G G‹‹RR‹‹fifi
Pierre Robin Sendromu (PRS) 1923 y›--l›nda Pierre Robin taraf›ndan tan›mlan--m›flt›r (1). Prenatal hayatta çeflitli neden--lerle amnion s›v›s›n›n azalmas› ve bu azalmaya ba¤l› olarak, fötüsün kafas›n› gö¤süne s›k›ca yaslamas› sonucu alt çene gelifliminin engellendi¤i ve bu sendromun ortaya ç›kt›¤› belirtilmektedir. Geliflimi engellenen alt çene nedeniyle sa¤ ve sol damak raflar› aras›nda konumlanan dil normal translokasyonunu gerçeklefltire--medi¤i için damak raflar›n›n normal bi--çimde kaynaflmas›na engel olur (2).
Anomali 3 temel k›sm›ndan oluflmakta--d›r: sekonder damak yar›¤›, mandibular retrognati ve/veya mikrognati, dilin geriye düflmesi (glossopitozis). Sendrom baflka sendrom ve malformasyonlarla birlikte olabilece¤i gibi izole de olabilir (3).
Bu sendromun en önemli özelli¤i, dilin geriye düflmesine ba¤l› olarak yeni
do¤an--IINNTTRROODDUUCCTTIIOONN
Pierre Robin Syndrome (PRS) was des--cribed by Pierre Robin at 1923 (1). It has been reported that amniotic fluid volume may decrease for some reasons during the prenatal life, and this event causes the he--ad to flex tightly against the chest, preven--ting the mandible from growing forward normally. Because the restriction on man--dibular development and displacement forces the developing tongue upward and prevents normal closure of the palatal shelves (2).
The anomaly consists of 3 main com--ponents: secondary cleft palate, mandibu--lar micrognathia and/or retrognathia and glossoptosis. This syndrome may occur as an isolated defect, as a part of a recogni--zed syndrome, or as a part of a complex of multiple congenital anomalies (3).
The major characteristic of this syndro--me is the mortality risk of newborns by asphyxia resulting from oro-pharyngeal
D
Dtt.. SSeemmaa AAKKPPIINNAARR** P
Prrooff..DDrr.. HHüüssaammeettttiinn OOKKTTAAYY**** P
Prrooff..DDrr.. AAbbddüüllvvaahhiitt EERRDDEEMM**
Y
Yaazz››flflmmaa aaddrreessii:: C
Coorrrreessppoonnddiinngg AAuutthhoorr:: Dt. Sema Akp›nar
Atatürk Üniversitesi Difl Hekimli¤i Fakültesi Ortodonti A.D.
Erzurum, Türkiye
Ataturk University, Faculty of Dentistry Department of Orthodontics, Erzurum, Turkey Tel: 0 442 231 13 83
Faks: 0 442 231 22 70
E-posta: drsemaakpinar@gmail.com
*Atatürk Üniv. Diflhek. Fak. Ortodonti A. D. Erzurum, ** Medipol Üniv. Diflhek. Fak. Ortodonti A. D. ‹stanbul / *Atatürk Univ. Faculty of Dentistry Dept. of
Orthodontics, Erzurum, ** Medipol Univ. Faculty of Dentistry Dept. of
Orthodontics, ‹stanbul, Turkey
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ÖÖZZEETT
Bu vaka raporunda PRS’lu bir bebek hastan›n glossopitozisinin konservatif bir yöntemle tedavisi ve hastan›n palatoplas--tiye haz›rlanmas› sunulmaktad›r. (Türk Ortodonti Dergisi 2010;23:284-289)
A
Annaahhttaarr KKeelliimmeelleerr:: Pierre Robin Sendromu, Ortodontik plak, Solunum.
G
Göönnddeerriimm TTaarriihhii:: 29.07.2009 K
Kaabbuull TTaarriihhii:: 17.01.2010
S
SUUMMMMAARRYY
In this case report, glossoptosis treatment of a newborn with PRS and her preparation for palatoplasty with a conservative method is presented. (Turkish J Orthod 2010; 23:284-289)
K
Keeyy WWoorrddss: Pierre Robin Syndrome, Orthodontic plate therapy, Respiration.
D
Daattee SSuubbmmiitttteedd:: 29.07.2009 D
Daattee AAcccceepptteedd:: 17.01.2010
da solunumun engellenmesi ve bebe¤in asfik--siden kaybedilmesidir. Hastalardaki mortalite oran› %30 olarak belirtilmifltir (4). Ayr›ca bes--lenme bozuklu¤u da bir di¤er problemdir (3). PRS’lu hastalara daha hayat›n ilk günlerin--de müdahale edilmeli, beslenmenin ve daha da önemlisi solunumun rahat bir flekilde ger--çeklefltirilmesi sa¤lanmal›d›r. Beslenme için yeterince emme bas›nc› oluflturulamad›¤› ve alt çene çok geride oldu¤u için anne memesi ememeyen bebekler özel biberonlarla bes--lenmektedir. Ayr›ca pasif beslenme plaklar›, nazogastrik sonda ve gastrostomi di¤er yön--temler aras›ndad›r (3,5,6).
Solunumun rahatça sa¤lanmas› yani üst solunum yolundaki obstrüksiyonun ortadan kald›r›lmas› için, hastan›n özel befliklerde yü--zükoyun yat›r›lmas› (prone pozisyonu) veya sürekli pozitif havayolu bas›nc› (Continuous Positive Airway Pressure, CPAP) gibi yöntem--ler uygulanabilmektedir. Ancak hastadaki CO2 seviyesi yüksekse, dilin duda¤a dikilme--si (glossopexy), trakeostomy, endotrakeal en--tübasyon, mandibular distraksiyon gibi cerra--hi yöntemler de önerilebilmektedir (3,5,6).
Bununla birlikte, hayat›n ilk günlerinde uy--gulanacak bu tip cerrahi yöntemler bebek ve aile için oldukça travmatik olarak alg›lanabil--mektedir. Uzun dönem çal›flmalar PRS’lu bi--reylerde retrognatik yüz profilinin düzelmesi ve estetik ve ahenkli bir yüz görünümünün el--de edilebilmesi için gerekli mandibular büyü--me potansiyelinin büyü--mevcut oldu¤unu göster--mifltir (7). Bu yüzden, mandibulan›n uzat›lma--s› amac›yla erken dönemde agresif giriflimler--den kaç›n›lmas› gerekti¤i belirtilmektedir (2).
Pierre Robin Sendrom’lu bireylerin solu--numunun düzenlenmesinin yan› s›ra beslen--meyi de kolaylaflt›ran çeflitli ortodontik apa--reyler mevcuttur. Velar uzant›l› plak (8) ve Tübingen palatal pla¤› (9) gibi ayg›tlar dilin önde konumlanmas›n› sa¤layarak solunum yolunun aç›lmas›n› sa¤larlar. Ayn› zamanda beslenme pla¤› olarak da vazife görürler.
Bu vaka raporunda sunulacak olan PRS’lu bebek oldukça konservatif bir yöntemle glos--sopitozis için tedavi edilmifl ve bebek pala--toplastiye haz›rlanm›flt›r.
O
OLLGGUU SSUUNNUUMMUU
H
Haassttaann››nn HHiikkaayyeessii vvee KKlliinniikk MMuuaayyeenneessii Üniversite klini¤ine do¤du¤u gün getirilen 50 cm. boy ve 3400 gr. a¤›rl›¤a sahip bebek
obstruction. The mortality ratio has been re--ported as 30 percent (4). Furthermore, nutriti--on difficulty is another important problem of these babies (3).
The treatment of PRS patients should be started as soon as possible, and feeding and especially respiration functions should be restored. Because of retrognathic mandible and deficiency of sucking pressure, these ba--bies are not able to suck breast milk. There--fore, they have to be fed with special feeding bottles (3-6). In addition, passive nutrition plates, nasogastric catheter and gastrostomy tubes are among the other nutrition techni--ques (3-6).
To improve the respiration by eliminating airway obstruction, laying the babies in pro--ne position or using continuous positive air--way pressure (CPAP) are the applicable tech--niques. If the CO2 saturation is higher, the surgical techniques such as suturing the ton--gue to the lip (glossopexy), tracheotomy, en--dotracheal intubation or mandibular distrac--tion can be applied (3-6).
These surgical techniques during the first days of life, however, may be perceived as traumatic for the newborns and parents. Long term studies have shown that the babies with PRS have adequate mandibular growth po--tential in order to reduce the retrognathic fa--cial profiles and to provide an aesthetic and harmonic facial appearance (7). Therefore, it has been recommended to avoid from heavy surgical approaches to lengthen the mandib--le during the early life (2).
There are a lot of orthodontic appliances which facilitate both breathing and feeding. Orthodontic appliances such as a palatal pla--te with velar spur or Tubingen plapla--te cause the tongue to locate anteriorly, and eliminate air--way obstruction. These plates also work as nutrition plates.
In this case report, glossoptosis of a new--born with PRS was treated with a conservati--ve method and the patient was prepared to palatoplasty.
C
CAASSEE RREEPPOORRTT
C
Caassee HHiissttoorryy aanndd CClliinniiccaall EExxaammiinnaattiioonn A baby, 3400 g weight and 50 cm length, was brought to our clinic on the day she was born. The baby was the fourth child of the parents and, there was no syndromic finding and familial cleft lip and palate trait. In
clini--hasta, ailenin 4. çocu¤u olarak dünyaya gel--mifl olup soy geçgel--miflinde dudak- damak yar›--¤› veya herhangi bir sendrom hikayesi tespit edilmemifltir. Klinik muayenede yumuflak da--mak ve uvulay› ilgilendiren sekonder dada--mak yar›¤› gözlenmifltir (fiekil 1-3).
fi
fieekkiill 11.. Tedavi
bafllang›c›ndaki cephe görünümü.
F
Fiigguurree 11.. Frontal facial view
before treatment.
fi
fieekkiill 22.. Tedavi
bafllang›c›ndaki profil görünümü.
F
Fiigguurree 22.. Lateral facial view
before treatment.
fi
fieekkiill 33.. Tedavi
bafllang›c›ndaki a€›z içi görünümü. Dilin orofarinkste yer ald›€›na dikkat ediniz.
F
Fiigguurree 33.. Intraoral view
showing the tongue located in oropharinx behind the palatal shelves.
cal examination, a cleft was observed in the posterior region of palate (Figures 1-3).
T
Trreeaattmmeenntt PPrrooggrreessss
At the beginning, a passive nutrition plate was applied to the patient because no respi--ratory problem or severe glossoptosis was ob--served. In the control visit (3 weeks later), res--piratory difficulty resulting from glossoptosis was observed when the baby was laid supine position, and thus it was decided to use the plate designed by Oktay et al. (10)
F
Faabbrriiccaattiioonn ooff tthhee AApppplliiaannccee
An impression was taken from the palate with a silicone based material at hospital conditions. A fine study cast was created with hard plaster, and the cleft area was filled with wax. A wire extension of 18–20 mm was pre--pared from 0.9-mm stainless steel orthodon--tic wire. Retention bends were constructed at the free ends of this wire extension. Acrylic portion of the plate was prepared using typi--cal methods, and the wire extension was pla--ced into the acrylic material. To prevent the wire extension from damaging soft tissues, the end of the extension was covered with an acrylic button. The acrylic plate was polyme--rized under pressure and the laboratory pha--se was completed. The wire extension was adjusted in order to force the tongue to disp--lace anteriorly (Figure 4). The rest adjust--ments were done on the patient.
C
Clliinniiccaall aapppplliiccaattiioonn
After the acrylic covered extension wire came into contact with tongue base, the amount of the bending was increased or dec--reased according to the tongue movement. After all adjustments were done correctly, the tongue placed anteriorly (Figure 5), and the baby could breathe easily and comfortably as cited by Oktay et al (10). Furthermore, the parents were thought how to insert and to re--move the nutrition plate.
The baby was seen at four-week intervals and when necessary the MFP was changed or its wire extension was adjusted. When the pa--tient was 8 months old, it was clinically obser--ved that the tongue located in the oral cavity and the respiratory difficulty improved even if the nutrition plate was not worn. Because of this result, the use of the appliance was dis--continued and the baby was directed to the oral surgeon for palatoplasty (Figure 6-8).
fi
fieekkiill 44aa.. Tel uzant›l› modifiye
beslenme pla€›.
F
Fiigguurree 44aa.. Modified nutrition
plate with the wire extension.
fi
fieekkiill 44bb..
F
Fiigguurree 44bb..
fi
fieekkiill 55.. Modifiye beslenme
pla€›n›n a€›z içindeki görünümü.
F
Fiigguurree 55.. Modified nutrition
plate within the mouth.
fi
fieekkiill 66.. Cerrahi öncesi cephe
görünümü.
F
Fiigguurree 66.. Frontal facial view
before surgical operation.
T
Teeddaavvii SSeeyyrrii
Hasta klini¤e ilk getirildi¤inde belirgin bir glossopitozis ve solunum güçlü¤ü dikkat çek--medi¤i için pasif bir beslenme pla¤› uygulan--m›flt›r. Üç hafta sonra yap›lan kontrolde, glos--sopitozisin belirginleflti¤i ve hasta s›rtüstü yat›--r›ld›¤›nda siyanoz geliflti¤i gözlenmifltir. Bu se--ansta hastan›n solunumunu rahatlatmak için Oktay ve arkadafllar›n›n (10) gelifltirdi¤i Modi--fiye Beslenme Pla¤› (MBP) uygulanm›flt›r.
A
Appaarreeyyiinn HHaazz››rrllaannmmaass››
Hastadan, hastane ortam›nda silikon esasl› ölçü maddesi ile tek aflamada üst çene ölçüsü al›nm›flt›r. Sert alç›dan çal›flma modeli elde edilerek yar›k sahas›nda gerekli mumlamalar yap›lm›fl ve 0,9 mm’lik paslanmaz çelik telden yaklafl›k 18–20 mm uzunlu¤unda bir uzant› haz›rlanm›flt›r. Bu uzant›n›n akrilik gövdeye girecek k›sm›nda retansiyon bükümleri yap›l--m›flt›r. Tüm dama¤› kaplayacak flekilde, orto--akrilden bir beslenme pla¤› yap›m›na bafllan--m›fl ve bu esnada tel uzant› haz›rlanbafllan--m›fl olan akrilik gövde içine yerlefltirilmifltir. Uzant›n›n plak d›fl›nda kalan serbest ucuna, yumuflak dokularda travma oluflturmamas› için akrilik bir yast›k eklenmifltir. Haz›rlanan plak bas›nç--l› tencerede polimerize edilmifl ve laboratuar ifllemleri tamamland›ktan sonra, plak üzerin--deki uzant›ya, dil köküne temas ederek dili a¤›z kavitesinde yer almaya zorlayacak flekil--de bir e¤im verilmifltir (fiekil 4). Geri kalan uyumlama hasta a¤z›nda yap›lm›flt›r.
K
Klliinniikk UUyygguullaammaa
Uç k›s›mda akrilik yast›k olan tel uzant› dil köküne arkadan temas ettirilmifl ve dil konu--munda oluflan etkiye göre e¤imin miktar› azal--t›lm›fl veya art›r›lm›flt›r. Tüm bunlar sa¤land›k--tan sonra, Oktay ve arkadafllar›n›n da (10) belirtti¤i gibi dilin önde konumland›¤› ve has--tan›n rahat bir biçimde solunum yapabildi¤i gözlenmifltir (fiekil 5). Ayr›ca ebeveyn pla¤› kullanma konusunda bilgilendirilmifltir.
Hasta 4 haftal›k aralar ile görülmüfl ve ge--rekli görüldü¤ü durumlarda tel uzant›n›n ye--niden uyumland›r›lmas› yap›lm›fl veya plak yenilenmifltir. Hasta 8 ayl›k oldu¤unda MBP ç›kart›ld›¤› halde dilin geriye gitmedi¤i ve so--lunum s›k›nt›s›n›n ortadan kalkt›¤› klinik ola--rak gözlenmifltir (fiekil 6-8).
Hasta palatoplasti operasyonu için cerraha yönlendirilmifltir.
T
TAARRTTIIfifiMMAA vvee SSOONNUUÇÇ
Smith (11) Pierre Robin Sendromlu çocuk--larda do¤umdan hemen sonra solunum prob--lemi olmayabilece¤ini, ancak bunun havayo--lu obstrüksiyonu olmayaca¤› anlam›na gel--medi¤ini ve 6-8 hafta içerisinde gittikçe a¤›r--laflan bir obstrüksiyon geliflebilece¤ini ifade etmifltir. Wilson ve arkadafllar› (12) incelemifl olduklar› Pierre Robin Sendromlu 10 bebe¤in 7’sinde (%70) havayolu obstrüksiyonunun 24–51 günler aras›nda ortaya ç›kt›¤›n› belirt--mifllerdir. Yazarlar hayat›n ilk günlerinde sa--dece klinik muayene ile yap›lan üst havayolu de¤erlendirmesinin yeterli olmayaca¤›n› ve polisomnografi gibi objektif ölçümlerin yap›l--mas› gerekti¤ini vurgulam›fllard›r. Bu makale--de sunulan olguda da hem yeni do¤an ünite--sinde hem de klini¤imizde herhangi bir solu--num problemi görülmemesi mikrognati ve damak yar›¤› flikayeti ile klini¤imize gelen bebeklerde daha dikkatli olmam›z gerekti¤ini ortaya koymaktad›r.
D
DIISSCCUUSSSSIIOONN aanndd CCOONNCCLLUUSSIIOONN
Smith (11) reported that there may be no respiratory problem at birth in some babies with PRS, but this does not mean that airway obstruction and respiratory problem will not occur during the ongoing days, and that the respiratory problem may worsen within the 8 weeks. Wilson et al. (12) presented a Pierre Robin sequence series of 10 infants, of whom seven (%70) showed upper airway obstructi--on between days 24 and 51. The authors highlighted that clinical examination alone cannot be adequate to reveal the severity of upper airway obstruction and they recom--mended close prospective respiratory moni--toring, including polysomnography. The fact that no upper airway obstruction was noted by both neonatologist and orthodontist in the early days of the presented infant remembers us to pay more attention on the infants with micrognathia and cleft palate.
MFP is a very effective appliance which facilitates both breathing and feeding of the babies having PRS. This treatment approach is quite conservative and economical, and preparation and usage of this appliance are very easy.
fi
fieekkiill 77.. Cerrahi öncesi profil
görünümü.
F
Fiigguurree 77.. Lateral facial view
before surgical operation.
fi
fieekkiill 88.. Cerrahi öncesi a€›z içi
görünümü. Dilin oral kavitede konumland›€›na dikkat ediniz.
F
Fiigguurree 88.. Intraoral view
showing the tongue located in the oral cavity.
K
KAAYYNNAAKKLLAARR//RREEFFEERREENNCCEESS 1. Wagener S, Rayatt SS, Tatman AJ, Gornall P,
Slator R. Management of infants with Pierre Robin sequence. Cleft Palate Craniofac J 2003;40(2):180-5.
2. Proffit WR. Etiologic factors in development of dentofacial deformity. In: Proffit WR, White RP eds. Surgical Orthodontic Treatment. St Louis: Mosby Year book 1991:38-9.
3. Tolarova M, Senders C. Pierre Robin Malformation. 2006 June Avaliable from: URL:http://www.emedicine.com.
4. Jones KL. Smith's Recognizable Patterns of Human Malformation. 5th ed. Philadelphia: WB Saunders; 1970. p. 234-5.
5. Wittenborn W, Panchal J, Marsh JL, Sekar KC, Gurley J. Neonatal distraction surgery for micrognathia reduces obstructive apnea and the need for tracheotomy. J Craniofacial Surg 2004;15(4):623-30.
6. Schaefer RB, Stadler JA, Gosain AK. To distract or not to distract: an algorithm for airway management in isolated Pierre Robin sequence. Cleft Palate-Craniofac J 1998;35(6):517-25. 7. Pruzansky S, Richmond JB, Pierre Robin
Sequence. In: Berkowitz S. Ed. Cleft Lip and
Palate Diagnosis and Management. 2nd ed. Berlin Heidelberg: Springer-Verlag; 2006. p. 222. 8. Buchenau W, Urschitz MS, Sautermeister J, Bacher M, Herberts T, Arand J, Poets CF. A randomized clinical trial of a new orthodontic appliance to improve upper airway obstruction in infants with Pierre Robin sequence. J Pediatr 2007;151(2):145-9.
9. Ludwig B, Glasl B, Sader R, Schopf P. conservative orthodontic primary care of four newborns with the Pierre-Robin sequence triad: case report. J Orofac Orthop 2007;68:56-61. 10. Oktay H, Baydafl B, Ersöz M. Using a modified
nutrition plate for early intervention in a newborn infant with Pierre Robin sequence: case report. Cleft Palate Craniofac Journal 2006;43(3): 370-3.
11. Smith JD. Treatment of airway obstruction in Pierre Robin Syndrome. Arch Otolaryngol 1981;107:419-21.
12. Wilson AC, Moore DJ, Moore MH, Martin AJ, Staugas RE, Kennedy JD. Late presentation of upper airway obstruction in Pierre Robin sequence. Arch Dis Child 2000;83(5):435-8. Modifiye Beslenme Pla¤› (MBP) PRS’lu
has--talarda hem beslenmeye yard›mc› olan hem de dil konumunu düzelterek hastan›n rahat solunum yapmas›n› sa¤layan bir apareydir. Bu uygulama hasta için oldukça konservatif olup laboratuar ve klinik uygulamas› basit, ekono--mik ve iyi sonuç veren bir yöntemdir.
