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| CASE REPORT |
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| Year : 2014 | Volume
: 1
| Issue : 2 | Page : 112-114 |
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Esthetic makeover of a syndromic face
Kapil S Agrawal, Nilesh K Shende, Nishant A Khare, Manoj V Bacchav
Department of Plastic Surgery, The Seth GS Medical College and KEM Hospital, Parel, Mumbai, Maharashtra, India
| Date of Web Publication | 2-Aug-2014 |
Correspondence Address:
Dr. Kapil S Agrawal
#17, Anand Bhavan, B-bldg., Nair Hospital, Mumbai Central, Mumbai - 400 011, Maharashtra
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2348-2125.137911
We present a case report of a 23-year-old female with syndromic face. She presented to us with typical facial features of Crouzon syndrome. Her childhood was uneventful except for the deformed face. Craniofacial surgeries to correct the deformation were not done in childhood due to risk and complications. Patient requested for the correction of facial deformity without major craniofacial surgery. She underwent two staged procedure. In the first stage, autofat grafting of forehead, left supraorbital rim and malar areas were performed with cheek fat removal. Chin and mid-face augmentation with costal cartilage grafts were done. Septorhinoplasty was done as a second stage procedure. She has got excellent outcome by this camouflage surgical approach. This approach can be used in selective cases with minimal risk. Excellent esthetic results can be achieved by thorough analysis and involvement of the patient and parents in planning. Keywords: Autofat grafting, camouflage, costal cartilage, Crouzon syndrome
How to cite this article:
Agrawal KS, Shende NK, Khare NA, Bacchav MV. Esthetic makeover of a syndromic face. J Cleft Lip Palate Craniofac Anomal 2014;1:112-4 |
How to cite this URL:
Agrawal KS, Shende NK, Khare NA, Bacchav MV. Esthetic makeover of a syndromic face. J Cleft Lip Palate Craniofac Anomal [serial online] 2014 [cited 2023 Jun 5];1:112-4. Available from: https://www.jclpca.org/text.asp?2014/1/2/112/137911 |
| Introduction | |  |
Crouzon syndrome or craniofacial dysostosis was named after the French neurosurgeon Octave Crouzon. In 1912, he described the hereditary syndrome of craniofacial dysostosis in a mother and her son, presenting with the characteristic triad of calvarial deformities, facial anomalies, and exophthalmos. [1]
The incidence of this syndrome appears to be approximately one in 25,000 in the general population. Crouzon syndrome is characterized by craniosynostosis, maxillary hypoplasia, shallow orbits with proptosis, and bifid uvula, intracranial abnormalities such as anomalous venous drainage and hydrocephalus. [2]
The most common clinical appearance is brachycephaly. Exophthalmos is regarded as a universal feature of Crouzon syndrome. Hypertelorism is thought to arise due to a decrease in the growth of sphenozygomatic and sphenotemporal sutures. A class III malocclusion is reported in 75% of patients with Crouzon syndrome. The etiology is generally due to a retrusive and short maxilla with relative mandibular prognathism. [3]
| Case Report | | |
A 23-year-old female approached us for esthetic correction of face. Her childhood was uneventful except for craniofacial deformity. She had wide and flat forehead slanting on the left side, flat supraorbital rim and malar eminences. She also had mid-face retrusion, small chin, square face, and nasal deformity [Figure 1]. Cranioplasty and mid-face advancement surgeries were not opted by parents in childhood due to risk and complications involved. Patient requested for the correction of facial deformity without major craniofacial surgery. | Figure 1: (a) Preoperative frontal view. (b) Preoperative lateral view. (c) Preoperative bird's eye view
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Surgical technique
Treatment plan took into account the parental concerns for the risk associated with craniofacial surgery and demands of patient for correction of forehead slant, augmentation of left supraorbital rim and correction of nasal deformity. On analysis of the deformity, patient had wide and flat forehead with the slant on left, flat left supraorbital rim and malar eminences, mid-face retrusion, small chin, square face, and nasal deformity [Figure 1]. The perfect anatomical correction of forehead slant and supraorbital rim was possible by use of bone or cartilage graft, but it would have resulted in apparent anopthalmos. Hence, very subtle correction of both deformities was planned with fat transfer. Two staged esthetic makeover was planned and executed.
First stage comprised of auto fat grafting of forehead, supraorbital rim, and malar area. Mid-face and chin augmentation were done with onlay cartilage grafts [Figure 2]. Cheek fat removal was done to create a more triangular face. Medical treatment for hair restoration was started in the form of topical minoxidil lotion and multivitamin tablets in the hope of increasing density of hair to cover broad forehead, which worked effectively and brought the hairline forward [Figure 3]. Finally, in the second stage, rhinoplasty was performed by placing struts and grafts in strategic locations like dorsal and columellar struts, septal extension, intercatilaginous and shield graft coupled with alar reduction. Rhinoplasty not only corrected her nasal deformity of short nose, depressed dorsum, wide alae, and ill-defined tip, but also prevented future retraction of alae due to intercartilaginous graft [Figure 4]. Results were esthetically pleasing and are well-maintained even at the end of 1 year [Figure 5]. | Figure 2: Intraoperative photo: Pyriform augmentation with costal cartilage
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 | Figure 3: (a) Postoperative frontal view first stage. (b) Postoperative lateral view first stage. (c) Postoperative bird's eye view first stage camoufl aging of forehead slant
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 | Figure 4: (a) Septal lengthening with septal extension graft. (b) Septoplasty schematic: Intercartilaginous graft. (c) Septoplasty schematic: Various grafts and struts
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 | Figure 5: (a) Postoperative 1 year: Frontal view stage 2. (b) Postoperative 1 year lateral view stage 2. (c) Postoperative 1 year: Oblique view stage 2
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| Discussion | | |
Surgical reconstruction requires thoughtfully sequenced and staged procedures with consideration for the individual's specific malformations, craniofacial growth patterns, and psychosocial needs. [4]
In 1 st year of life (4-6 months of age) surgery for correction of craniosynostosis is advised in which release of synostotic skull suture is done. At 4-6 years of age mid-facial advancement done to provide adequate orbital volume. Finally, after facial growth is completed class III malocclusion is corrected by Le-Fort 1 osteotomy with or without orthodontic intervention. Adjuvant procedures such as genioplasty and rhinoplasty can be performed for further improvement of facial esthetics. [5]
Crouzon syndrome is one of those few syndromes in which cosmetic results of surgeries can be strikingly effective. Prognosis depends on the severity of malformation. Innovations in craniofacial surgery have enabled patients to achieve their full potential by maximizing their opportunities for intellectual growth, physical competence, and social acceptance. Patients usually have a normal life span. [6]
In cases presenting late and with mild deformity, as in our case, patient grows into a normal adult without any functional deficit except esthetic craniofacial deformity. Presently with improved technique and experience the results of fat grafting have improved considerably. We have used fat transfer in this case to correct forehead and supraorbital deformities, and to contour the face. Mid-face and chin augmentation by onlay grafting using cartilage instead of mid-facial advancement or genioplasty has been used effectively. Rest of the facial contouring was done by fat grafting. All these procedures and finally rhinoplasty changed a syndromic face to a normal looking one.
The authors are of the opinion that this is not substitute for craniofacial and maxillofacial surgery in severe deformities, but can be used in selective cases with minimal risk.
| References | | |
| 1. | Stavropoulos D. The Apert and Crouzon syndromes: General and dental aspects. Available from: http://hdl.handle.net/2077/24321. ISBN: 978-91-628-8259-4. [Last cited on 2011]. 
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| 2. | Kanaparthy R, Kanaparthy A. Craniofacial dysostosis - The dental perspective: A case report. Open Access Scientific Reports. 2012;1:196. Available from: http://www.omicsonline.org/scientific-reports/srep196.php. [Last cited on 2012 Jul 26].
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| 3. | Ahmed I, Afzal A. Diagnosis and evaluation of Crouzon syndrome. J Coll Physicians Surg Pak 2009;19:318-20.
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| 4. | Posnick JC, Ruiz RL. The craniofacial dysostosis syndromes: Current surgical thinking and future directions. Cleft Palate Craniofac J 2000;37:433.
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| 5. | Bartlett SP. Craniosynostosis syndromes. In: Thorne CH, editor-in-chief. Grab and Smith Textbook of Plastic Surgery. 6 th ed. Philadelphia, USA: Lippincott Williams & Wilkins, A Wolters Kluwer Business; 2007. p. 237-47.
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| 6. | Bowling EL, Burstein FD. Crouzon syndrome. Optometry 2006;77:217-22.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
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