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Grow Skull Fracture-Special Consideration on a 5 Week Old Case

Ciurea AV¹, Iliescu A², Sandu AM², Gheorghita A³

¹First Department of Neurosurgery, Emergency Clinical Hospital Bagdasar-Arseni, UMF “Carol Davila”

²First Department of Neurosurgery, Emergency Clinical Hospital Bagdasar-Arseni

³Anesthesiology and Intensive Care Unit, Emergency Clinical Hospital Bagdasar-Arseni

Bucharest, Romania

 

Correspondence:

Prof Alexandru Vlad Ciurea

First Department of Neurosurgery

Emergency Clinical Hospital Bagdasar-Arseni, Sos. Berceni Nr. 12, Sector 4, 041915 Bucharest, Romania

Tel: 0040.21.3343025, E-mail : This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Grow skull fracture (GSF), also known as leptomeningeal cyst, craniocerebral erosion or posttraumatic cephalocele, is a rare complication of skull fracture, mainly encountered during infancy and early childhood, characterized by progressive enlargement of diastatic fracture line, along with leptomeningeal cyst and brain hernia.

We report a case of a 5 weeks old girl, who suffered a head trauma. Following trauma the child developed a progressive growing right frontal mass. CT-scan and 3D reconstruction CT-scan showed the skull defect, leptomeningeal cyst, and brain herniation protruding through the fracture line. History trauma, child’s age, history of progressive growing mass following trauma, clinical examination at admission and imagistic studies conducted to the diagnostic of GSF.

The child was operated. Postoperative antiepileptic therapy was initiated. The patient has good outcome, without neurological deficits or seizure under therapy. Grow skull fracture is specific posttraumatic lesion in infants and young children. Surgery is always required to prevent neurological deficits and/or seizures occurrence. Cranioplasty is not indicated in infants.

Key words: grow skull fracture, leptomeningeal cyst, craniocerebral erosion, posttraumatic cephalocele, infant, trauma

  

Introduction

Grow skull fracture (GSF), also known as leptomeningeal cyst, craniocerebral erosion or posttraumatic cephalocele, is a rare complication of skull fracture, mainly encountered during infancy and early childhood [1]. GSF was described for the first time by Howship in 1816. In 90% cases, it is found in children under 3 years old. It is characterized by progressive enlargement of diastatic fracture line, along with leptomeningeal cyst and brain hernia.

It is usually located in the parietal region, and the reported incidence is only 0.05 to 1.6% of skull fractures in childhood [2-5]. History of trauma, clinical aspects and cerebral CT-scan establish the diagnostic. The treatment of GSF is exclusively surgical. Sometimes it requires dural and bone defect grafting.

Case report

We report a case of a 5 weeks old girl, admitted into the First Department of Neurosurgery, Emergency Clinical Hospital Bagdasar-Arseni, Bucharest. The child suffered a minor head trauma, 2 weeks previously, accidental fall from 1.5 m. Family noted that the child developed a progressive growing right frontal mass. The child had normal cranial development prior head trauma.

Clinical examination at admission showed a fully alert child, without neurological deficits, and presenting a right frontal cystic, nontender mass underlying palpable bony defect. Child’s mother had no health problems during pregnancy. The child had normal development till the age of 5 weeks. She presented no loss of consciousness, seizures or neurological deficits following head trauma. Head examination noted a 3.5/2 cm bony defect within the right frontal area, at the level of coronal suture. The bony defect riches superiorly the anterior fontanelle. Through bony rims a cystic, nontender mass was noted, in bold relief of approximately 2.5 cm in the highest point. Superjacent skin showed no changes, when compared to the rest of the scalp. Child’s family refused taking pictures of the child, so preoperative pictures cannot be provided.

grow skull 1

Figure 1. Diastatic skull fracture; leptomeningeal herniation through diastatic fracture; brain contusion.

Laboratory tests and plain thoracic X-ray were normal. CT-scan (fig. 1) and 3D reconstruction CT-scan (fig. 2) showed the skull defect, leptomeningeal cyst, and brain herniation protruding through the fracture line. History of head trauma at the age of 3 weeks, history of progressive growing mass following trauma, clinical examination at admission and imagistic studies conducted to the diagnostic of GSF. Surgical treatment was proposed. The child was operated, in 2 days time after admission, under general anesthesia, orotraheal intubation and mechanical ventilation.

The child was positioned supine on the operating table, with head turn 45° to the left, to expose the lateral right side of the head.

Skin was incised in “horse shoe” manner, having the fracture in the center. After taking-off the skin flap, a leptomeningeal cyst protruding through the skull fracture rims was indentified (fig. 3). The leptomeningeal cyst was punctioned, and CSF evacuated, in order to release pressure of the cystic mass (fig. 4). Cyst collapsed, revealing the borders of the fracture. The fracture was extended to a craniectomy, with the aid of a Kerrison, until free borders of the normal dura on both sides of the cranial defect were exposed. Lacerated dura and herniated brain laceration were removed (fig. 5 and 6). We performed duraplasty by periosteal patch (fig. 7). Cranioplasty was not performed, taking in consideration the age of the patient (figure 8). Wound was closed in multiple layers.

Postoperative course was uneventful. Postoperative antiepileptic therapy was initiated. The patient has good outcome, without neurological deficits or seizure under therapy.

Discussion

More than 90% of growing skull fractures occurs in children under 3 years old and in infants under 1 year old [2,3]. Grow skull fractures have been reported in utero [6], after instrumental delivery [7-9], falls, road traffic accidents and other head traumas. Grow skull fracture are usually located at the cranial vault, into parietal or frontoparietal areas. Skull base [4,5,10,11] and posterior fossa [10,12,13] can also be involved.

Time interval from head injury and positive diagnosis of GSF can go up to many years [14,15]. GSF develop within 3-4 months following head trauma. Specific signs, such as progressive enlargement of skull fracture and leptomeningeal cyst protruding through the bone defect occur late during the natural history of GSF. The cyst is pulsating with child’s cough and cry. Differentials must be done in infants with cephalhematoma, usually occurring in infants at the cranial vault, like GSF.

grow skull 2

Figure 2. Disjunction of the coronal suture, reaching up to the anterior fontanelle

Conditioning factors contributing to GSF occurrence, such as rapid brain growth and brain pulsation, are common in young infants and children. Other factors contributing to GSF development are intracranial pressure, leptomeningeal cysts, parenchimatal injury and alteration of CSF flow [16].

Identification of risk group for developing GSF immediately after trauma is challenging. Diastatic fracture and dural tear are necessary for developing GSF. Cranial X-rays and cerebral CT cannot identity dural tears [2,7,11,12]. Cerebral MRI can detect dural tears, and the predictive value of developing GSF is high. Unfortunately, we, like many other fracture line can be used successfully to identify dural tears [2,8].

Pathogenesis of GSF consists of three phases. First, a linear skull fracture with periosteum tear and dural laceration occurs, than healing of the fracture margins is hindered by the presence of an intracranial hypertension syndrome and constant pulsation of the CSF, with invagination and entrapment of arachnoids into a diastatic fracture. During the last phase, there is an important bone diastasis, associated with leptomeningeal herniation and elevated intracranial pressure [17]. The osteodural defect enlarges due to leptomeningeal and brain tissue hernia through the defect, preventing apposition of bone. A progressive gliotic tissue occurs on the underlying brain tissue with a subsequent development of a porencephalic cavity [3, 7, 14, 18].

grow skull 3 grow skull 4
Figure 3. Leptomeningeal cyst protruning through the fracture borders, immediatly under the skin. Figure 4. Punction of the cyst and CSF vacuation.

grow skull 5

Figure 5 - 6. Lacerated dura and lacerated brain removal

grow skull 7

Figure 7. Duraplasty with periosteum.

grow skull 8

Figure 8. Postoperative 3D reconstruction CT; Frontoparietal craniectomy. 

Clinically during the first phase occur bone diastasis and seizures, during the second phase bone diastasis, seizures and motor deficits and during the third phase the patients develop large bone diastasis, focal neurological signs and increased cranial pressure.

Cerebral CT-scan, native bone window and 3D reconstruction Surgery is always required to prevent neurological deficits and/or seizures occurrence.

Tight dural closure is the key step in surgical management. Dura is often lacerated and cannot be sutured. Resection of the lacerated dura is performed followed by duraplasty with periosteum, free flap or pediculated, or arficial dura. We prefer dural closure with free flap periosteum, water-tight sutured to the free border of normal dura. Dural tear usually extends beyond the fracture borders.[4, 16, 19, 20] Extending the fracture to craniectomy, free borders of dura are reveled. In many cases dural tears involves dural sinus (transverse or sagittal) [12]. In repairing the dura bleeding from dural sinus can occur, that in this particular age, can be life-threatening. Even though the sinus is not teared in the initial trauma, it may pose problems regarding dural repair. If the fracture line extends perpendicularly to the sinus the end closest to the sinus does not need repair. If the fracture is parallel to the sinus, dural repair is difficult owing to the narrowness of the dural edge next to the sinus. In cases in which the proximity of the venous sinus does not allow graft suture with borders of intact dura, periosteum can be suture with dura across the sinus after extending the craniectomy or directly to the skull edge above the sinus. We extended the fracture to a right frontoparietal craniectomy over the coronal suture, reaching up to the anterior fontanelle. We suture a patch of periosteum to free dural borders. We preferred periosteum because it is an autologous tissue and most convenient dural substitute. Water-tight dural closure is mandatory to avoid GSF recurrence or CSF leakage.

Cranioplasty depends on the age of the patient. Our attitude regarding cranioplasty, is that we do not recommend cranioplasty in children under 3 years old. We consider cranioplasty in young children dangerous because a child’s head is growing, and there is a high risk of bone graft displacement. Besides, new bone apposition within the skull defect occurs in most children. In infants dura mater has osteogenic potential [21-24]. Cranioplasty with methylmethacrylate was reported with good long-term follow-up [3]. CSF shunt diversion is indicated in CSF leakage occurring in a patient with adequate repair of the dura mater and concomitant hydrocephalus.

Conclusions

Grow skull fracture is a specific posttraumatic lesion in infants and young children. Surgery is always required to prevent neurological deficits and/or seizures occurrence. Tight dural closure is the key step in surgical management. Duraplasty can be done with a patch of periosteum. Cranioplasty is not indicated in infants.

 

 

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