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CRANIOMAXILLOFACIAL DEFORMITIES/COSMETIC SURGERY
Neonatal Coronoid Hyperplasia: A Report of a Case and Concepts to Promote Early Diagnosis and Treatment Aaron Wallender, DDS, MD,* Imran Ahson, DMD, MD,y and Barry Steinberg, MD, DDS, PhDz
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Limited mouth opening in the neonatal patient is primarily caused by either soft tissue or hard tissue pathologic features. Differentiation between the 2 can usually be elicited by physical examination with the patient under anesthesia. Limited opening from soft tissue pathologic features can be increased with stretching. In contrast, osseous pathologic features will produce an anatomic stop. Syndromic cases with hard tissue pathologic features are primarily due to coronoid hyperplasia. Our aims are to help clinicians evaluate and identify mandibular hypomobility in the pediatric patient resulting from coronoid hyperplasia and to promote early treatment to improve long-term oral function. We present the case of a 2-month-old male who was born premature at 30 weeks by emergency cesarean section. Examination revealed multiple anomalies, including significant trismus with a maximal opening of 4 mm. A computed tomography scan revealed significant bilateral coronoid hyperplasia. At the age of 90 days, the patient underwent bilateral coronoidectomies with endoscopic guidance under general anesthesia. After resection, the patient was able to open his mouth to 25 mm. This opening was maintained with postoperative physiotherapy. Clinical problems can arise from the potential sequelae of neonatal trismus. Acutely, these problems can range from feeding difficulty and potential malnutrition to aspiration and emergent airway compromise. Long-term consequences include growth restrictions because of malnutrition, speech delay, muscle contracture and atrophy, facial asymmetry, and the risk of infection owing to poor oral hygiene. Information is limited about neonatal treatment of condylar hyperplasia in the published data. Treatment tends to be delayed owing to a late diagnosis and referral, and patients are prone to developing relapse. Postoperative physical therapy will help to prevent relapse and allows for maintenance of the improved jaw range of motion. Ó 2015 American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg -:1.e1-1.e7, 2015
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The causes of neonatal trismus in the United States include congenital temporomandibular joint (TMJ) ankylosis, masseter spasm, fibrous bands in the associated soft tissue, contracture of the muscles of mastication, tetanus union of the bones involved in mastication, and coronoid hyperplasia.1 These findings can occur in isolation or be associated with syndromes, with the inciting cause generally divided into soft tissue and hard tissue pathologic entities. Any syndrome causing soft tissue contracture can lead to mandibular hypomobility, although this presentation is rare.
Syndromes in which this is commonly found include Crisponi syndrome, distal arthrogryposis 2A and 2B, and van der Woude syndrome. Worldwide, neonatal tetanus remains the most common cause of mandibular hypomobility because of substandard perinatal care and multifactorial causes. With public health advances in industrialized nations, neonatal trismus has become a rare disease process. Hard tissue pathology is primarily due to coronoid hyperplasia, which can occur in isolation or in association with syndromes such as trismus pseudocamptodactyly (TPS).1
Received from Department of Oral and Maxillofacial Surgery,
Jacksonville, 653-1 West 8th Street, LRC 2, Jacksonville, FL 32209-
University of Florida–Jacksonville, Jacksonville, FL.
6511; e-mail: [email protected]
*Pediatric Craniofacial Surgery Fellow.
Received January 5 2015
yChief Resident.
Accepted March 2 2015
zProgram Director, Pediatric Craniofacial Surgery Fellowship.
Ó 2015 American Association of Oral and Maxillofacial Surgeons
Address correspondence and reprint requests to Dr Wallender: Department of Oral and Maxillofacial Surgery, University of Florida-
0278-2391/15/00263-3 http://dx.doi.org/10.1016/j.joms.2015.03.014
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EARLY DIAGNOSIS AND TREATMENT OF NEONATAL CORONOID HYPERPLASIA
Differentiation between soft tissue and hard tissue pathology can usually be elicited from the physical examination with the patient under sedation or general anesthesia. Limited opening can be minimally increased with stretching in the case of soft tissue pathologic features. Trismus resulting from bony pathologic entities, such as ankylosis or coronoid hyperplasia, cannot be manipulated beyond a certain point. This initial evaluation can help determine the need for additional radiographic evaluation. Once the inciting cause has been diagnosed as coronoid hyperplasia, the treatment of choice is bilateral coronoidectomies with aggressive postoperative physical therapy.1-7 This has been performed effectively in a wide variety of age groups, with the youngest patient reported in a published study aged 8 months.8 Although relapse is common, those patients who were able to sustain physical therapy were able to achieve increased mouth opening for years after the initial diagnosis.9 However, children with neonatal trismus are often undiagnosed and untreated, leading to difficulty with nutrition, speech, and potential airway compromise.8 We present a case of neonatal trismus diagnosed in a patient 2 months old with a unique presentation of multiple anomalies, including bilateral coronoid hyperplasia, and our early treatment approach. Although multiple scientific studies have been reported about syndromic and nonsyndromic coronoid hyperplasia, no information is available regarding neonatal treatment. We hypothesized that early diagnosis and treatment of coronoid hyperplasia would improve the function and development of such children. Our aim was to help clinicians evaluate and identify mandibular hypomobility due to coronoid hyperplasia to improve their long-term growth and function. Our patient possessed a novel constellation of physical examination findings that might aid in identifying other patients with this presentation. In addition to our case, we present a review of the published data to also aid in diagnosis of this rare, but clinically significant, finding.
Case Report The oral and maxillofacial surgery department at the University of Florida–Jacksonville was consulted to evaluate a neonatal patient with limited mouth opening. The patient was a 2-month-old male who had been born prematurely at 30 weeks by emergency cesarean section because of fetal distress related to group B streptococcal-positive preterm premature rupture of membranes. His birth history included an Apgar score of 1 at 1 minute, with a subsequent score of 8 at 5 and 10 minutes. His appearance at birth was floppy, apneic, cyanotic, and pale with poor pulmonary function, giving clinical evidence for pulmonary
hypoplasia. Cardiopulmonary resuscitation was initiated with uneventful endotracheal intubation and administration of surfactant. The patient’s vital signs then stabilized. On additional examination, multiple anomalies were noted, including hydrocephalus, ventriculomegaly, left renal duplication, vertical talus, partial agenesis of the corpus callosum, periventricular white matter changes, hypospadias, micrognathia, polydactyly, bilateral talipes equinovarus (clubbed feet), and a cleft palate (Fig 1). He was later extubated, and enteral feeding was administered by way of a nasogastric tube. He continued to have difficulty feeding, and an attempt was made to place a feeding gastrostomy tube. This was aborted because of difficulty with intubation. Previously unnoticed, the patient had significant trismus and attempts to intubate the patient using nasal endoscopy were unsuccessful. It was at this point that the oral and maxillofacial surgery department was consulted to evaluate the trismus. The physical examination revealed significant trismus with a jaw opening of 4 mm. Downward pressure on the patient’s mandible revealed a minute amount of movement and then an abrupt stop, consistent with bony obstruction. The patient exhibited grimacing with additional manipulation. Computed tomography (CT) revealed hyperplasia of the mandibular coronoid processes bilaterally (Fig 2). The remainder of the mandible was mildly hypoplastic. Genetic analysis revealed interstitial duplication of chromosome 7P21.1. We reviewed the published data findings associated with this genetic abnormality. Once medically stable at 90 days old, the patient underwent bilateral coronoidectomies under general anesthesia. He underwent endotracheal intubation using a fiberoptic nasal approach. The airway was noted to be difficult to access owing to the overriding posterior tongue in the hypopharynx, preventing proper visualization. Successful intubation was achieved on the third attempt. The examination with the patient under general anesthesia and the administration of paralytics revealed the patient’s jaw opening was 7 mm with the assistance of a Molt mouth prop (Fig 3). An incision was made along the anterior mandibular ramus. Subperiosteal dissection was performed superiorly to expose the full extent of the coronoid. Care was taken to stay within this plane and to completely remove the attachments of the temporalis muscle. A straight Kocher clamp was attached to the superior portion of the coronoid process. A nerve hook was then placed in the mandibular notch just posterior to the coronoid process. An endoscope was used to directly visualize the notch and proper placement of the nerve hook (Fig 4). The coronoid was osteotomized using straight scissors placed just above the nerve hook.
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FIGURE 1. A, Polydactyly of the right hand. B, Bilateral talipes equinovarus (clubbed feet). C, cleft palate. Wallender, Ahson, and Steinberg. Early Diagnosis and Treatment of Neonatal Coronoid Hyperplasia. J Oral Maxillofac Surg 2015.
Complete removal of the coronoid process was confirmed using endoscopic assistance (Fig 5). The sharp edges were removed with rongeurs, the bleeding points were cauterized, and the incisions
were closed with 4-0 Vicryl sutures. This technique was performed bilaterally. After completion, the patient was able to open to 25 mm (20 mm passively; Fig 6). The patient remained intubated postoperatively
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WALLENDER, AHSON, AND STEINBERG
FIGURE 2. A, Three-dimensional (3D) computed tomography (CT) scan of the right side. B, 3D-CT scan of the left side. Wallender, Ahson, and Steinberg. Early Diagnosis and Treatment of Neonatal Coronoid Hyperplasia. J Oral Maxillofac Surg 2015.
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EARLY DIAGNOSIS AND TREATMENT OF NEONATAL CORONOID HYPERPLASIA
FIGURE 3. Preoperative mandibular opening. Wallender, Ahson, and Steinberg. Early Diagnosis and Treatment of Neonatal Coronoid Hyperplasia. J Oral Maxillofac Surg 2015.
and was given scheduled corticosteroids because of the difficult intubation. Jaw physiotherapy began 48 hours postoperatively every 4 hours with finger manipulation. At 1 week postoperatively, his opening at rest was 10 mm and could be manipulated to 25 mm with little resistance.
Discussion In the initial evaluation of the patient with neonatal trismus, a differentiation must be made between hard tissue and soft tissue pathologic entities. Soft tissue pathologic features can have a variety of sources, including masseter spasm, fibrous bands in the associated soft tissue, and contracture of the muscles of mastication. Syndromes with soft tissue limitation include Crisponi syndrome, distal arthrogryposis 2A
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FIGURE 4. Visualization of placement of the nerve hook under endoscopy. Wallender, Ahson, and Steinberg. Early Diagnosis and Treatment of Neonatal Coronoid Hyperplasia. J Oral Maxillofac Surg 2015.
and 2B, and van der Woude syndrome. In general, any of the many congenital disorders that involve contracture of the associated soft tissues have the potential to cause limited mouth opening, although the presentation is rare.1 In contrast, osseous pathologic features in neonates are primarily limited to coronoid hyperplasia owing to the rarity of true congenital TMJ ankylosis. In our review of the published data, only 5 cases of congenital TMJ ankylosis have been described, all unassociated with syndromes. Although not a true trismus, deformations of the oral structures such as in Pierre-Robin sequence can also lead to limited mouth opening and potential hypomobility.1 Because our patient was eventually diagnosed with trismus secondary to syndromic coronoid hyperplasia, our review targeted the syndromes associated with this finding. The most common syndrome associated with congenital bilateral coronoid hyperplasia is TPS or Hecht-Beals syndrome.1 First described by Van Hoof and Besling, it was later determined to be an autosomal dominant syndrome by Hecht and Beals and Wilson in 1969. Genetic analysis of patients with TPS has revealed a defective MYH8 gene, which is hypothesized to interfere with the catalytic activity of myosin and possibly the binding of myosin to actin. This defect leads to distal arthrogryposis with multiple muscle contractures. Classic findings include bilateral coronoid hyperplasia and limited finger extension with wrist dorsiflexion. The spectrum of this disease has multiple other associated findings, including lower extremity deformities and a reduced stature. A full listing of the findings associated with TPS are presented in Table 1. The syndrome has been colloquially termed in the past as DutchKentucky syndrome by Mabry in 1974 who analyzed a family pedigree that expressed these findings.2 However, TPS has been shown to be associated with other populations, and this term has fallen out of favor. Multiple other syndromes have been shown to produce coronoid hyperplasia, with a common finding of hypotonia. One such presentation is Moebius syndrome, which is characterized by unilateral or bilateral paralysis of the facial mimetic muscles and the lateral rectus muscle of the eye. The pathophysiology is thought to be either a central nervous system hypoplasia or a primary peripheral muscle defect with secondary nerve degeneration. Bilateral coronoid hypoplasia has been reported in patients with this syndrome.10 In addition, Puche et al8 described 2 unrelated cases of patients presenting with coronoid hyperplasia with syndromic diagnoses of Kabuki syndrome and PenaShokeir syndrome. Both syndromes have associated hypotonia, leading the investigators to suggest that hypotonia of the mandibular depressors leads to
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FIGURE 5. A, Partial removal of the coronoid. B, Complete removal of the coronoid. Wallender, Ahson, and Steinberg. Early Diagnosis and Treatment of Neonatal Coronoid Hyperplasia. J Oral Maxillofac Surg 2015.
relative hyperactivity of the temporalis muscle.8 The investigators hypothesized that this exaggerated muscle activity causes a subsequent enlargement of the coronoid, with the eventual presentation of trismus.8 Although hyperactivity of the temporalis in the presence of hypotonia has been supported as a cause in isolated animal studies, the exact pathogenesis of both syndromic and nonsyndromic coronoid hyperplasia remains undetermined. Electromyographic studies of temporalis muscles in other animal models
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WALLENDER, AHSON, AND STEINBERG
FIGURE 6. Passive postoperative mandibular opening. Wallender, Ahson, and Steinberg. Early Diagnosis and Treatment of Neonatal Coronoid Hyperplasia. J Oral Maxillofac Surg 2015.
with coronoid hyperplasia were consistent with normal values, leading to contention of this theory.8 Other theories include endocrine abnormalities and persistence of the coronoid cartilage growth center. In our patient, the duplication of chromosome 7p21.1 was a particularly interesting finding. The short arm of chromosome 7 harbors different developmental regulator genes, including the TWIST gene on 7p21 and the HOXA gene cluster on 7p15.11 Haploinsufficiency of these genes by either deletion or mutation leads to Saethre-Chotzen syndrome and handfoot-uterus syndrome, respectively.12 The 7p duplication most commonly results from malsegregation of a parental balanced translocation or through abnormal recombination caused by a parental inversion.13 The phenotypic expression of any chromosome duplication can include mental and growth retardation, hypotonia, microcephaly, large anterior fontanelle, high forehead, hypertelorism, slanted palpebral fissures, low nasal bridge, low set ears, abnormal palate (highly arched or cleft), cardiovascular abnormalities, abnormal palmar creases, skeletal anomalies, and autistic disorder. In pure 7p duplication without other chromosome involvement, the most common symptoms include mental retardation, hypotonia, craniofacial dysmorphism, abnormal palmar creases, skeletal anomalies, and cardiovascular abnormalities. Our patient had some of these findings, plus symptoms as yet not described in association with 7p duplication (eg, coronoid hypertrophy). Some investigators have suggested that the critical segment causing the characteristic craniofacial dysmorphism in the 7p duplication syndrome maps at or around 7p21, just as in our patient.12
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EARLY DIAGNOSIS AND TREATMENT OF NEONATAL CORONOID HYPERPLASIA
Table 1. CHARACTERISTICS OF TRISMUS PSEUDOCAMPTODACTYLY SYNDROME REPORTED IN PUBLISHED STUDIES
Variable
Description
General characteristics Inheritance Autosomal dominant Growth Mildly reduced stature (3rd to 25th percentile) Head and neck Head Macrocephaly Eyes Ptosis Mouth Limited opening Normal temporomandibular joints Coronoid hyperplasia Shortened muscles of mastication Fibrous bands between muscles of mastication Micrognathia Face Deep philtrum Nodular cords in nasolabial folds Neck Torticollis Webbed neck Skeletal Skull Lateral pterygoid plate hyperplasia Pelvis Hip dislocation Upper extremity Reduced elbow supination Lower extremity Metatarsus adductus Talipes equinovarus Vertical talipes Muscle, soft tissue Upper extremity Interphalangeal webbing Flexion of fingers when dorsiflexed (pseudocamptodactyly) Shortening of flexor profundus muscle–tendon unit Lower extremity Short gastrocnemius Shortening of various muscle– tendon groups in legs and feet Soft tissue syndactyly of toes Wallender, Ahson, and Steinberg. Early Diagnosis and Treatment of Neonatal Coronoid Hyperplasia. J Oral Maxillofac Surg 2015.
Aside from the novelty of our case, we believe that the presentation of our patient highlights many important clinical issues noted in published studies. For example, in our review, the most objective method to evaluate coronoid hyperplasia described is to measure the coronoid/condylar volume and surface area ratios.14 Although we had 3-dimensional CT imaging scans to evaluate the hyperplasia, we did not possess the postprocessing software to quantify these ratios. For clinicians who treat pediatric patients on a routine basis, we do not believe this measurement is necessary. The findings from a physical examination and CT scan will be sufficient to diagnose coronoid hyperplasia.
In addition, it is important to note that clinical problems can arise from the potential sequelae of limited mouth opening. Acutely, these problems can range from feeding difficulty and potential malnutrition to airway compromise causing apnea and emergent airway compromise. Neonates with trismus are also reported to have difficulty clearing secretions, causing more frequent aspirations. Our patient was experiencing all these issues at the diagnosis. The long-term consequences of trismus can include growth restriction due to malnutrition, speech delay, muscle contracture and atrophy, facial asymmetry, and the risk of infection owing to poor oral hygiene. We believe that prompt diagnosis and treatment is essential to prevent these acute and long-term consequences. Most importantly, our treatment of this neonate highlights the airway risk associated with neonatal trismus. Whether isolated or appearing in conjunction with syndromic findings, most patients appear to have airway abnormalities. In our patient, multiple anesthesia practitioners had difficulty visualizing the tracheolaryngeal anatomy. Even with fiberoptic capabilities, our anesthesia colleagues had difficulty with visualization and management of our patient’s airway. The published studies have reported various approaches to managing the airway in patients with TPS ranging from mask anesthesia with spontaneous ventilation and the avoidance of muscle relaxants to blind nasoendotracheal intubation.15-17 In our patient, endotracheal intubation became increasingly more difficult as the child grew older, likely because of the worsening trismus. It is unclear whether this situation was similar in other cases. Although multiple sequelae can result from neonatal trismus, surgical treatment tends to be delayed by late diagnosis and referral. Once diagnosed and treated, physical therapy was frequently attempted, with minimal improvement.4 In the published studies, all reported patients eventually underwent bilateral coronoidectomy, with the intraoral approach the most common.1-7 Regarding coronoid hyperplasia, the youngest patient treated with surgery reported in the published data was 8 months old.4 In the few cases reported, surgical treatment has been prone to relapse, and the trismus often recurs. Postoperative physical therapy will help to minimize recurrence and allows for sustained improvement in the jaw range of motion. If surgical correction can be accomplished at an early age with the combination of jaw physiotherapy, we believe such children have the potential for permanent normalization of jaw mobility. Patients who underwent surgical correction at older ages underwent physical therapy with poor compliance. This was associated with greater rates of relapse. The published data have shown that compliance with physical therapy has a bimodal distribution.1 Patients
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younger than 2 years old and older than 16 years have reportedly had better results. Our patient is currently the youngest reported patient to have undergone this therapy. His postoperative result was maintained for months after surgery without the need for more aggressive therapy. Long-term management and surveillance of this patient population is of paramount importance because of the risk of relapse. Lefaivre and Aitchison6 attributed the absence of relapse in the treatment of TPS to continuous jaw physiotherapy, independent of the surgical technique. When comparing physical therapy options, they achieved a greater opening after 12 months of passive stretching of the jaw using tongue blade therapy. They noted that long-term therapy might be the most important variable affecting the long-term outcomes and advised waiting until patient cooperation would be optimal to otherwise avoid necessary intervention. Hirano et al9 also noted the importance of postoperative manipulation after initial surgery in achieving normal mouth opening, because the TMJ might have been restricted in its movement during the fetal period. In any form of trismus, it is important to have close follow-up to monitor the patient’s postoperative progression. Because the incidence of relapse is significant in these patients, it is important to discover relapse early to make modifications to the physical therapy or perform another operative procedure. If the trismus recurs and is left untreated, the aforementioned complications could occur, and the patient will be at an increased risk of ankylosis. Our patient presented with multiple congenital abnormalities, not described for any particular spectrum or syndrome. It remains unknown if these resulted from a new pathologic process or a worsening spectrum of syndromes already presented in the scientific data. Regardless, after our patient underwent surgery at 90 days of age, we were able to significantly increase his mouth opening and maintain the result with physiotherapy. Although more research is needed, we believe that early treatment would benefit these patients and can be performed safely and effectively.
Uncited References 18, 19, 20, 21, 22, 23.
References 1. Jaskolka MS, Eppley BL, van Aalst JA: Mandibular coronoid hyperplasia in pediatric patients. J Craniofac Surg 18:849, 2007
2. Carlos R, Contreras E, Cabrera J: Trismus-pseudocamptodactyly syndrome (Hecht–Beals’ syndrome): Case report and literature review. Oral Dis 11:186, 2005 3. Costello BJ, Edwards SP: Pediatric mandibular hypomobility: Current management and controversies. Oral Maxillofac Surg Clin North Am 17:455, 2005 4. Fabie L, Boutault F, Gas C, Paoli J-R: Neonatal bilateral idiopathic hyperplasia of the coronoid processes: Case report. J Oral Maxillofac Surg 60:459, 2002 5. Karras SC, Wolford LM: Trismus-pseudocamptodactyly syndrome: Report of a case. J Oral Maxillofac Surg 53:80, 1995 6. Lefaivre J-F, Aitchison MJ: Surgical correction of trismus in a child with Hecht syndrome. Ann Plast Surg 50:310, 2003 7. Visscher SH, Schortinghuis J, Bos RRM: Congenital mandibular hypomobility: A rare condition with little consensus—A case report. J Oral Maxillofac Surg 67:444, 2009 8. Puche M, Guijarro-Martınez R, Perez-Herrezuelo G, et al: The hypothetical role of congenital hypotonia in the development of early coronoid hyperplasia. J Craniomaxillofac Surg 40:e155, 2012 9. Hirano A, Iio Y, Murakami R, et al: Recurrent trismus: Twentyyear follow-up result. Cleft Palate Craniofac J 31:309, 1994 10. Turk A, McCarthy J, Nichter L, Thorne C: Moebius syndrome: The new finding of hypertrophy of the coronoid process. J Craniofac Surg 10:93, 1999 11. Fryssira H, Makrythanasis P, Kattamis A: Severe developmental delay in a patient with 7p21.1–p14.3 microdeletion spanning the TWIST gene and the HOXA gene cluster. Mol Syndromol 2: 45, 2011 12. Cai T, Yu P, Tagle DA, Xia J: Duplication of 7p21.2/pter due to maternal 7p;21q translocation: Implications for critical segment assignment in the 7p duplication syndrome. Am J Med Genet 86: 305, 1999 13. Papadopoulou E, Sifakis S, Sarri C: A report of pure 7p duplication syndrome and review of the literature. Am J Med Genet Part A 140A:2802, 2006 14. Chang C, Allori A, Wang E, Farina R: A quantitative threedimensional analysis of coronoid hypertrophy in pediatric craniofacial malformations. Plast Reconstr Surg 129:312e, 2012 15. Nagata O, Tateoka A, Shiro R, Kimizuka M: Anaesthetic management of two paediatric patients with Hecht–Beals syndrome. Paediatr Anaesth 9:444, 1999 16. Seavello J, Hammer GB: Tracheal intubation in a child with trismus pseudocamptodactyly (Hecht) syndrome. J Clin Anesth 11:254, 1999 17. Vaghadia H, Blackstock D: Anaesthetic implications of the trismus pseudocamptodactyly (Dutch-Kentucky or Hecht Beals) syndrome. Can J Anaesth 35:80, 1988 18. Callewaert BL, Loeys BL, Ficcadenti A, et al: Comprehensive clinical and molecular assessment of 32 probands with congenital contractural arachnodactyly: Report of 14 novel mutations and review of the literature. Hum Mutat 30:334, 2009 19. Gerbino G, Bianchi SD, Bernardi M, Berrone S: Hyperplasia of the mandibular coronoid process: Long-term follow-up after coronoidotomy. J Craniomaxillofac Surg 25:169, 1997 20. Minzer-Conzetti K, Wu E, Vargervik K, Slavotinek A: Phenotypic variation in trismus-pseudocamptodactyly syndrome caused by a recurrent MYH8 mutation. Clin Dysmorphol 17:1, 2008 21. Nordone TP, Li P: Arthrogryposis multiplex congenita in association with bilateral temporomandibular joint hypomobility: Report of a case and review of literature. J Oral Maxillofac Surg 68:1197, 2010 22. Stopa Z, Wanyura H, Kowalczyk P: Coronoid-condylar index in assessing of mandibular coronoid hyperplasia: Preliminary results. Adv Med Sci 58:429, 2013 23. Zhong S-C, Xu Z-J, Zhang Z-G, et al: Bilateral coronoid hyperplasia (Jacob disease on right and elongation on left): Report of a case and literature review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 107:e64, 2009
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CRANIOMAXILLOFACIAL DEFORMITIES/COSMETIC SURGERY
Neonatal Coronoid Hyperplasia: A Report of a Case and Concepts to Promote Early Diagnosis and Treatment Aaron Wallender, DDS, MD,* Imran Ahson, DMD, MD,y and Barry Steinberg, MD, DDS, PhDz
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Limited mouth opening in the neonatal patient is primarily caused by either soft tissue or hard tissue pathologic features. Differentiation between the 2 can usually be elicited by physical examination with the patient under anesthesia. Limited opening from soft tissue pathologic features can be increased with stretching. In contrast, osseous pathologic features will produce an anatomic stop. Syndromic cases with hard tissue pathologic features are primarily due to coronoid hyperplasia. Our aims are to help clinicians evaluate and identify mandibular hypomobility in the pediatric patient resulting from coronoid hyperplasia and to promote early treatment to improve long-term oral function. We present the case of a 2-month-old male who was born premature at 30 weeks by emergency cesarean section. Examination revealed multiple anomalies, including significant trismus with a maximal opening of 4 mm. A computed tomography scan revealed significant bilateral coronoid hyperplasia. At the age of 90 days, the patient underwent bilateral coronoidectomies with endoscopic guidance under general anesthesia. After resection, the patient was able to open his mouth to 25 mm. This opening was maintained with postoperative physiotherapy. Clinical problems can arise from the potential sequelae of neonatal trismus. Acutely, these problems can range from feeding difficulty and potential malnutrition to aspiration and emergent airway compromise. Long-term consequences include growth restrictions because of malnutrition, speech delay, muscle contracture and atrophy, facial asymmetry, and the risk of infection owing to poor oral hygiene. Information is limited about neonatal treatment of condylar hyperplasia in the published data. Treatment tends to be delayed owing to a late diagnosis and referral, and patients are prone to developing relapse. Postoperative physical therapy will help to prevent relapse and allows for maintenance of the improved jaw range of motion. Ó 2015 American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg -:1.e1-1.e7, 2015
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The causes of neonatal trismus in the United States include congenital temporomandibular joint (TMJ) ankylosis, masseter spasm, fibrous bands in the associated soft tissue, contracture of the muscles of mastication, tetanus union of the bones involved in mastication, and coronoid hyperplasia.1 These findings can occur in isolation or be associated with syndromes, with the inciting cause generally divided into soft tissue and hard tissue pathologic entities. Any syndrome causing soft tissue contracture can lead to mandibular hypomobility, although this presentation is rare.
Syndromes in which this is commonly found include Crisponi syndrome, distal arthrogryposis 2A and 2B, and van der Woude syndrome. Worldwide, neonatal tetanus remains the most common cause of mandibular hypomobility because of substandard perinatal care and multifactorial causes. With public health advances in industrialized nations, neonatal trismus has become a rare disease process. Hard tissue pathology is primarily due to coronoid hyperplasia, which can occur in isolation or in association with syndromes such as trismus pseudocamptodactyly (TPS).1
Received from Department of Oral and Maxillofacial Surgery,
Jacksonville, 653-1 West 8th Street, LRC 2, Jacksonville, FL 32209-
University of Florida–Jacksonville, Jacksonville, FL.
6511; e-mail: [email protected]
*Pediatric Craniofacial Surgery Fellow.
Received January 5 2015
yChief Resident.
Accepted March 2 2015
zProgram Director, Pediatric Craniofacial Surgery Fellowship.
Ó 2015 American Association of Oral and Maxillofacial Surgeons
Address correspondence and reprint requests to Dr Wallender: Department of Oral and Maxillofacial Surgery, University of Florida-
0278-2391/15/00263-3 http://dx.doi.org/10.1016/j.joms.2015.03.014
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EARLY DIAGNOSIS AND TREATMENT OF NEONATAL CORONOID HYPERPLASIA
Differentiation between soft tissue and hard tissue pathology can usually be elicited from the physical examination with the patient under sedation or general anesthesia. Limited opening can be minimally increased with stretching in the case of soft tissue pathologic features. Trismus resulting from bony pathologic entities, such as ankylosis or coronoid hyperplasia, cannot be manipulated beyond a certain point. This initial evaluation can help determine the need for additional radiographic evaluation. Once the inciting cause has been diagnosed as coronoid hyperplasia, the treatment of choice is bilateral coronoidectomies with aggressive postoperative physical therapy.1-7 This has been performed effectively in a wide variety of age groups, with the youngest patient reported in a published study aged 8 months.8 Although relapse is common, those patients who were able to sustain physical therapy were able to achieve increased mouth opening for years after the initial diagnosis.9 However, children with neonatal trismus are often undiagnosed and untreated, leading to difficulty with nutrition, speech, and potential airway compromise.8 We present a case of neonatal trismus diagnosed in a patient 2 months old with a unique presentation of multiple anomalies, including bilateral coronoid hyperplasia, and our early treatment approach. Although multiple scientific studies have been reported about syndromic and nonsyndromic coronoid hyperplasia, no information is available regarding neonatal treatment. We hypothesized that early diagnosis and treatment of coronoid hyperplasia would improve the function and development of such children. Our aim was to help clinicians evaluate and identify mandibular hypomobility due to coronoid hyperplasia to improve their long-term growth and function. Our patient possessed a novel constellation of physical examination findings that might aid in identifying other patients with this presentation. In addition to our case, we present a review of the published data to also aid in diagnosis of this rare, but clinically significant, finding.
Case Report The oral and maxillofacial surgery department at the University of Florida–Jacksonville was consulted to evaluate a neonatal patient with limited mouth opening. The patient was a 2-month-old male who had been born prematurely at 30 weeks by emergency cesarean section because of fetal distress related to group B streptococcal-positive preterm premature rupture of membranes. His birth history included an Apgar score of 1 at 1 minute, with a subsequent score of 8 at 5 and 10 minutes. His appearance at birth was floppy, apneic, cyanotic, and pale with poor pulmonary function, giving clinical evidence for pulmonary
hypoplasia. Cardiopulmonary resuscitation was initiated with uneventful endotracheal intubation and administration of surfactant. The patient’s vital signs then stabilized. On additional examination, multiple anomalies were noted, including hydrocephalus, ventriculomegaly, left renal duplication, vertical talus, partial agenesis of the corpus callosum, periventricular white matter changes, hypospadias, micrognathia, polydactyly, bilateral talipes equinovarus (clubbed feet), and a cleft palate (Fig 1). He was later extubated, and enteral feeding was administered by way of a nasogastric tube. He continued to have difficulty feeding, and an attempt was made to place a feeding gastrostomy tube. This was aborted because of difficulty with intubation. Previously unnoticed, the patient had significant trismus and attempts to intubate the patient using nasal endoscopy were unsuccessful. It was at this point that the oral and maxillofacial surgery department was consulted to evaluate the trismus. The physical examination revealed significant trismus with a jaw opening of 4 mm. Downward pressure on the patient’s mandible revealed a minute amount of movement and then an abrupt stop, consistent with bony obstruction. The patient exhibited grimacing with additional manipulation. Computed tomography (CT) revealed hyperplasia of the mandibular coronoid processes bilaterally (Fig 2). The remainder of the mandible was mildly hypoplastic. Genetic analysis revealed interstitial duplication of chromosome 7P21.1. We reviewed the published data findings associated with this genetic abnormality. Once medically stable at 90 days old, the patient underwent bilateral coronoidectomies under general anesthesia. He underwent endotracheal intubation using a fiberoptic nasal approach. The airway was noted to be difficult to access owing to the overriding posterior tongue in the hypopharynx, preventing proper visualization. Successful intubation was achieved on the third attempt. The examination with the patient under general anesthesia and the administration of paralytics revealed the patient’s jaw opening was 7 mm with the assistance of a Molt mouth prop (Fig 3). An incision was made along the anterior mandibular ramus. Subperiosteal dissection was performed superiorly to expose the full extent of the coronoid. Care was taken to stay within this plane and to completely remove the attachments of the temporalis muscle. A straight Kocher clamp was attached to the superior portion of the coronoid process. A nerve hook was then placed in the mandibular notch just posterior to the coronoid process. An endoscope was used to directly visualize the notch and proper placement of the nerve hook (Fig 4). The coronoid was osteotomized using straight scissors placed just above the nerve hook.
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FIGURE 1. A, Polydactyly of the right hand. B, Bilateral talipes equinovarus (clubbed feet). C, cleft palate. Wallender, Ahson, and Steinberg. Early Diagnosis and Treatment of Neonatal Coronoid Hyperplasia. J Oral Maxillofac Surg 2015.
Complete removal of the coronoid process was confirmed using endoscopic assistance (Fig 5). The sharp edges were removed with rongeurs, the bleeding points were cauterized, and the incisions
were closed with 4-0 Vicryl sutures. This technique was performed bilaterally. After completion, the patient was able to open to 25 mm (20 mm passively; Fig 6). The patient remained intubated postoperatively
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WALLENDER, AHSON, AND STEINBERG
FIGURE 2. A, Three-dimensional (3D) computed tomography (CT) scan of the right side. B, 3D-CT scan of the left side. Wallender, Ahson, and Steinberg. Early Diagnosis and Treatment of Neonatal Coronoid Hyperplasia. J Oral Maxillofac Surg 2015.
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EARLY DIAGNOSIS AND TREATMENT OF NEONATAL CORONOID HYPERPLASIA
FIGURE 3. Preoperative mandibular opening. Wallender, Ahson, and Steinberg. Early Diagnosis and Treatment of Neonatal Coronoid Hyperplasia. J Oral Maxillofac Surg 2015.
and was given scheduled corticosteroids because of the difficult intubation. Jaw physiotherapy began 48 hours postoperatively every 4 hours with finger manipulation. At 1 week postoperatively, his opening at rest was 10 mm and could be manipulated to 25 mm with little resistance.
Discussion In the initial evaluation of the patient with neonatal trismus, a differentiation must be made between hard tissue and soft tissue pathologic entities. Soft tissue pathologic features can have a variety of sources, including masseter spasm, fibrous bands in the associated soft tissue, and contracture of the muscles of mastication. Syndromes with soft tissue limitation include Crisponi syndrome, distal arthrogryposis 2A
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FIGURE 4. Visualization of placement of the nerve hook under endoscopy. Wallender, Ahson, and Steinberg. Early Diagnosis and Treatment of Neonatal Coronoid Hyperplasia. J Oral Maxillofac Surg 2015.
and 2B, and van der Woude syndrome. In general, any of the many congenital disorders that involve contracture of the associated soft tissues have the potential to cause limited mouth opening, although the presentation is rare.1 In contrast, osseous pathologic features in neonates are primarily limited to coronoid hyperplasia owing to the rarity of true congenital TMJ ankylosis. In our review of the published data, only 5 cases of congenital TMJ ankylosis have been described, all unassociated with syndromes. Although not a true trismus, deformations of the oral structures such as in Pierre-Robin sequence can also lead to limited mouth opening and potential hypomobility.1 Because our patient was eventually diagnosed with trismus secondary to syndromic coronoid hyperplasia, our review targeted the syndromes associated with this finding. The most common syndrome associated with congenital bilateral coronoid hyperplasia is TPS or Hecht-Beals syndrome.1 First described by Van Hoof and Besling, it was later determined to be an autosomal dominant syndrome by Hecht and Beals and Wilson in 1969. Genetic analysis of patients with TPS has revealed a defective MYH8 gene, which is hypothesized to interfere with the catalytic activity of myosin and possibly the binding of myosin to actin. This defect leads to distal arthrogryposis with multiple muscle contractures. Classic findings include bilateral coronoid hyperplasia and limited finger extension with wrist dorsiflexion. The spectrum of this disease has multiple other associated findings, including lower extremity deformities and a reduced stature. A full listing of the findings associated with TPS are presented in Table 1. The syndrome has been colloquially termed in the past as DutchKentucky syndrome by Mabry in 1974 who analyzed a family pedigree that expressed these findings.2 However, TPS has been shown to be associated with other populations, and this term has fallen out of favor. Multiple other syndromes have been shown to produce coronoid hyperplasia, with a common finding of hypotonia. One such presentation is Moebius syndrome, which is characterized by unilateral or bilateral paralysis of the facial mimetic muscles and the lateral rectus muscle of the eye. The pathophysiology is thought to be either a central nervous system hypoplasia or a primary peripheral muscle defect with secondary nerve degeneration. Bilateral coronoid hypoplasia has been reported in patients with this syndrome.10 In addition, Puche et al8 described 2 unrelated cases of patients presenting with coronoid hyperplasia with syndromic diagnoses of Kabuki syndrome and PenaShokeir syndrome. Both syndromes have associated hypotonia, leading the investigators to suggest that hypotonia of the mandibular depressors leads to
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FIGURE 5. A, Partial removal of the coronoid. B, Complete removal of the coronoid. Wallender, Ahson, and Steinberg. Early Diagnosis and Treatment of Neonatal Coronoid Hyperplasia. J Oral Maxillofac Surg 2015.
relative hyperactivity of the temporalis muscle.8 The investigators hypothesized that this exaggerated muscle activity causes a subsequent enlargement of the coronoid, with the eventual presentation of trismus.8 Although hyperactivity of the temporalis in the presence of hypotonia has been supported as a cause in isolated animal studies, the exact pathogenesis of both syndromic and nonsyndromic coronoid hyperplasia remains undetermined. Electromyographic studies of temporalis muscles in other animal models
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WALLENDER, AHSON, AND STEINBERG
FIGURE 6. Passive postoperative mandibular opening. Wallender, Ahson, and Steinberg. Early Diagnosis and Treatment of Neonatal Coronoid Hyperplasia. J Oral Maxillofac Surg 2015.
with coronoid hyperplasia were consistent with normal values, leading to contention of this theory.8 Other theories include endocrine abnormalities and persistence of the coronoid cartilage growth center. In our patient, the duplication of chromosome 7p21.1 was a particularly interesting finding. The short arm of chromosome 7 harbors different developmental regulator genes, including the TWIST gene on 7p21 and the HOXA gene cluster on 7p15.11 Haploinsufficiency of these genes by either deletion or mutation leads to Saethre-Chotzen syndrome and handfoot-uterus syndrome, respectively.12 The 7p duplication most commonly results from malsegregation of a parental balanced translocation or through abnormal recombination caused by a parental inversion.13 The phenotypic expression of any chromosome duplication can include mental and growth retardation, hypotonia, microcephaly, large anterior fontanelle, high forehead, hypertelorism, slanted palpebral fissures, low nasal bridge, low set ears, abnormal palate (highly arched or cleft), cardiovascular abnormalities, abnormal palmar creases, skeletal anomalies, and autistic disorder. In pure 7p duplication without other chromosome involvement, the most common symptoms include mental retardation, hypotonia, craniofacial dysmorphism, abnormal palmar creases, skeletal anomalies, and cardiovascular abnormalities. Our patient had some of these findings, plus symptoms as yet not described in association with 7p duplication (eg, coronoid hypertrophy). Some investigators have suggested that the critical segment causing the characteristic craniofacial dysmorphism in the 7p duplication syndrome maps at or around 7p21, just as in our patient.12
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EARLY DIAGNOSIS AND TREATMENT OF NEONATAL CORONOID HYPERPLASIA
Table 1. CHARACTERISTICS OF TRISMUS PSEUDOCAMPTODACTYLY SYNDROME REPORTED IN PUBLISHED STUDIES
Variable
Description
General characteristics Inheritance Autosomal dominant Growth Mildly reduced stature (3rd to 25th percentile) Head and neck Head Macrocephaly Eyes Ptosis Mouth Limited opening Normal temporomandibular joints Coronoid hyperplasia Shortened muscles of mastication Fibrous bands between muscles of mastication Micrognathia Face Deep philtrum Nodular cords in nasolabial folds Neck Torticollis Webbed neck Skeletal Skull Lateral pterygoid plate hyperplasia Pelvis Hip dislocation Upper extremity Reduced elbow supination Lower extremity Metatarsus adductus Talipes equinovarus Vertical talipes Muscle, soft tissue Upper extremity Interphalangeal webbing Flexion of fingers when dorsiflexed (pseudocamptodactyly) Shortening of flexor profundus muscle–tendon unit Lower extremity Short gastrocnemius Shortening of various muscle– tendon groups in legs and feet Soft tissue syndactyly of toes Wallender, Ahson, and Steinberg. Early Diagnosis and Treatment of Neonatal Coronoid Hyperplasia. J Oral Maxillofac Surg 2015.
Aside from the novelty of our case, we believe that the presentation of our patient highlights many important clinical issues noted in published studies. For example, in our review, the most objective method to evaluate coronoid hyperplasia described is to measure the coronoid/condylar volume and surface area ratios.14 Although we had 3-dimensional CT imaging scans to evaluate the hyperplasia, we did not possess the postprocessing software to quantify these ratios. For clinicians who treat pediatric patients on a routine basis, we do not believe this measurement is necessary. The findings from a physical examination and CT scan will be sufficient to diagnose coronoid hyperplasia.
In addition, it is important to note that clinical problems can arise from the potential sequelae of limited mouth opening. Acutely, these problems can range from feeding difficulty and potential malnutrition to airway compromise causing apnea and emergent airway compromise. Neonates with trismus are also reported to have difficulty clearing secretions, causing more frequent aspirations. Our patient was experiencing all these issues at the diagnosis. The long-term consequences of trismus can include growth restriction due to malnutrition, speech delay, muscle contracture and atrophy, facial asymmetry, and the risk of infection owing to poor oral hygiene. We believe that prompt diagnosis and treatment is essential to prevent these acute and long-term consequences. Most importantly, our treatment of this neonate highlights the airway risk associated with neonatal trismus. Whether isolated or appearing in conjunction with syndromic findings, most patients appear to have airway abnormalities. In our patient, multiple anesthesia practitioners had difficulty visualizing the tracheolaryngeal anatomy. Even with fiberoptic capabilities, our anesthesia colleagues had difficulty with visualization and management of our patient’s airway. The published studies have reported various approaches to managing the airway in patients with TPS ranging from mask anesthesia with spontaneous ventilation and the avoidance of muscle relaxants to blind nasoendotracheal intubation.15-17 In our patient, endotracheal intubation became increasingly more difficult as the child grew older, likely because of the worsening trismus. It is unclear whether this situation was similar in other cases. Although multiple sequelae can result from neonatal trismus, surgical treatment tends to be delayed by late diagnosis and referral. Once diagnosed and treated, physical therapy was frequently attempted, with minimal improvement.4 In the published studies, all reported patients eventually underwent bilateral coronoidectomy, with the intraoral approach the most common.1-7 Regarding coronoid hyperplasia, the youngest patient treated with surgery reported in the published data was 8 months old.4 In the few cases reported, surgical treatment has been prone to relapse, and the trismus often recurs. Postoperative physical therapy will help to minimize recurrence and allows for sustained improvement in the jaw range of motion. If surgical correction can be accomplished at an early age with the combination of jaw physiotherapy, we believe such children have the potential for permanent normalization of jaw mobility. Patients who underwent surgical correction at older ages underwent physical therapy with poor compliance. This was associated with greater rates of relapse. The published data have shown that compliance with physical therapy has a bimodal distribution.1 Patients
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younger than 2 years old and older than 16 years have reportedly had better results. Our patient is currently the youngest reported patient to have undergone this therapy. His postoperative result was maintained for months after surgery without the need for more aggressive therapy. Long-term management and surveillance of this patient population is of paramount importance because of the risk of relapse. Lefaivre and Aitchison6 attributed the absence of relapse in the treatment of TPS to continuous jaw physiotherapy, independent of the surgical technique. When comparing physical therapy options, they achieved a greater opening after 12 months of passive stretching of the jaw using tongue blade therapy. They noted that long-term therapy might be the most important variable affecting the long-term outcomes and advised waiting until patient cooperation would be optimal to otherwise avoid necessary intervention. Hirano et al9 also noted the importance of postoperative manipulation after initial surgery in achieving normal mouth opening, because the TMJ might have been restricted in its movement during the fetal period. In any form of trismus, it is important to have close follow-up to monitor the patient’s postoperative progression. Because the incidence of relapse is significant in these patients, it is important to discover relapse early to make modifications to the physical therapy or perform another operative procedure. If the trismus recurs and is left untreated, the aforementioned complications could occur, and the patient will be at an increased risk of ankylosis. Our patient presented with multiple congenital abnormalities, not described for any particular spectrum or syndrome. It remains unknown if these resulted from a new pathologic process or a worsening spectrum of syndromes already presented in the scientific data. Regardless, after our patient underwent surgery at 90 days of age, we were able to significantly increase his mouth opening and maintain the result with physiotherapy. Although more research is needed, we believe that early treatment would benefit these patients and can be performed safely and effectively.
Uncited References 18, 19, 20, 21, 22, 23.
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2. Carlos R, Contreras E, Cabrera J: Trismus-pseudocamptodactyly syndrome (Hecht–Beals’ syndrome): Case report and literature review. Oral Dis 11:186, 2005 3. Costello BJ, Edwards SP: Pediatric mandibular hypomobility: Current management and controversies. Oral Maxillofac Surg Clin North Am 17:455, 2005 4. Fabie L, Boutault F, Gas C, Paoli J-R: Neonatal bilateral idiopathic hyperplasia of the coronoid processes: Case report. J Oral Maxillofac Surg 60:459, 2002 5. Karras SC, Wolford LM: Trismus-pseudocamptodactyly syndrome: Report of a case. J Oral Maxillofac Surg 53:80, 1995 6. Lefaivre J-F, Aitchison MJ: Surgical correction of trismus in a child with Hecht syndrome. Ann Plast Surg 50:310, 2003 7. Visscher SH, Schortinghuis J, Bos RRM: Congenital mandibular hypomobility: A rare condition with little consensus—A case report. J Oral Maxillofac Surg 67:444, 2009 8. Puche M, Guijarro-Martınez R, Perez-Herrezuelo G, et al: The hypothetical role of congenital hypotonia in the development of early coronoid hyperplasia. J Craniomaxillofac Surg 40:e155, 2012 9. Hirano A, Iio Y, Murakami R, et al: Recurrent trismus: Twentyyear follow-up result. Cleft Palate Craniofac J 31:309, 1994 10. Turk A, McCarthy J, Nichter L, Thorne C: Moebius syndrome: The new finding of hypertrophy of the coronoid process. J Craniofac Surg 10:93, 1999 11. Fryssira H, Makrythanasis P, Kattamis A: Severe developmental delay in a patient with 7p21.1–p14.3 microdeletion spanning the TWIST gene and the HOXA gene cluster. Mol Syndromol 2: 45, 2011 12. Cai T, Yu P, Tagle DA, Xia J: Duplication of 7p21.2/pter due to maternal 7p;21q translocation: Implications for critical segment assignment in the 7p duplication syndrome. Am J Med Genet 86: 305, 1999 13. Papadopoulou E, Sifakis S, Sarri C: A report of pure 7p duplication syndrome and review of the literature. Am J Med Genet Part A 140A:2802, 2006 14. Chang C, Allori A, Wang E, Farina R: A quantitative threedimensional analysis of coronoid hypertrophy in pediatric craniofacial malformations. Plast Reconstr Surg 129:312e, 2012 15. Nagata O, Tateoka A, Shiro R, Kimizuka M: Anaesthetic management of two paediatric patients with Hecht–Beals syndrome. Paediatr Anaesth 9:444, 1999 16. Seavello J, Hammer GB: Tracheal intubation in a child with trismus pseudocamptodactyly (Hecht) syndrome. J Clin Anesth 11:254, 1999 17. Vaghadia H, Blackstock D: Anaesthetic implications of the trismus pseudocamptodactyly (Dutch-Kentucky or Hecht Beals) syndrome. Can J Anaesth 35:80, 1988 18. Callewaert BL, Loeys BL, Ficcadenti A, et al: Comprehensive clinical and molecular assessment of 32 probands with congenital contractural arachnodactyly: Report of 14 novel mutations and review of the literature. Hum Mutat 30:334, 2009 19. Gerbino G, Bianchi SD, Bernardi M, Berrone S: Hyperplasia of the mandibular coronoid process: Long-term follow-up after coronoidotomy. J Craniomaxillofac Surg 25:169, 1997 20. Minzer-Conzetti K, Wu E, Vargervik K, Slavotinek A: Phenotypic variation in trismus-pseudocamptodactyly syndrome caused by a recurrent MYH8 mutation. Clin Dysmorphol 17:1, 2008 21. Nordone TP, Li P: Arthrogryposis multiplex congenita in association with bilateral temporomandibular joint hypomobility: Report of a case and review of literature. J Oral Maxillofac Surg 68:1197, 2010 22. Stopa Z, Wanyura H, Kowalczyk P: Coronoid-condylar index in assessing of mandibular coronoid hyperplasia: Preliminary results. Adv Med Sci 58:429, 2013 23. Zhong S-C, Xu Z-J, Zhang Z-G, et al: Bilateral coronoid hyperplasia (Jacob disease on right and elongation on left): Report of a case and literature review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 107:e64, 2009
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