction in intracranial pressure, thereby minimizing the chances of occurrence of this unusual complication.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.