Negi A, Negi A, Mohanan M Solitary median maxillary central incisor syndrome: a rare entity. J Oral Maxillofac Pathol. 2020; 24
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Monteagudo A Holoprosencephaly. Am J Obstet Gynecol. 2020; 223:B13-B16
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A multidisciplinary approach to managing a missing central incisor, impacted canines and complex medical history: a case report

From Volume 17, Issue 2, April 2024 | Pages 70-74


Judi Humphreys


Senior Registrar

Articles by Judi Humphreys

Email Judi Humphreys

Asma Keshtgar

BDS, MJDF, MClinDent, MOrthRCS (Eng), BDS, MJDF, MClinDent, MOrth RCS (Eng), PGCert

Dentist, London (

Articles by Asma Keshtgar

Cathy Bryant


Consultant in Oral Surgery, King's College Hospital, Denmark Hill, London SE5 9RS, UK

Articles by Cathy Bryant

Maalini Patel

BDS(Hons), MPaedDent RCSEng, FDS RCSEng, BDS, MPaedDent RCS (Eng), FDS RCS (Eng)

Specialty Registrar in Paediatric Dentistry, Guy's and St Thomas' NHS Foundation Trust, Bristol, UK

Articles by Maalini Patel

Sanjeev Sood


Consultant in Paediatric Dentistry, King's College Dental Hospital, London, UK

Articles by Sanjeev Sood

Shruti Patel

BDS, FDS RCS (Eng), MSc (Orth), MOrth RCS (Eng), FDS RCS (Orth)

Consultant Orthodontist; King's College Hospital NHS Foundation Trust, London

Articles by Shruti Patel


Hypodontia of a maxillary central incisor is infrequent and can indicate Solitary median maxillary central incisor syndrome. We report on an 11-year-old child with medical comorbidities who presented with hypodontia of a maxillary central incisor, buccally impacted maxillary canines and root resorption of the maxillary lateral incisors. She had previously had dental extractions. Joint orthodontic–paediatric–oral surgery treatment included loss of the maxillary lateral incisors and fixed orthodontic appliances to idealize space for prosthetic replacement of a central incisor. The value of multidisciplinary planning is emphasized and the dental considerations for patients with complex medical histories explored.

CPD/Clinical Relevance: The reader should have an understanding of the importance of a joint orthodontic-paediatric-oral surgery approach when managing complex paediatric dental patients.


Solitary median maxillary central incisor (SMMCI) syndrome is a rare genetic condition that affects approximately 1:50,000 live births.1 It is characterized by hypodontia of a central incisor, with the remaining incisor appearing symmetrical, and is often associated with multisystem defects usually in the midline of the body.1 Structural defects may include absence of the upper labial frenum, an unclear philtrum, nasal cavity anomalies, septal deviation, ridge down midline of palate and hypotelorism.2 While the exact pathophysiology is not well understood, it has been reported that defects in the sonic hedgehog gene and deletion of parts of chromosome 18 and 7q can result in SMMCI syndrome.2,3,4 Genetic testing may be appropriate because it often presents as part of the phenotypic spectrum of holoprosencephaly. Holoprosencephaly occurs when the prosencephalon (embryonic forebrain) fails to divide into two separate lobes. It can also result in microcephaly, hypotelorism, single maxillary incisors and cleft lip and palate. It varies in severity with the condition being fatal prior to birth for some children, and others having nearly normal brain development.5,6 A multidisciplinary approach to the management of patients who present with one or more signs of SMMCI is therefore recommended.

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