Growing skull fracture in a 5-month old child: a case report.
|Abstract:||Growing skull fractures are a rare complication of linear skull fractures in children. The authors report a case of a growing skull fracture in a 5-month-old patient with a review of the literature. CT and MRI scans revealed a growing skull fracture with complication of leptomeningeal cyst formation. Surgical removal of the cyst, duraplasty and cranial reconstruction were performed. Follow up showed that the patient was stable neurologically and had improving left upper extremity weakness.|
|Article Type:||Case study|
Cysts (Complications and side effects)
Cysts (Case studies)
Magnetic resonance imaging (Usage)
Skull (Case studies)
Skull (Complications and side effects)
Skull (Care and treatment)
Schmidt, John H.
Trenton, Brooke A.
Sheets, Nicholas W.
|Publication:||Name: West Virginia Medical Journal Publisher: West Virginia State Medical Association Audience: Academic Format: Magazine/Journal Subject: Health Copyright: COPYRIGHT 2010 West Virginia State Medical Association ISSN: 0043-3284|
|Issue:||Date: March-April, 2010 Source Volume: 106 Source Issue: 2|
More than 600,000 children are evaluated in the emergency department each year following blunt head trauma and approximately 95,000 have intracranial injuries (11). A study of 278 pediatric patients presenting with head trauma revealed a 29% incidence of skull fracture in patients less than 12 months and 4% in children aged 13-24 months. (6) Growing skull fractures are a rare complication of linear skull fractures and occur almost exclusively in children less than three years of age. (16) These fractures are characterized by a laceration in the dura that enlarges with time to produce a cranial defect. It is important to identify and treat this condition due to the progressive nature of the widening fracture. This is associated with brain herniation and progressive neurologic deficit. (2, 10, 19, 20, 22) We present a case of a 5-month old male who presented with a skull fracture resulting from a motor vehicle collision. Three weeks later, follow up imaging revealed a growing skull fracture in the right parietal region.
A previously healthy 18-week old male infant was transferred to Charleston Area Medical Center (CAMC) after being involved in a motor vehicle collision in which he was an unrestrained passenger. At the scene he was unconscious and was intubated. He had superficial abrasions over his face and bilateral periorbital ecchymoses. He had marked edema over the right parietal aspect of his scalp. Cranial nerve testing revealed small but reactive pupils. He was able to withdraw to pain and flail his right upper extremity. He exhibited no withdrawal to painful stimulus with his left upper extremity. His deep tendon reflexes were decreased throughout. A CT Scan preformed on the day of admission revealed a significant contusion in the frontoparietal region with evidence of a linear skull fracture (Figure 1).
[FIGURE 1 OMITTED]
Twelve days after admission the patient exhibited increased irritability and a subgaleal fluid collection. A repeat CT scan revealed a comminuted fracture involving the left parietal bone as well as a fracture involving the right parietal skull. A large defect at the site of the right parietal skull fracture was noted with evidence of external cerebral herniation into the subgaleal space. MRI confirmed the presence of a significant dural rent with formation of a leptomeningeal cyst (Figure 2).
Definitive surgical address was delayed until cerebral edema had resolved. At three weeks post injury the patient underwent a right parietal craniotomy, dural exposure, dural repair and cranial reconstruction (Figures 3-6).
[FIGURE 2 OMITTED]
The postoperative course was uneventful and he was discharged home. At his two week post-operative follow-up visit the patient was stable neurologically and exhibited mild residual weakness in his left upper extremity. A post-op CT scan demonstrated encephalomalacia in the right posterior parietal region, dilation of the third and lateral ventricles, and calcification in the right subdural region (Figure 7).
At his four-month-post-operative follow up, the weakness in his left upper extremity was resolving with physical therapy. The patient was progressing well, and was discharged from the neurosurgical service.
The complication of leptomeningeal cyst formation following linear skull fractures in young children, though rare, has been identified since the nineteenth century beginning with John Howship's report in 1816.17 Most of the data and observations contributing to the hypotheses of the pathophysiology have been reported in the 20th century. Dyke used the term leptomeningeal cyst in 1937 to describe an enlarging cystic structure at the site of a skull fracture with erosion of the bone edges and diastasis of the fracture line. (3) In 1953 Taveras and Ransohoff concluded that the leptomeningeal cyst was formed by a ball valve mechanism at the dural rent. They deduced through their own surgical experience that leptomeningeal cysts arise from the sequestration of cerebrospinal fluid (CSF) within arachnoid tissue at the fracture site. (21) This theory has since been refuted by repeated observation that the contents of these expanding masses are composed solely of brain matter without a cystic component. Rosenthal et al. conducted an experiment in which India ink injected into the CSF circulation failed to accumulate in the cyst thus providing evidence against the ball-valve mechanism. (19) Finally Pia and Tonnis described the growing skull fracture of childhood to include patients with cysts or cerebral herniation in the fracture. (17)
[FIGURE 3 OMITTED]
A triad of signs strongly associated with the development of leptomeningeal cysts has been described including isolated swelling, neurological symptoms (most commonly hemiparesis, quadriparesis, and seizure activity), and diastasis of the fracture greater than 4 mm. (2,10,19,20,22) Physical exam usually reveals a cranial defect with a bulging, pulsatile mass.2, 5 21, 10 On rare occasions the lesions were depressed rather than bulging. (16) Radiographs taken after the development of the leptomeningeal cyst commonly reveal a widening fracture with margins that have become smooth and elongated. (21) Also commonly seen are ipsilateral ventricular dilation and cystic mass or cerebral tissue herniation. (8,10,15)
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Elevated ICP has been implicated in the development of leptomeningeal cysts. Lateral ventricle enlargement is invariably noted ipsilateral to the lesion, and there have been a few reported cases in which placement of shunts have prevented further growth. (14) However, MRI studies have not provided evidence of transependymal flow or mass effect to support the role of intracranial hypertension. (16) Instead current theory holds that the development of the herniation is secondary to the normal pressure dynamics of the developing brain and lack of ossification of the infant skull; the dilation of the ventricle forms as a result of the herniation. (16)
Observation has revealed necrosis of brain tissue deep to the lesion and degeneration at the edges of the defect. The surface damage is thought to result from the interface of the now unprotected brain parenchyma with the uneven bony surface of the fracture and friction along these surfaces caused by the physiologic pulsations of the brain. (7) The deeper damage may be the result of interference with blood supply after the development of a pressure cone that forms between the lateral ventricle and the skull. (25) Much of the literature supports the notion that this kind of lesion, if left untreated will enlarge to a maximal point and remain stable throughout adulthood without further emergence or worsening of neurological status. (1,17,18,24) However, there have been reports of untreated cases which continue to worsen even into adulthood. (12) It is believed that hemorrhages may occur within the cystic lesion which leads to further enlargement and pressure build-up, thus new neurological complaints can emerge. (12) These patients have been reported to complain of local pain, discomfort, giddiness, or headache dependant on body position resembling greatly the "syndrome of the trephined." (4)
Surgical repair commonly involves dural repair and cranioplasty. (2,5,20) Craniotomy should extend far beyond the edges of the fracture; some reports recommend the craniotomy flap should be twice the area of the bony defect. (7,12) The rationale for removal of such a large portion of the skull in some literature is based on the observation that the dural defect extends far beyond the sclerotic edges of bone. (16) This finding is confirmed in our case (Figure 4). It is imperative that the dura be closed in this procedure for persistence of an opening is associated with a high recurrence of the leptomeningeal cyst. (16) Ventriculoperitoneal shunting has been advocated when the dura cannot be closed, in advanced and recurrent cases, or cases complicated by hydrocephalus. (9,13,25) Debridement of gliotic brain and granulation tissue is recommended. (23)
Growing skull fractures are a rare but treatable complication of skull fractures and should be considered when there is radiographic evidence of pediatric skull fractures. This report details a case of a growing skull fracture developing after a traumatic head injury and demonstrates the techniques of surgical correction. Diagnosis is straightforward, based on a history of head and subsequent progressive development of neurological symptoms and a pulsatile mass over the skull fracture. Treatment consists of cerebral debridement, removal of the cyst, duraplasty, and cranioplasty to prevent enlargement of this lesion and further brain damage.
CT -- Computed Tomography
MRI -- Magnetic Resonance Imaging
CAMC -- Charleston Area Medical Center
CSF -- Cerebrospinal Fluid
ICP -- Intracranial Pressure
Special thanks to Dr. Mary Emmett, PhD from CAMC Health Education and Research Institute.
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Michael Yu, MD
Neurology Resident, Ohio State
University Hospital Columbus, OH
John H. Schmidt, III, MD
Clinical Professor of Neurosurgery, West
Virginia University Charleston Division
Brooke A. Trenton, MD
Pediatrics Resident, Georgetown
University Hospital, Washington, D.C.
Nicholas W. Sheets, MPH
Medical Student First Year, West Virginia
University, Morgantown, WV
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