A rare congenital anomaly of maxillomandibular fusion with pectus excavatum and craniosynostosis in a neonate is presented. The child was kept alive by nasogastric tube feeding.Amodification of classification of syngnathia is proposed. The aetiopathogenesis and difficulty in management in our environment are discussed. Keywords: Bilateral; alveolar fusion; craniosynostosis; pectus excavatum  Nigerian Journal of Clinical Practice Vol. 11 (1) 2008: pp.78-8

Adebayo, ET

Adeosun, OO

Ajike, SO

Anyiam, JO

Chom, ND

Jalo, I

English

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Nigerian Journal of Clinical PracticeMarch 2008 Vol. 11 (1): 78-81CONGENITAL BILATERAL FUSION OF THE MAXILLOMANDIBULARALVEOLAR PROCESSES WITH CRANIOSYNOSTOSIS: REPORT OF A RARECASE.A rare congenital anomaly of maxillomandibular fusion with pectus excavatum and craniosynostosis in aneonate is presented. The child was kept alive by nasogastric tube feeding. A modification of classification ofsyngnathia is proposed. The aetiopathogenesis and difficulty in management in our environment arediscussed.Keywords: Bilateral, alveolar fusion, craniosynostosis, pectus excavatum*SO, Ajike, *OO Adeosun, , **ET Adebayo, ***JO Anyiam, , ****I Jalo, *****ND ChomABSTRACTDepartments of *Dental Surgery, **Maxillofacial Surgeon, ***Paediatric ****. Paediatric, Radiology*Federal Medical Center, Nguru, , **44 Army Reference Hospital, Kaduna, , ***Ahmadu Bello UniversityTeaching Hospital, Kaduna, ****Federal Medical Center, Nguru, . *****Ahmadu Bello UniversityTeaching Hospital, Kaduna, Nigeria.(Accepted 14 March 2007)INTRODUCTIONCASE REPORTThe temporo-mandibular joint is a unique andcomplex joint. Abnormal immobility of the jointcould be due to causes within the joint (trueankylosis) or outside the joint (false ankylosis). Veryfew of this disorder are of congenital origin.Congenital fusion of the jaws may be due to softtissue (synechiae) or bone (syngnathia) withmajority due to bony fusion. Occurrence of ankylosisin neonates and children produces severemicrognathia if bilateral and a deviation of themandible to the affected side when unilateral .In childhood ankylosis, treatment should beinstituted as early as possible to restore functionalmobility and to stimulate growth of the condyle, thuscorrecting or minimizing future facial disfigurement.The aim of this paper is to present a case ofcongenital fusion of the gingival pads withassociated craniosynostosis and pectus excavatumand to discuss the problems of management in a ruralresource-poor center.A two-day-old female neonate was referred to theDepartment of Dental Surgery, Federal MedicalCentre, Nguru, Nigeria, with a history of inability toopen the mouth since birth. The baby was the fourthchild of a 25-year-old mother and was delivered atthe Maternity wing of the hospital by spontaneousvertex delivery after a full-term of uneventfulpregnancy There was no history suggestive of birth12,3Correspondence: Dr SO AjikeE mail: mfujiks@yahoo.comtrauma, asphyxia, convulsion or spasms. However,there was a maternal history of ingestion of traditionalherbal medications during the ante-natal period.Similar presenting symptoms did not occur amongother siblings. Clinical examination revealed a fullterm, ill and hypotonic baby weighing 2.2 kg atpresentation.There was mandibular retrognathia and micrognathiawith fusion of the gingival pads (mandible to maxilla)anteriorly. The mouth opening was limited to only aslit of about 2mm,Figure 1:The anterior fontanelle was absent and a posteriorfontanelle was noted measuring 0.5 x 0.5 cm theoccipito-frontal circumference was 32 cm. Pectusexcavatum was evidentPhotograph showingmaxilliomandibular fusion with a narrow slit(about 2mm) anteriorly7879Figure.2 :Figure. 3:The palate could not be examined due to limitedmouth opening. There were, however, noabnormalities noted in the cardiovascular, urogenitaland musculoskeletal systems. Radiographicexamination revealed absence of the sutures of thecranial vault and fontanelles, an ill-defined sellaturcica with intracranial calcifications. The mandiblewas irregular and small, with fusion of the alveolarprocesses of the mandible and the maxilla anteriorlyFigure.4Photograph showing severemicrognathic and retrognathic mandiblephotograph showing pectus excavatum: True lateral view of the left mandibleshowing irregular and small mandible withmaxillomandibular fusion.Figure. 5Based on the history, clinical and radiologicalfindings, a diagnosis of bilateral fusion of themaxillo-mandibular processes associated withcraniosynostosis and pectus excavatum was made.Patient was fed via nasogastric tube while preparingpatient for surgery. Unfortunately, the patient diedone week after birth. Post mortem examination wasdisallowed by parents for religious reasons.Congenital ankylosis of the jaws is rare. Accordingto Laster et al it may be due to soft tissue (synechiae)or bone (syngnathia). Occurrence in childhoodleaves a negative psychosocial effect because ofinability to enjoy feeding and poor facial aesthetic.When it occurs bilaterally, there is impaired growthand function of the jaws . In a review of theliterature, Laster at al reviewed 24 cases ofcongenital ankylosis to which they added anothercase. Extensive search of the Internet and the Englishliterature revealed four additional case reports whichwere not included in the Laster et al's report. Of these, one was Nigerian . Analysis of these 29 casesrevealed that 20 involved maxillo-mandibular fusionwhile 9 were of zygomatico-mandibular fusion. Ourcase reported here had maxillo-mandibular bonyfusion, a case of syngnathia thus adding to theprevious case report from Nigeria.Syngnathia has been classified by some authors.Dawson et al. classified syngnathia into 2 maintypes: Type 1: Simple syngnathia with no othercongenital anomalies in the head and neck. Type 2:Complex syngnathia with subgroups: 2a: complexsyngnathia with aglossia and subgroup 2b complexsyngnathia co-existent with agenesis or hypoplasia ofthe proximal mandible.In 2001, Laster et al. proposed another classificationof bony syngnathia:Type 1a: Simple anterior syngnathia characterized: True lateral view of the right mandibleshowing irregular and small mandible withmaxillomandibular fusion.DISCUSSION45,6443,6,7,8 794Nigerian Journal of Clinical Practice March. 2008 Vol.11(1) Congenital Bilateral Fusion Ajike et alby bony fusion of the alveolar ridges only andwithout other congenital deformity on the head andneck. Type 1b: Complex anterior syngnathiacharacterized by bony fusion of the alveolar ridgesonly and associated with other congenital deformityin the head and neck. Type 2a: Simple zygomaticmandibular syngnathia characterized by bony fusionof the mandible to the zygomatic complex causingonly mandibular micrognathia. Type 2b: Complexzygomatic mandibular syngnathia characterized byfusion of the mandible to the zygomatic complex,and associated with clefts or temporomandibularjoint ankylosis.The case presented here is similar to but differentfrom type 1b of Laster et al's classification. Our casehas a bony fusion of the alveolar ridges withcraniosynostosis and pectus excavatum (Fig 3). Wetherefore propose a modification of Laster et al'sclassification of syngnathia by adding a type 3,which will take care of the pectus excavatumobserved in this patient.Type 3: Complex anterior syngnathia characterizedby bony fusion of the alveolar ridges only, associatedcongenital deformity of the head and neck andassociated extracranial skeletal deformity.The aetiopathogenesis and timing of occurrence ofcongenital jaw ankylosis remains a subject ofcontroversy. Teratogenic agent or trauma ,anomalous fusion , depressed fetal swallowing ,hypervitaminosis A , abnormality of the stapedialartery in the fetus , early loss of neural crest cellshave been speculated. In this patient we suspect thatthe aetiopathogenesis is probably related to theingestion of herbal concoctions during pregnancy. Itis possible for the herbs to have some teratogeniceffect. In our local environment, it is commonpractice for ante-natal patients and indeed thegenerality of our patients to ingest herbalmedications because of fetish beliefs, poverty andignorance. The actual pharmacology of thesepreparations awaits comprehensive investigations.Demonstration of syngnathia as early as the 23rdweek of gestation may be of assistance inelucidating the aetiology and the cause-effectrelationship of herbal concoctions in further studies.In our patient there was microcephaly, anoccipitofrontal circumference of 31 cm, which wasless than the 10 percentile for age and sex at 36weeks of fetal gestation. An occipitofrontalcircumference of 31 cm is appropriate for a fetus of34 weeks gestation. The microcephaly observed maybe related to the existence of craniosynostosis. Whilethe syndromic presentation of congenital ankylosisof the temporo-mandibular joint, with cranialdeformities are noted, the pectus excavatum seen in4447 101112 134ththis patient may be a clinical manifestation of theshort sternum often noted in the trisomy heart diseaseas in Treacher Collins syndrome The surgical planwas the excision of the bony fusion but this was notaccomplished before the expiration of the patient.The management of ankylosis is challengingparticularly in a resource-poor third world countrylike Nigeria. The problems of management werefurther aggravated by the rural environment of ourhospital. Other problems are poor nutrition, the lackof neonatal anaesthetic facilities such as lack ofcorrect size of neonatal and paediatric anaesthetic/tracheostomy tube, paediatric fibre-opticlaryngoscope, absence of neonatal anesthesiologistand compliance with jaw exercise. The cause of deathin this patient remains a speculation of respiratoryd i f f i c u l t y, a s a u t o p s y w a s n o t d o n e .Temporomandibular ankylosis in children usuallylead to a compromise of the upper airway and corpulmonale .Congenital oral synechiae. Ann Otol RhinolLaryngol 1993; 102: 186-197.Treatment oftemporomandibular joint ankylosis: a casereport. J Canadian Dent Assoc 2001; 67: 659-663.C o n g e n i t a ltemporomandibular ankylosis an extreme case.The online J. Dent Oral Med.Accessed on line atComplete bony fusion of the mandible to thezygomatic complex and maxillary tuberosity:case report and review. Int J Oral MaxillofacSurg 2001; 30: 75-79.Temporomandibular joint reconstruction inchildren using autogenous costochondral grafts.J Oral Maxillofac Surg 1999; 57: 789-798.Displacementof the mandible in a child with congenitalunilateral joint ankylosis treated with the aid ofroentgen stereometric analysis. Cleft PalateCraniofacial J 2001; 38: 636-644.14,1516,17REFERENCES1. Gartlan MG, Davies J, Smith RJH.2. Rishiraj B, McFadden LR.3. Filho, MM, Palombo CR, Maccari CIT,D ' O t t a v i a n o N .4. Laster, Z, Temkin D, Zarfin Y, Kushir A.5. K o E I V, H u o n g C S , C h e n Y R .6. Wittbjer J, Sarnas K, Rune B.www.epub.org.br/ojdom/vol 04 no2/caso24/ancil_e.htm. March 2005.80Nigerian Journal of Clinical Practice March. 2008 Vol.11(1) Congenital Bilateral Fusion Ajike et al7. Nwoku AL, Kekere Ekun TA.8. Manganello Souza LC, Mariani PB.9. Dawson KL, Gruss JS.10. Humphrey T.11 Nada R.12 Poswillo D.Congenitalankylosis of the mandible. Report of a casenoted at birth. J Maxillofac Surg 1986; 14:150-152.Temporomandibular joint ankylosis. Reportof 14 cases. Int J. Oral Maxillofac Surg2003; 32: 24-29.Congenital bonysyngnathia: A proposed classification. CleftPalate Craniofacial J 1997; 34: 141-146.The relation between humanfetal mouth opening reflexes and closure of thepalate.Am JAnat 1969; 125: 317-344.Maxillo-mandibular ankylosis andcleft palate in rat embryos. J Dent Res 1970;49: 1086-1090.The pathogenesis of the first andsecond brachial arch syndrome. Oral SurgOral Med Oral Path 1973; 35:302-32813 Hegtvedt AK.14. Smith DW.16. Alday LE, Vega PJ, Heller A.17 Shashikir ND, Reddy SVV, Patil R, YavagalG.Diagnosis and management offacial asymmetry In: Peterson L, Principles oforal and maxillofacial surgery. Philadelphia: JPLipincott, 1992; 1400-1401.Recognizable pattern of humanmalformations. Vol vii. In the series SchafferAJ . Major problems in clinic pediatrics.Saunders:Toronto, 1970; 38-39, 110-111, 283.15. Nelson textbook of Paediatrics 13 ed.:Behrman and Vaughan. 1987; 245-246.Congenitalankylosis of the temporomandibular joint:resultant upper airway obstruction and corepulmonale Chest 1979; 75: 384- 386.Management of temporomandibular jointankylosis in growing children. J Indian SocPedod Preven Dent 2005; 23: 35-37.th81Nigerian Journal of Clinical Practice March. 2008 Vol.11(1) Congenital Bilateral Fusion Ajike et al

Congenital bilateral fusion of the maxillomandibular alveolar processes with  craniosynostosis: report ofa rare case

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Congenital bilateral fusion of the maxillomandibular alveolar processes with craniosynostosis: report ofa rare case

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