Whats Wrong With My Babys Head?

Cathy Cartwright, RN, MSN, PCNS Pediatric Clinical Nurse Specialist Neurosurgery c2cartwright@gmail.com Plagiocephaly asymmetry and twisted condition of the head ree! "plagios o#li$ue % kephal head& 'istory of (eforming the 'ead )rtificial deformation of the neonatal s!ull ) way to differentiate from others "li!e tattoos, #ody piercing& *nown since +,,, -C in ancient Phoenicia Mechanisms Compressing front and #ac! of head with #oard and pads Cradle.#oard /ightly wrapping the head with a #inding Most craniosynostosis is recogni0a#le at #irth Some molding of the s!ull during #irth process, #ut usually normali0es #y 1 wee!s of age Craniosynostosis premature closure of one or more cranial sutures Nonsyndromic most common 23er 4, syndromes Partial closure of one suture can cause a deformity 5mportant to recogni0e craniosynostosis or positional molding early so appropriate inter3ention can ta!e place Causes enetic conditions "mutations in fi#ro#last growth factor receptors& Meta#olic disorders . hyperthyroidism 'ematologic disorders 23ershunting hydrocephalus "secondary craniosynostosis& 5n utero head restraint multiple #irths, maternal smo!ing, 3alproate

6tiology -rain is contained in neurocranium which is comprised of s!ull #ase and cranial 3ault Sutures allow infants head to reshape during and after #irth process and to accommodate rapidly e7panding #rain Remo3ing s!ull in neonate with intact dura results in the dura regenerating the s!ull with suture placed as dictated #y the dura -one growth occurs from the e7panding #rain S!ull is 189 adult si0e at #irth and 4,9 adult si0e #y : yrs Nonsyndromic Craniosynostosis ; out of 2;,, children Multiple suture synostoses in3ol3ing 2 or more cranial sutures occur in +.<9 of nonsyndromic craniosynostosis Simple craniosynostosis is usually random in occurrence #ut 2.=9 of isolated sagittal synostosis and <.;+9 of coronal synostosis were found to #e familial 5n utero head restraint also thought to #e a cause #ut is most commonly seen as positional plagiocephaly Sagittal Synostosis most common Called scaphocephaly "#oatli!e shape to s!ull& +,.=,9 of all craniosynostosis -itemporal narrowing, frontal #ossing, occipital cupping, palpa#le sagittal ridge

C/ . Closed sagittal suture with ridge

>ateral s!ull 7.ray

Coronal Synostosis )nterior plagiocephaly 2,.1,9 of all craniosynostosis ?ertical dystopia, nasional de3iation, flattening of frontal #one on affected side Stra#ismus from ipsilateral superior o#li$ue paresis and compensatory contralateral head tilt present in 8,.=89 of unilateral coronal synostosis See ophthalmologist familiar with craniosynostosis )P s!ull film shows harle$uin appearance to or#it as superior or#ital rim is elongated

C/ . closed > coronal suture

)P s!ull 7.ray . 'arle$uin sign ">&

Metopic Synostosis /rigonocephaly 5n3ol3es metopic suture ;,9 of all craniosynostosis /riangular shape -itemporal narrowing Parietal #ossing 'ypotelorism Metopic ridge Can ha3e normal s!ull shape and @ust a ridge "fussyA&

>am#doid Synostosis 2ccipital "or posterior& plagiocephaly 5n3ol3es lam#doid suture ;.29 of all craniosynostosis /rape0oid shape to s!ull when 3iewed from a#o3e /ilted s!ull #ase "affected side displaced inferiorly )ffected ear displaced inferiorly and posteriorly Palpa#le ridge along suture line 2ccipitomastoid #ulge /owneBs ?iew s!ull 7.ray

Classifications of Craniosynostosis /ype of Craniosynostosis Scaphocephaly "dolicocephaly& Suture 5n3ol3ed Sagittal 5ncidence +,.=,9 Characteristics -itemporal narrowing Crontal #ossing 2ccipital cupping Palpa#le sagittal ridge ?ertical dystopia Nasional de3iation Clattening of frontal #one on /riangular shape -itemporal Parietal #ossing 'ypotelorism Metopic ridge Posterior plagiocephaly >am#doid ;.29 /rape0oid shape /ilted s!ull #ase 2ccipitomastoid #ulge

)nterior plagiocephaly

Coronal

2,.1,9

affected side /rigonocephaly narrowing Metopic ;,9

5mportant to differentiate #etween lam#doid synostosis "treatment is surgery& and positional plagiocephaly "usually no surgery&.

Lambdoid Synostosis Dsually present at #irth /rape0oid shape when 3iewed from a#o3e 5psilateral ear displaced posteriorly and inferiorly -ony ridge palpa#le o3er closed lam#doid suture Dnilateral occipito.parietal flattening posteriorly Ehen 3iewed posteriorly there is an ipsilateral occipitomastoid #ulge and the s!ull #ase appears tilted Radiographic e3idence of closed suture "/owneBs 3iew, C/ with #one windows, C/ with 1( recon&

Positional Plagiocephaly Dsually not present at #irth Parallelogram shape when 3iewed from a#o3e 5psilateral ear displaced anteriorly No #ony ridge o3er lam#doid suture Dsually unilateral occipito.parietal flattening #ut can #e #ilateral Ehen 3iewed posteriorly the s!ull #ase is hori0ontal and no occipito. mastoid #ulge Radiographic e3idence of open sutures May ha3e torticollis

Positional Plagiocephaly (eformational forces such as the #irth process, can shape the s!ull 5nfant #rain grows rapidly during first se3eral months after #irth /his growth e7pands the s!ull into its normal shape '.C. is 18 cm at #irth and increases 4 cm #y = mo and ;2 total #y ; year 2 F cm from age ; .2 yrs and G cm from age 2.1 yrs (eformational forces can ha3e a significant impact during the period of rapid s!ull growth "infant seat, car seat, mattress, swing, stroller& 2 mo :,, hours sleeping . Cre$uently noticed #y pediatrician at 2 mo e7am "normal at #irth& 'ead must #e rotated to redistri#ute the forces of gra3ity Curther aggre3ated #y torticollis . tightening of sternocleidomastoid or cer3ical muscles that pre3ent the infant from turning his head ;<, degrees

 Significant increase in positional plagiocephaly since ;442 when the )merican )cademy of Pediatrics initiated the #ac! to sleep campaign and recommended that infants sleep on their #ac!s or sides to decrease the incidence of S5(S Parents relate that #a#y preferred to sleep on #ac! with head turned to one side Can ha3e flattening of one side or the entire occipital #one 'ead shaped li!e parallelogram

/reatment for Positional Plagiocephaly Can #e pre3ented reposition the infantBs head when lying supine, starting from #irth . doesnBt cause #rain damage /oys or o#@ects of interest placed on nonpreferential side )lternate arms when feeding Put si#ling on nonpreferred side /ummy time when awa!e Cranial orthotic de3ice to correct moderate to se3ere cases if parents ha3e tried to reposition without success Most effecti3e #etween + ;2 months Refer to orthotist e7perienced in cranial orthotic de3ices for positional plagiocephaly Reim#ursement issues "careful with documentation indicate that parents tried repositioning without success if true& Correct torticollis if present Static stretching e7ercises Confirm no C.spine defect first

 Slowly turn head 4, degrees toward non.preferential side, hold it for ;, sec someone may need to hold shoulders Correct head tilt (o 8.= times per day or with e3ery diaper change Sternocleidomastoid tumor of infancy Syndromic Craniosynostosis Present with characteristic group of findings Multiple cranial suture synostoses including sutures of the cranial #ase, resulting in comple7 face and s!ull deformities Cre$uently associated with medical pro#lems including hydrocephalus, papilledema, respiratory distress and failure to thri3e Most common are Crou0on, )pert, Pfeiffer Crou0on Syndrome Cirst descri#ed #y Crench neurologist in ;4;2 )utosomal dominant 5ncidence ;H28,,,, #irths Caused #y multiple mutations in fi#ro#last growth factor receptor 2 "C CR2& -icoronal synostosis "short cranium, #road, flat forehead, may ha3e sagittal or lam#doid synostosis or e3en a clo3erleaf deformity *lee#lattschadel& ?arious degrees of e7or#itism "e7ophthalmos, proptosis&, hypertelorism and ma7illaryHmidface hypoplasia 'igh ris! for serious ocular a#normalities "papilledema, optic atrophy, corneal e7posure and proptosis& /arsorraphy Conducti3e hearing loss Serious airway compromise, challenges with oral feeding "trach, /& )t ris! for de3eloping hydrocephalus andHor Chiari )pert Syndrome )crocephalosyndactaly /ype 5 "named after Crench neurologist who descri#ed the syndrome in ;4,=& Most comple7 of craniofacial syndromes )utosomal dominant

5ncidence ;H8,,,,, to ;H;=,,,,, Mutation of C CR2 gene Multiple suture synostosis S!ulls are turricephalic "tower.li!e& Clat and elongated forehead, #itemporal widening and #ilateral flattening of the occiput -ea!ed nose May ha3e hydrocephalus and agenesis of the corpus callosum Syndactaly "fusion of digits in hands and feet& (ental a#normalities "cleft palates&, conducti3e hearing loss, cardiac anomalies and chronic acne Mental retardation and learning disa#ilities are higher in this group than in Crou0on, although many can ha3e normal intelligence

Pfeiffer Syndrome )utosomal dominant 5ncidence of appro7. ; in 2,,,,,, Caused #y mutations in C CR; or C CR2 Multiple suture synostosis, 3arying degrees of mental retardation, midface hypoplasia and upper airway anomalies Proptosis -road thum#s and great toes 5mportant for these children to #e e3aluated #y an e7perienced craniofacial team Craniofacial /eam Craniofacial Surgeon Neurosurgeon 2rthodontist eneticist Speech Pathologist Social Eor!er )udiologist Treatment for Craniosynostosis )d3anced Practice Nurse Pediatrician Psychologist 2tolaryngologist 2phthalmologist Prosthodontist Pediatric (entist

Surgical /he )merican Medical )ssociation defines cosmetic surgery as Isurgery performed to reshape normal structures of the #ody in order to impro3e the patientBs appearance and self.esteem. Reconstructi3e surgery is performed on a#normal structures of the #ody, caused #y congenital defects, de3elopmental a#normalities, trauma, infection, tumors or disease. 5t is generally performed to impro3e function, #ut may also #e done to appro7imate a normal appearanceJ )lso done for cosmetic and psychological #enefits CanBt wear #all caps, helmets, hard to pull shirts on o3er head ?ertical dystopia Possi#le increased 5CP can lower 5K ;<<< >.C. >ane performed first craniectomy to remo3e a stenosed suture on a 4 mo old infant 'igh mortality rates, anesthesia high ris!, a#andoned until ;42: to pre3ent #lindness Dsually not #efore 1 mo of age "1.;2 mo is usually time to operate& Many techni$uesL Cronto.or#ital metopic, unicoronal, #icoronal, syndromic "usually staged procedures& with cal3arial 3ault remodeling Pi e7tended strip craniectomy 'ung span correct se3ere sagittal synostosis Strip craniectomy treats sagittal synostosis, #est #efore = mo of age 6ndoscopic strip craniectomy all sutures, #est results #efore + months of age 5mportant to identify craniosynostosis early so pt. has more options and may undergo less in3asi3e treatments 6ndoscopic strip craniectomy (e3eloped #y Mimene0 and -arone in ;44= Cal3arial 3ault remodeling #lood losses from 289 . 8,,9 2=: patients o3er < years (ecrease in #lood lossHtransfusions 23ernight stay Molding helmet for appro7imately ; year

Postoperati3e Nursing Care fre$uent 3.s. with neuro assessments watch for early signs of #lood loss, electrolyte im#alance, neurologic deterioration or CSC lea! swelling, airway pro#lems pain control parent participation discharge and follow up care -a#ies with sagittal synostosis should lie with the #ac! of the head on the mattress to decrease the )P length >ong /erm 2utcome 2<= patients from ;44=.2,,+ Kuestionnaires mailed to ;+; !nown addresses )s!ed parents to e3aluate cosmetic, psychosocial, neurocogniti3e and o3erall satisfaction Mean follow up =.; years "range 1.;; years& ;,,9 would recommend procedure to familyHfriends and do it again 429 e7cellent school performance N89 had re3ision of any !ind ;,9 teased sometimes No child reported #eing teased often References
)merican Medical )ssociation Policy of 'ouse of (elegates, Mune ;4<4, p.).<4. (efinitions of IcosmeticJ and Ireconstructi3eJ surgery, '.+18.442. Council of Medical Ser3ices )nnual Meeting. Cartwright, C.C., Mimene0, (.C., -arone, C.M., O -a!er, >. "2,,1&. 6ndoscopic strip craniectomyL a minimally in3asi3e treatment for early correction of craniosynostosis. Journal of Neuroscience Nursing, "18&1,;1,.;1<. Cartwright, C.C. Craniosynostosis and Positional Plagiocephaly. 5n Cartwright, C.C. O Eallace, (.E. "2,,:&. Nursing Care of the Pediatric Neurosurgery Patient. SpringerL 'eidel#erg.

Mimene0, (.C. O -arone, C.M. "2,;,&. Multiple.suture nonsyndromic craniosynostosis L early and effecti3e management using endoscopic techni$ues. J Neurosurg Pediatrics, 8L221.21;. *omotar, R.M., Pacharia, -.6., 6llis, M.)., Celdstein, N.)., and )nderson, R.C. "2,,=&. Pitfalls for the pediatricianL positional molding or craniosynostosisA Pediatric Annals, 18"8&, 1=8.1:8. *oren )., Reece, S.M., *ahn.(Bangelo, >. and Medeiros, (. "2,;,&. Parental information and #eha3iors and pro3ider practices related to tummy time and #ac! to sleep. J Pediatric Health Care, 2+, 222.21,. Moon RQ, (arnall, R), oodstein M', 'auc!, CR. )merican )cademy of Pediatrics, /as! Corce on Sudden 5nfant (eath Syndrome "2,;;&. /echnical Report S5(S and other sleep.related infant deathsL e7pansion of recommendations for a safe infant sleeping en3ironment. Pediatrics ;2<"8&, ;,1,.;,14. (25L ;,.;8+2Hpeds.2,;;.22<+ Ridgeway, 6.-., -erry.Candelario, M., rondin R./., Rogers, .C., and Proctor, M.R. "2,;;&. /he management of sagittal synostosis using endoscopic suturectomy and postoperati3e helmet therapy. J Neurosurg Pediatrics, :L=2,.=2=. Ri3ero. ar3ia, M., Mar$ue0.Ri3as, M., Rueda./orres, ).-. O 2llero.2rti0, ). "2,;;&. 6arly endoscopy.assisted treatment of multiple.suture craniosynostosis. Childs Nerv Syst, 2<"1&L+2:.1;. Shah, M.N., *ane, ).)., Petersen, M.(., Eoo, ).S., Nadoo, S. (. and Smyth, M.(. "2,;;&. 6ndoscopically assisted 3ersus open repair of sagittal craniosynostosisL the St. >ouis ChildrenBs 'ospital e7perience. J of Neurosurg Pediatrics, <L;=8.;:,. /as! Corce on Sudden 5nfant (eath Syndrome "2,;;&. S5(S and other sleep.related infant deathsL e7pansion of recommendations for a safe infant sleeping en3ironment. Pediatrics, ;2<"8&L ;,1,.;,14. doiL;,.;8+2Hpeds.2,;;.22<+.