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    British Journal of Neurosurgery (1994) 8 , 667-679

    ORIGINAL ARTICLE

    Growing skull fractures: classification and management

    N A I M - U R - R A H M A N , ZA I N A L A B E D E E N B . J A M J O O M , A B D E L H A K I M B .

    J A M J O O M & W A L E E D R . M U R S H I D

    Division of Neurosurgery, College of Medicine, King Saud University & King Khalid University

    Hospital, P.O. Box 2925, Riyadh 11461, Saudi Arabia

    Abstra c t

    Seven patients with growing skull fractures treated between 1983 and 1993 are described. These growing

    fractures consti tute d 1.6% of all the cases of skull fractures seen during the period (a total of 449 c ases) . Base d

    on aetiopathogertesis, computed tomography (CT) appearances, operative findings and management strategies

    requir ed, three main types of growin g skull fractures were reco gni sed. In type I (n 3) a lept omcn ingc al cyst, in

    type II in = 3) damaged and gliotic brain, and in type III (n = 2) a porencephalic cyst extended through the skull

    defect into the subgalcal sp ace. A combi nat ion of type I and type III co-e xist ed in one patient . Initial head injury

    and neurolo gical deficit were judged to be mi ld to mod erat e in all the seven cases. C ont inu ed growth of skull

    fractures correlated closely to the increasing neurological deficit in five cases. In two patients natural arrest of

    fracture growth at 5 and 7 months after trauma was accompanied by arrest in progress of neurological deficit.

    Available surgical options are discussed and general guidelines for the management are given.

    Key wo rds : Trauma, lepzomeningeal cyst, porencephalic cyst, meningocele spuria, progressive neurological deficit, skull

    fracture

    Introduction

    Linear or non-linear skull fractures in chil

    dren that enlarge with t ime are termed grow

    ing skull fractures.1

    Although these lesions are

    much more common in ch i ld ren ,2

    and 90%

    occ ur un de r the age of 3 years;3

    the process

    may occur following a skull fracture in an

    a d u l t .2 , 4 , 5

    The incidence of 'growth ' as a de

    layed compl icat ion of skull fracture is rare and

    occ urr ed in only 0. 6% of the cases in on e large

    series ,6

    It is important to realize that the lesion

    expands not only between the frac ture edges ,

    but also intracranially and, thus, may cause

    atrophy of under lying cereb ra l t issue with re

    sul t ing progress ive neurological defic i t ;7

    as was

    seen in mo st of our cases. Beca use of the

    diverse clinical, radiolo gical (C T) an d ope rat

    ive f indings and variable temporal course ,

    there is controversy concerning the terminol

    ogy, ae t iopathogenes is and management of

    growing skul l frac tures .3

    '6 , 8

    Classification of

    the growing skull fractures into three types

    (Fig. 1) suggested here was found to be help

    ful in explaining these diversities and planning

    the treatment. Similarly, based on the clinical

    presenta t ion and temporal course , two formsof the gro wing skull fractur es could be di s

    tinguished. An active form with evidence of

    raised intracranial pressure (ICP), mass effect

    on C T , progress ive separa t ion of bo ne edges

    with a tense bulge between them; and an ar-

    667

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    668 Naim-Ur-Rahman et al.

    S e a r r t d a r a c h n o i d

    TYPE m

    FI G. 1. Th e three main types of the growing skull fractures.

    rested or 'burnt out' form with a normal ICP,

    and a slack/sunken gap between the bone

    edges often showing som e evide nce of bon e

    regrowth and healing at the fracture site.

    Duro-cranioplasty was the correct treatment

    for type I fractures without raised intracranial

    pressure; whereas a shunting procedure was

    required as an initial or definitive management

    for type I and III fractures with raised intracra

    nial pressure.

    Report of cases

    A su mm ar y of the seven patien ts is pres ent ed

    in Table L and management options summa

    rized in Table III .

    Clinical Data

    Six patients were children while one was 39

    years old. In the paediatric group, the age at

    the tim e of diagnosis rang ed from 4 mo nt hs to

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    Growing skull fractures 669

    6 years (mea n: 24,6 mont hs) . Six pat ien ts

    were female and one male. The interval be

    twe en the history of he ad injury and diagn osis

    of grow ing skull fracture ra nge d from 2

    months to 1 year (mean: 7 months) . Locat ion

    of the skull fracture was pariet al in th ree

    pat ients , frontoparie ta l in one, occipi toparie ta l

    in one, and occipital in the remaining two

    children.

    Two children (cases 1 and 2) had experi

    enced permanent remiss ions af ter a period of

    act ive frac ture growth. Th e rema inin g f ive

    sho wed evide nce of raised intracr anial pres s

    ure and more or less progressive neurological

    deficits . Five children presented with swelling

    in the re gion of th e fra ctur e site, whi le in two

    (cases 1 an d 7), the area be tw een the gaping

    bone edges was slack and depressed. Seizures

    and hem ip a re sis were the next common

    present ing symptoms and occurred in four

    patients. Blindness or progressive visual

    deterioration was present in two children with

    occipital fractures, while retardation was seen

    in one child.

    Radiological evaluation

    Plai n radio gra phs of the skull an d CT were

    performed on a l l pa t ients . Skul l radiographs

    correct ly demonstra ted the locat ion and extent

    of separ ation of bo ny edges at the fra cture site.

    Extens ive bony resorpt ion with widely gaping

    everted fracture margins, seen in three chil

    dre n, corr elated well with the size of the u n

    derlying cyst and brain damage on a CT scan.

    CT clearly demonstra ted the bony defect ,

    the natu re of intracrania l cont ents hernia t ing

    th ro ug h it (lepto meni ngea l cyst = 3, con tuse d

    cereb ral tissue = 3, por enc eph ali c cyst -2);

    and the extent of the underlying intracranial

    les ion. Thus , a leptomeningeal cys t with CSF

    density was seen in three patients, extensive

    low density areas of bra in dam ag e an d en-

    cephalomalacia in three pat ients and poren

    cephal ic cys ts communicat ing with di la ted

    ventricles in two children.

    Operative findings

    Five of the seven pat ien ts und er we nt surgery.

    One child (case 4) had a ventriculopcritoneal

    shunt for tense porencephalic cyst and dilated

    ventricles. In the remaining four children the

    defect was repaired by duroplasty using fascia

    lata or pericranial graft, and an acrylic cranio-

    plasty. Of these four chil dren who un der

    went craniotomies at the fracture sites, two

    (cases 2 and 4) showed that the scalp and

    pericranium were densely adherent to the

    underlying conges ted, gl iot ic and hernia ted

    brain. The dura was totally absent under the

    cranial defect. More or less extensive nibbling

    of the ma rgi ns of bo ne defect was requi red to

    find a reas ona ble dural e dge. D issect ion an d

    elevation of the scalp flap from the unde rly ing

    congested gliotic brain was tedious and ac

    companied by much bleeding. In case 5, oper

    ation was performed for a large tense occipital

    swelling thr ou gh a growin g fracture of the

    skull. Ele vat ion of a large occi pital scalp flap

    revealed a porencephalic cyst on the right side

    and a leptomeningeal cyst overlying a con

    gested and softened left occipital lobe. The

    occipital cyst was aspirated and an acrylic

    cranioplas ty carr ied out . Durocranioplas ty was

    straightforward in the seventh case.

    Surgical results

    Com ple te reso lut ion of the ra ised intracrania lpressure, extracranial swelling and progressive

    closur e of skull defect was o bta ine d in the

    child who underwent shunt surgery (case 3).

    Fol low-up a t 3 and 6 months has shown re

    du ct io n in th e size of cran ial defect with evi

    den ce of bo ne regr owt h at the fract ure site.

    Co mp le te obli tera tion of the crania l defect was

    achiev ed in three ou t of four pat ien ts who

    underwent duro-cranioplas ty (mean fol low-

    up : 4 years ). In the fo urth pati ent, the acrylic

    plate loosened and bulged 2 years after

    surger y. Re ope rat ion was refused. All the five

    pat ients who underwent surgery recoveredwithout any increase in the pre-exis t ing neuro

    logical deficits and definite improvement was

    recorded in four pat ients .

    Case 1. This 30-monrh-old gir l was wel l unt i l

    10 months before admiss ion when she was

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    TA BL L Summary of seven patients with growing skull fractures-Jo

    Case

    no. Age/sex*

    Location of

    fracture

    Type &

    temporal

    classification Presentat ion and c'imca] examinat ion

    Skull

    radiography

    C T

    appearance Treatment Outcome

    53

    30 months

    F

    72 months

    F

    4 months

    F

    Parietal

    Parietal

    Occipital

    15 mont hs Occipital

    I

    Arrested

    (burnt-out)

    II

    Arrested

    [|]

    Active

    36 mont hs Frontoparietal II

    h" Active

    I&III

    Active

    Slack depression in the region of

    fracture^ improving left berra'paresis,

    Retardation

    Slack swelling right parietal region

    seizures, minimal left hemiparesis

    Tense, increasing bulge overlying

    left occipital bone defect.

    RICPj vomitings papi l ledema,

    ?Blindness

    Tense swelling left forehead,

    occasional headaches and vomiting,

    seizures, Q progressive) right

    hemiparesis

    Tense enlarging swelling occipital

    region, blindness, progressive

    neurologic deficits

    Gaping bone

    defect with

    everted margins

    Gaping defect

    right parietal

    Large, wedge-

    shape bone gap

    with extensive

    re^irprion

    Atrophic (drained) right

    parietal leptomeningeal

    cysts with ipsila i:ral

    pulling of midline

    Low density, atrophic

    cerebral tissue

    underlyin g skull defect

    I-argc left oceipital

    porencephalic cyst

    communicating with

    the dilated lateral

    ventricles and

    herniating through the

    occipitoparietal defect

    Extensive gaping

    fracture left

    fronto-parietal

    involving anterior gaping fracture

    cranial fossa

    Low density atrophic

    cerebral tissue

    herniating through

    Gaping,

    bioccipital

    bony defects

    Conservative

    ? Spontaneous cure

    Duropla&ty and acrylic

    craniopiasty

    Cysto- ventricuiop eritoneal

    shunt

    Bilateral occipital

    cephalomalacia? large

    right occipital

    porencephalic cyst

    communicating with

    dilated lateral

    ventricles and

    herniating through bone

    defect + left

    leptomeningeal cyst

    Duroplasty and acrylic

    craniopiasty

    Acrylic craniopiasty

    followed by

    recurrence,

    followed by repeat

    duro-cranioplasty

    Being followedjf

    Evidence of

    improving deficits

    and bone regrowth

    at fracture site

    C u r e d !

    Curedf

    Recurrence!

    after surgery

    Improved!

    Followed-up to 3

    years has

    defective vision

    V.E.P. = poor

    response =

    cortical

    blindness

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    672 Naim-Ur-Rahman et al.

    TA BL E II. Management options

    Growing skull fracture

    With normal

    1CP

    With raised

    1CP

    Tense porencephalic

    cyst with

    hydrocephalus

    Ventri c ulo peritoneal

    shunt

    I

    I

    I

    I

    Tense

    leptomcningcal

    cyst

    Cysto-peritoneal

    shunt

    I

    I

    Duro-cranioplasry

    tre ate d for he ad injur y with a frac ture of th e

    skull. Over the next 5 months the child devel

    ope d bulgin g of the scalp i n the right parieta l

    region which became progressively larger.

    This was accompanied by progressive left

    hem ip ares is , headache, vomiting and oc

    casional seizures. Five months prior to ad

    miss ion the mother not iced a spontaneousimprovement in chi ld ' s condi t ion. The scalp

    bulge became softer then rapidly disappeared

    leaving a depressed gap between the bone

    edges . This was accompanied by resolut ion of

    headache and vomit ing, and improvement in

    the left-sided weakness. The child was admit

    ted to the neurosurgical unit with the chief

    com pla int s of a soft gap on rig ht side of hea d,

    seizures and residual left hemiparesis. Examin

    ation revealed a parietal bony defect with a

    slack depression, in an alert girl walking inde

    pendent ly . Neurological examinat ion showed

    minimal left hemiparesis with pyramidal signsand mild re tardat ion. Skul l radiographs on

    admission showed a gaping right parietal frac

    ture with everted saucerized margins . CT

    (Fig. 2) showed a leptomeningeal cys t under

    lying the crania l defect and communicat ing

    freely with the pcrimesencephalic cistern. As

    ther e was evid ence of regr owth of bo ne edg es

    (Fig, 2) a long with cont inued improvement in

    her neurological status, it was decided to ob

    serve. At 3-month follow-up the bone gap had

    na r rowed .

    Case 2. This 6-year-old girl presented withseizures and a 'soft area' over the right parietal

    region, noticed for the last 4 years. A head

    injury had occurred when the child was 10

    months old. One year after the head injury the

    parents noticed a soft, gradually increasing

    bulge in the right parietal region which gradu

    ally 'flattened* in time leaving a 'soft area'.

    Th er e was no his tory of head ache o r vomit ing.

    Examination revealed an alert, bright child. A

    skull defect in the right parietal region measur

    ing about 4 cm and covered by slack scalp

    could be fe l t . Neurological examinat ion re

    vealed minimal left hemiparesis with brisk tendon jerks . There was no papi l ledema. Skul l

    rad iog rap h (Fig. 3) sho wed a gap ing fracture

    in right parietal region. CT (Fig. 4) showed a

    low density lesion of the und erl yin g brai n wit h

    slight dilat ation a nd ipsilateral 'pull ing' of the

    right lateral ventricle. At operation, a large,

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    Growing skull fractures 673

    k AFCG. 2, Case 1: CT scan showing lcptomening eal cyst

    underlying cranial defect and its communi catio n with the

    perimcscncephalic cistern (arrow). Note regrowth of

    fracture edges.

    righi fronto-parietal scalp flap was raised.

    Dense adhes ions between the scalp and un

    derlying brain were dissected. Fracture edgeswere nibbled to expose normal dura . Tissues

    were abnormally vascular and bled excessively.

    Duroplasty and acrylic cranioplasty was car

    ried out. Postoperatively the patient did very

    well and was discharged home on phenytoin.

    Three years la ter , she is asymptomatic and

    free of seizures.

    Case 3 . This 6-month-old girl was seen in the

    neurosurgical clinic with the history that she

    fell and struck the back of her head on a

    concrete floor at the age of 1 month. Shortlyafterwards, a bulge was noticed in the left

    occipi to-parie ta l region. This bulge became

    progressively larger and tense. On admission

    the child was irritable, refused feeds and had

    been vomiting for past few days. Skull radio

    graphs showed a widely gaping wedge-shaped

    bone defect with extensive resorption in the

    left occipito-parietal region. CT (Fig. 5a and

    b) showed a large porencephal ic cys t commu

    nicat ing with the occi pital ho rn of a dil ated left

    la tera l ventr ic le and hernia t ing through the

    wide bony gap into the tense occipital bulge.

    The chi ld was t rea ted with ventr iculo-peri-

    toneal shunt following which the occipital

    bulge and hydrocephalus resolved. At fol low-

    up examinations at 3 and 6 months, the child

    was thriving, the occipital bulge had disap

    peared and the bone defect was much smaller,

    FlG. 3. Case 2: skull radiograph y, lateral view, sho win g

    a widely gaping parietal fracture.

    F l G . 4. Case 2:CT scan showing the right parietal bone

    defect and the underlying low-density brain lesion.

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    6 7 4 Naim-Ur-Rahman et al.

    FI G. 5. (a) and (b) CT scans sho wn a large left occip ital

    ventricle (a) and herniating, through the wide bony gap

    with definite eviden ce of bo ne re- grow th at the

    fracture edges.

    Case 4. This 3-year-old girl was originally

    admitted and treated elsewhere for a linear

    fractu re of left front o-par ietal reg ion. Seven

    months after the original head injury, she was

    admit ted to our neurosurgical uni t because a

    bulge had developed over the left forehead

    which became progressively larger. There was

    a his tory of occas ional headaches , vomit ing,

    seizures and progressive right hemiparesis dur

    ing the preceding 3 months . On examinat ion

    there was a tense linear swelling in the left

    forehead extending from the left fronto

    parie ta l region to the supra-orbi ta l margin

    and ro ot of the no se. T he mar gin s of the

    underlying skull defect could be palpated. She

    had mil d weakn ess of right ar m and leg but

    no pa p i l l ed em a . Sku l l rad iograph (F ig . 6 )

    showed a linear defect in the left fronto

    parietal region extending to the supraorbital

    margin. CT (Fig. 7) showed the gaping frac

    ture and an underlying low density brain

    les ion. At opera t ion, a large frontoparie to-

    temporal sca lp f lap was ra ised. Underneath

    the flap an adhe ren t linear bulge of gliotic

    brain was seen between the gaping fracture

    edges . This hernia ted cerebra l t issue , extend-

    porcnccphalic cyst communicating with a dilated lateral

    (b).

    ing from coronal suture to the supra-orbital

    margin, was tense, congested and bled easily.

    Dural repair with fascia lata and craniopiasty

    with methyl methacryla te was carr ied out . The

    pos topera t ive course was uneventful and the

    chi ld was discharged hom e, free of hea dach e,

    but with res idual mild r ight-s ided weakness .

    Fol low-up a t 2 years showed that the im

    planted plastic was loose and bulging over a

    tense cystic swelling. Reoperation was refused.

    Case J . The patient was originally admitted to

    the neurosurgical unit at the age of 9 months

    following a road traffic accident. On ad

    miss ion, she was drowsy, but without any ab

    normal neurological f indings . A radiograph of

    the skull show ed an obl iquely place d fissu re

    fractur e of the skull crossin g the m idli ne just

    above torcula and CT showed this fracture

    with min ima l cont usi on of the occipital lobes

    (Fig. 8). The baby recovered rapidly from the

    head injury and was discharged home after 5

    days. Six months later (at the age of 15months) she was re-admitted with a tense

    swelling in the region o f the occi pital fractur e

    site and a susp icio n of progressi ve visual loss.

    Th e occipital swelling ha d been gradual ly en

    larging during the weeks prior to re-admiss ion.

    Examinat ion now revealed that a l though the

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    Growing skull fractures 675

    FlG. 6. Case 4: skull radiograph, AP view, showing a

    gaping linear defect (arrows) in the fronto-parictal

    region.

    FI G. 7. Case 4: CT scan sho win g the left frontal growing

    fracture (arrows) and the underlying low density brainlesion.

    child could see, the vision was clearly defective

    as shown by her inability to fixate and lack of

    reac tion to visual stimuli. Re act ion of the

    pupils to the light was very sluggish but equal.

    Visual evoked potentials (VEP) confirmed

    cortical blindn ess. Plain radio gra phy of the

    skull on this second admission showed a large,

    occipital bony defect with scalloped margins

    (Fig. 9). CT at this stage showed resorption of

    a port ion of the occipit al bo ne an d und erl ying

    brain lesions (Fig. 10). At surgery, the CSF-

    containing occipital cyst was aspirated and an

    acrylic cranio plasty carried out. This child was

    re-admitted 2 months later, this time with a

    collection of fluid under the acrylic cranio-

    plasty. A further operation was carried out. A

    wate r-t ight repair of the du ral defect was

    achieved using a pericranial graft and a fresh

    cranioplas ty was performed. The pos topera

    t ive course w as uneventful a nd the wo un d

    healed satisfactorily. Review 3 years later

    showed defective vision.

    Case 6. This child was hospitalized for 3 days

    for hea d injury at the age of 5 m on th s. Sk ull

    radiography and CT showed a left parieto-oc-

    cipital fissured fr acture of skull with mi nim al

    underlying contus ion (Fig. 11). Nine months

    later she presented with a large skull defect

    an d tense scalp sw ellin g at the site of the

    fractu re, alon g wit h increasingl y frequ ent

    seizures and delayed speech. Radiography now

    showed a large, gaping skull defect at the siteof original fractur e (Fig. 12) and CT showe d

    the gap ing fracture with hyp ode nsi ty of the

    under lying bra in an d di la ta t ion of the ips i la t -

    eral ventricle (Fig. 13). Sur gery was refused in

    spite of progr essive neu rolog ical and radiolo gi

    cal deterioration.

    Case 7. A 39-y ear-o ld wom an was seen for

    increasing right-sided head pain. At the age of

    18 years she had suffered a head injury in a car

    accident. On examination, a large gaping skull

    defect was present in the right parietal region.Skull radiography showed a widely gaping

    right pariet al defect (Fig. 14), CT sho wed the

    skul l defect and underlying leptomeningeal

    cys t (Fig. 15). Durocran iopl as ty was s t ra ight

    forward, a nd resul ted in resolut ion of hea d

    ac he and clo sur e of th e skull defec t.

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    6 7 6 Naim-Ur-Rahman et al.

    FlG. 8. Case 5: CT scan at the tim e of initial trauma

    showing right occipital linear fracture.

    FIG,9 , Case 5: skull radiography 6 mon ths later sho win g

    large occipital bony defect with scalloped margins.

    Discussion

    Growing skull fracture has been described in

    the literature as an entity synonymous to a

    leptomeningeal cys t ,2

    ' ' ' '9

    T hus , a pos t t rauma t icextra axial leptomeningeal cyst was assumed

    to be responsible for the progressive enlarge

    me nt of the skull fracture. T h e cases descri bed

    here suggest that the leptomeningeal cyst is

    not a consistent feature and that a variety of

    progressively evolving morphological and

    pathological changes in the underlying bra in

    may play an important role in the genesis of

    growing frac tures . Morphological ly , the pre

    dominant factor responsible for fracture

    growth may lie in the subarachnoid space (a

    leptomeningeal cys t) , cerebrum (hernia ted

    brain) or ventricle (dilated underlying ven

    tricle with porencephalic cyst). These events

    constitute the morphological basis for the frac

    ture types I, II and III, respectively.

    At the initial traum a, disto rtio n of the mal

    leable infant skull on impact generates press

    ure fields within the cranium, leading to a

    skull fractu re and tea ring of the tightly adh er

    en t unde r ly ing dura .1 0 , 1 1

    Six of the seven pa

    tients in our scries and most in other series3

    were child ren. T hu s, malleabi lity of the infant

    skull and tighter adhe ren ce of dura to t he bone

    in chi ldren may account for the common

    occ urr enc e of grow ing fractures in this age

    g r o u p ,1 2

    Pathog enes is of the lepto mening eal cys ts , as

    seen in our cases 1, 5 and 7, is well described

    e l s e w h e r e .2 , 9

    In type II fractures, as seen in our

    cases 2, 4 and 6, cerebral pulsations erode the

    fracture edges, and drive the cerebrum

    through the dura l vent and bony gap. With

    t ime, the hernia t ing and the underlying bra in

    suffers progressive damage as shown by local

    atrophy and cephalomalacia. In type III frac

    tur e (cases 3 an d 5), the und erl ying ventricle

    expands into a porencephal ic cys t . The ent i re

    ventricle may dilate as was seen in case 3 or

    jus t the o n e h o r n subjac ent to the g rowing

    fracture may dilate as occurred in case 5. In

    these cases, the porencephalic cyst extends

    from ventricular wall through the skull defect

    and into the subgaleal space, and is roofed by

    a th in layer of soft ened gliotic cor tex .

    In mos t of the r epor ted cases t he initial he ad

    injury was a minor one and focal neurological

    deficits were rare.1 1

    Th us , at the tim e of initial

    skull fracture, only two of our patien ts sh owe dminimal hemipares is . However, over the ensu

    ing weeks or months, all of these children

    sho wed e viden ce of mo re or less progr essive

    neurological deficits . Neurological status and

    fracture st abilized, after a per iod o f pr o

    gression and growth, in two children. This

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    Growing skull fractures 611

    FlG. 10. Case 5: six months later. CT scan showing

    marked wid eni ng of the occipital fracture with scall oping

    of the margins and right occipita l porenc eph aly and a left

    occipital leptomeningeal cyst.

    resolution was quite dramatic in case 1, and

    was thought to be due to rupture and drainage

    of the leptomen ingeal cys t into the peri mesen -

    cephalic cistern (Fig. 2),

    The natura l his tory and temporal course of

    the growing skull fractures varied from case to

    case . The rapid progress ion with ra ised ICP

    seen in case 3, and spontaneous arrest and

    healing seen in case 1, appear to be the two

    ends of a spect rum. Gradual ly progress ive

    neurological defic i ts with increas ing underly

    ing bra in damage was the more common oc

    curr ence a nd was seen in four of ou r pati ent s.

    In active growing skull fractures, a tense

    overlying scalp bulge may indicate raised ICPwhich may be caused by a tense lep-

    tomeni ngeal / porcnc cphal i c cys t , or pos t t rau

    m a t i c h y d r o c e p h a l u s .1 3

    Slack scalp depression

    at the fracture site wit h evidence o f bo ne re-

    growth and fracture healing, along with the

    arrest of the previousl y progr essive neur olo gi

    cal deficits indicates spontaneous resolution of

    a leptomeningeal cyst, as was seen in case 1.

    Gradually progressive focal neurological

    deficits , seen in four children, coincided

    closely with th e incr easing se para tion of the

    bone edges at the fracture site and CT evi

    dence of extens ion of the underlying bra in

    da ma ge . Th e nat ure of thes e focal deficits

    depended on the frac ture s i te . Thus , the two

    children with occipital fractures (cases 3 and

    5) suffered progressive visual loss, while he mi-

    paresis was seen in the cases with fronto

    parietal fractures.

    Skull radiography and CT were the two

    most useful investigations for the diagnosis

    an d mon ito rin g of grow ing skull fractures.

    Th us , at the time of the initial tr aum a, all the

    seven patients had skull radiographs which

    show ed linear fracture s. Th e initial hea d injury

    was not cons idered serious enough to warrant

    CT in the first instance, except in cases 5 and

    6 whe re CT at the time of tr au ma conf irme d

    the fissured skull fractures without evidence of

    s ignif icant contus ions or damage to the under

    lying bra in (Figs. 8 an d 11). Del aye d CT

    sho wed the fracture grow th (Figs. 10 an d 13)

    and, mor e import ant ly , appea rance of exten

    sive fresh lesions with marked and possibly

    cont inuing damage to the underlying bra in

    (Figs. 1 0 an d 13). CT correc tly dem on str at ed

    the nat ure and ex tent of the intracrania l path

    ology includi ng areas of cephalomalacia , sub

    dural , leptomeningeal and porencephal ic

    cysts, as well as ventricular dilatation (com

    plete or only one horn).

    Becau se of the variab le pathologi cal

    f indings , the s tandard surgical procedure de

    scribed in the l i te ra ture2

    '1 1 , 1 4

    for the t rea tment

    of the gr owi ng skull fr actures is no t justifiable

    or applicable in all the cases. Standard surgical

    appr oach involves resect ion of the lept o

    meningeal cys t and hernia ted bra in, repair of

    the dural defect with a graft and cranio

    p i a s t y2 , 1 1

    For the s t ra ightforward cases without

    any evidence of raised IC P, s uch as our cases

    2 and 7, this procedure is curative. We have

    used pericranium/fascia la ta and methyl

    methacryla te to repair dura l and crania l de

    fects in our cases; but others have used split

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    678 Naim-Ur-Rahman et al.

    FI G. 1 1 . Case 6: CT scan at the time of initial trauma

    showing left parieto-occipital fracture with minimal

    underlying contusion and normal ventricular system.

    FLG. 1 2. Case 6: skull radiograph, 9 mont hs later

    show ing large, gapi ng skull defect at the site of original

    fracture.

    calvarial bone, ribs, iliac crest and metallicmateria ls with sa t is fac tory resul ts .

    1 5

    "1 8

    H o w

    ever, ch ild ren un de r 2 year s of age have a skull

    bone thickness that is too difficult to split in

    orde r to repair the defect .1 8

    In some pat ients ,

    such as our case 4, the dural defect extends

    well beyond the skull defect, necessitating

    FlG. 13. Case 6: CT sc an 9months posttrauma showing

    gaping fracture with hypoden sity of underlying brain and

    dilatation of the ipsilateral ventricl e.

    FIG . 1 4 . Case 7: skull radiograph showin g gaping skull

    defect, right parietal region.

    rongeuring bone edges for long dis tances inorder to expose reasonable dura l edges to

    which the graft can be stitched. In others, such

    as our case 5, one may be obliged to resect the

    cortex which roofs the hernia t ing poren

    cephalic cyst. This extensive enlargement of

    the skull defect an d resectio n of the possibly

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    Growing skull fractures 679

    FlG. 15. Case 7: CT scan sho win g right parietal skull

    defect overlying a leptomeningeal cyst.

    functiona l a nd viable a reas of the infant brain

    appear to be somewhat excessive. In these

    ci rcumstances, the modif ied method for re

    pairing this defect, as described by Halliday et

    al.,"* seems more appropriate. This technique

    involves mobi l izing the per icran ium ci rcum-

    fercntially ar ou nd the edges of the bone defect

    an d reflecti ng it over the site of the du ra l

    defect to provide t issue for repair, 1

    In growing skull fractures associated with

    raised ICP, shunt surgery should be con

    sidered as an init ial or alternative procedure,

    as i t ma y resu lt in res olut ion of rais ed IC P,

    dis appea ran ce of scal p swellings and re growth

    of bon e edges at the fracture si te ; 1 3 as i l lus

    trated by case 3.

    Recurrence after surgical repair in two of

    our pat ien ts and o ther repor ted cas es 1 3 co r r e

    lated with failure to secure watertight dural

    clos ure or failure to addr ess the raised IC P.

    Bec au se of the kno wn risk of frac tur e

    growth along with the frequently progressive

    natu re of brain dam age an d neuro logical

    deficits, children with linear skull fractures

    should be examined clinically 2-3 months

    later to chec k for eviden ce of growing fractur e.

    If a scal p bul ge or gapi ng bone defe ct is foun d,

    radiography and CT should be repeated. If

    growing skull fracture is confirmed, surgical

    repair should be advised.

    Address for correspondence: P ro fesso r Naim -Ur-

    Rahm an , Division of Neur os ur ger y, Colle ge of

    Medicine, King Saud University & King

    Khalid University Hospital, PO Box 2925,

    Riyadh 11461, Sau di Ara bia.

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