A Novel Nonoperative Technique in the Initial Management and Treatment of Congenital Microstomia

Transcription

1 CASE REPORTS A Novel Nonoperative Technique in the Initial Management and Treatment of Congenital Microstomia Kristen M. Rezak, M.D., Amy Bouvier, D.M.D., Brian Olack, M.D., Jose C. Larumbe, D.D.S., M.S., Eric Stelnicki, M.D., F.A.C.S. Congenital microstomia in the newborn can result in poor functional and aesthetic outcomes. In the past, treatment options have included surgical methods such as commissurotomies and z-plasties as well as nonsurgical treatments using oral splints. In severe microstomia, a surgical release may be required to permit splint placement. Because of the small diameter of the stoma, such surgical releases are not optimal and frequently must be repeated. We devised a nonoperative technique for the initial treatment of congenital microstomia that will slowly enlarge the stomal diameter without the need for surgical release. The patient is a newborn male born with multiple congenital anomalies including severe mandibular hypoplasia and retrusion, microstomia, and microglossia. The size of his oral cavity was less than 1 cm at birth with no clinical signs of a tongue. After initial dilation in the operating room with Hagar dilators, the oral stoma was serially dilated using Boston Scientific esophageal balloons. Once full dilation was achieved, we fabricated oral splints to continue the dilation process while maintaining oral competence with no surgical intervention. KEY WORDS: commissure, congenital, craniofacial, Freedman Sheldon, microglossia, microstomia, Pierre Robin, stoma, Treacher Collins Congenital microstomia is a rare condition that occurs in newborns and is usually associated with other congenital anomalies. In severe cases, microstomia can result in poor functional and aesthetic outcomes. Because of the rarity of the condition, management of congenital microstomia stems mostly from experience in treating burn patients who develop microstomia. In the past, treatment options have included surgical methods such as commissurotomies and z-plasties as well as nonsurgical treatments using oral splints (Guyuron and Winkler, 1988; Ferreira et al., 1994). In severe microstomia, a surgical release may be required to permit splint placement (Heinle et al., 1988; Koymen et al., Dr. Rezak is Plastic Surgery Resident, Department of Plastic Surgery, Cleveland Clinic Florida, Weston, Florida. Dr. Bouvier is Craniofacial Surgery Fellow, Department of Plastic Surgery, Cleveland Clinic Florida, Weston, Florida. Dr. Olack is Plastic Surgery Resident, Department of Plastic Surgery, Cleveland Clinic Florida, Weston, Florida. Dr. Larumbe is Pediatric Dentist and Assistant Professor, Department of Pediatric Dentistry, Nova Southeastern University, College of Dental Medicine, Davie, Florida. Dr. Stelnicki is Plastic and Reconstructive Craniofacial Surgeon, Adjunct Faculty, Cleveland Clinic Florida Plastic Surgery Residency, Joe DiMaggio Children s Hospital, Cleft and Craniofacial Surgery Center, Hollywood, Florida. Submitted July 2010; Accepted April Address correspondence to: Dr. Kristen M. Rezak, Department of Plastic Surgery, Cleveland Clinic Florida, 2950 Cleveland Clinic Blvd., Weston, FL rezakk@ccf.org. DOI: / ). Because of the small diameter of the stoma, such surgical releases are not optimal and frequently must be repeated. Micrognathia, usually associated with congenital microstomia, must first be corrected because of the difficulty in airway management. Frequently, this is done with placement of an external mandibular distraction device (Pons and Bouhours, 1969; Boraz et al.,1985). Microstomia can then be treated once the airway is secured, usually via a tracheostomy, with the mandibular distraction device in place. Treatment is undertaken at an early age because of the difficulty in feeding, oral hygiene problems, and speech disorders that are associated with microsomia. We devised a novel nonoperative technique for the initial treatment of severe congenital microstomia that will slowly enlarge the stomal diameter without the need for surgical release. We describe a case and the clinical management of a newborn male born with multiple congenital anomalies including severe mandibular hypoplasia and retrusion, microstomia, microglossia, and ventricular septal defect. The size of his oral cavity was less than 1 cm at birth with no clinical signs of a tongue. CASE REPORT The patient is a newborn fraternal twin boy born by cesarean delivery at 34 weeks, 5 days gestation alongside a healthy female twin sister (Fig. 1). The mother, who was 352

2 Rezak et al., INITIAL MANAGEMENT AND TREATMENT OF CONGENITAL MICROSTOMIA 353 FIGURE 1 Patient predilation. also in good health, did not have any perinatal complications. The patient was born with multiple congenital anomalies and experienced severe respiratory distress due to mandibular retrusion and airway obstruction at birth. An emergency tracheostomy was performed in the delivery room. Craniofacial computed tomography showed normal brain parenchyma, micrognathia, and small lingual remnant at the base of the oral cavity and no evidence of a cleft palate, as seen in Figure 2. To facilitate feeds, a gastrostomy tube was subsequently placed. Mandibular distraction osteogenesis was performed at 1 month with placement of external distraction devices (Boraz et al., 1985). Distraction was necessary to align the mandibular and maxillary alveolus prior to placement of the expansion device. Without the alveolar support, the soft tissue alone would not retain the expander, and the expander would literally fall out. Our initial assessment of the microstomia showed that the oral sphincter mechanism appeared intact and could potentially be dilated with balloon dilation. Measurement of the intercommissural distance was 6 mm. The patient was taken to the operating room for the first stage of dilation. Endoscopy was performed revealing a small tongue bud and no other abnormalities. We proceeded with the initial dilation by using Hagar dilators to stretch the oral sphincter and then followed with the placement of a 15- mm Boston Scientific esophageal balloon and secured it with Steri-Strips (Fig. 3). The balloon was placed approximately half way into the oropharynx and expanded to a size of 10 mm, while carefully avoiding any dilation into the pharyngeal tissue. The patient was sent back to the neonatal intensive care unit for close observation. Serial expansion of the balloon was performed over the next week until the balloon was dilated to 15 mm. The balloon was secured to the patient using Logan Bars, a neonatal endotracheal tube holder, after several dislodgments occurred with tape(fig. 4). The plastic surgery team inflated the balloon daily and also checked the oral mucosa twice daily for any mucosal breakdown. The inflation limit was to the point of oral FIGURE 2 Computed tomography scan three-dimensional reconstruction. A: Lateral facial view. B: Skeletal view. mucosal blanching. Prior to increasing the tension and diameter of the balloon inflation, the balloon was deflated once daily to suction any oral secretions as well as to check for any pressure necrosis or tears on the oral mucosa (Heinle et al., 1988). Once full inflation of the 15-mm balloon occurred, we changed balloons to the 18-mm balloon and continued with the same protocol. The time to dilation with each balloon was 1 week to reach full inflation. After 2 weeks, the 18-mm esophageal balloon was fully dilated and was replaced with a 25-mm balloon. The 25-mm balloon was fully inflated after 1 week and remained in place until the oral splint was available.

3 354 Cleft Palate Craniofacial Journal, May 2012, Vol. 49 No. 3 FIGURE 3 Hagar dilation intraoperatively. Two oral splints were designed and fabricated by the pediatric dentistry team from Nova Southeastern University; the first splint was placed into the patient s oral cavity 1 month after the initial dilation began. Preliminary molds were based on the patient s pacifier, and modifications were made to allow proper position in the mouth with the posteriorly displaced lower lip due to the retrognathia (Fig. 5). At the 1-month follow-up visit, the mandibular distraction device was removed, and the first oral splint was replaced with a second static-dynamic oral splint that included distraction capabilities (Fig. 6). A Leone Rapid Palatal Expansor screw was incorporated into the second oral splint. This screw was activated 1 mm per week for the first month and 2 mm per week after that. The screw is able to open up to a maximum of 13 mm, which will be able to continue the dilation process. The final intercommissural distance measured just prior to placement of the second splint was 20 mm (Fig. 7; Heinle et al., 1988). Final measurements and long-term results could not be obtained, as the patient unfortunately died due to complications from FIGURE 5 Oral splint. A: Initially fabricated static oral splint with design based on a pacifier. B: First modification to allow for better lip position. C: Second modification to accommodate the mandibular retrusion. the tracheostomy. Further intervention for our patient would have required additional mandibular distraction placement as well as a possible surgical flap for tongue reconstruction (Boraz et al., 1985; Yamada et al., 2000). DISCUSSION FIGURE 4 Balloon dilation with Logan Bar. Congenital microstomia has not been well described in the literature. This is in part due to its association with other craniofacial syndromes that occur with a very low incidence.

4 Rezak et al., INITIAL MANAGEMENT AND TREATMENT OF CONGENITAL MICROSTOMIA 355 FIGURE 7 Patient postdilation. FIGURE 6 A: Static-dynamic oral splint. B: Intraoral view. Microstomia, which is defined as an abnormally small mouth, is typically identified at birth. Initial treatment involves securing an adequate airway with placement of a tracheostomy because of the difficulty in oral intubation with these patients (Temperley and Walker, 1995). Further management entails attempting to widen the oral stoma. This can be done either surgically or with the use of oral splints (Pons and Bouhours, 1969; Heinle et al., 1988). Adequate stomal size is needed for oral hygiene, facial expression, speech, and proper nutrition intake (Escoda- Francolí et al., 2009). Because of the rarity of the condition, most of the information regarding treatment options for congenital microstomia has been obtained from treatment of facial burns and scleroderma patients (Hartford et al., 1975; Ortiz- Monasterio and Factor, 1980; Naylor et al., 1984; La Trenta et al., 1992). Congenital microstomia occurs in combination with syndromes such as Treacher Collins, Pierre Robin, or Freedman Sheldon (Guyuron and Winkler, 1988; Ferreira et al., 1994; Escoda-Francolí et al., 2009). Because of the complexity of the genetic abnormalities associated with these syndromes, the treatment of microstomia usually involves concomitant management with other genetic anomalies. To devise a technique that would allow for sufficient correction of the microstomia, we first analyzed all of the original studies that included the treatment options available. Most of the techniques that are used to correct microstomia involve a surgical release or some type of surgical flap to enlarge the stomal opening. Surgical correction has included a wide range of procedures such as commissuroplasties, commissurrotomies, z-plasties, and stair-step lengthening of the orbicularis oris (Pons and Bouhours, 1969; Guyuron and Winkler, 1988; Ferreira et al., 1994; Mehra et al., 1998; Langstein and Robb, 2005). Using the balloon technique to correct the microstomia will allow the native tissue to remain intact and possibly avoid the functional impairment and scarring associated with surgical treatments. This technique also maintains the integrity of the oral sphincter. Previous descriptions using oral commissure splints to correct or prevent microstomia have been described, but they do not address the problem of making the oral stoma wide enough to place the splint (Heinle et al., 1988; Taylor and Walker, 1997). Our dilation technique to correct the microstomia can be done concomitantly with other procedures, such as mandibular distraction, and allows for future placement of the oral splint without a surgical procedure to increase the diameter of the oral stoma. The dilation process using the esophageal balloon proceeded as planned; however, we had difficulty in securing the balloon due to the micrognathia. Using tape alone, the balloon would slip out inferiorly due to the retrusion of the mandible. The Logan Bar was able to secure the balloon in place without causing any additional trauma to the patient such as with suture ligation or a prosthesis/immobilization. Once secured properly, the plastic surgery team inflated the balloon daily until the final diameter of 25 mm was achieved. The final diameter distance was based on the normal intercommissural size for an infant male at 1 year of age of 32 mm as documented by Cervenka et al. (1969). To maintain the dilation, the dental

5 356 Cleft Palate Craniofacial Journal, May 2012, Vol. 49 No. 3 team was consulted to fabricate an oral splint. This was a challenge for the dental team because the microstomia splints that they have used in the past would not work for our patient (Dougherty and Warden, 2003). The first oral splint fabricated was used to maintain the same oral diameter as previously made by the final balloon dilation. Modifications were made, and the dental team designed a dynamic oral splint that would allow for a slow dilation without having to fabricate a new splint each time. The length of time required to use the splint was not able to be determined for our patient because of his unexpected early demise. CONCLUSION Congenital microstomia can be treated with a safe, nonoperative treatment option that will allow dilation to the desired stomal opening. The overall goal is to maintain and restore as much functional normalcy of the oral stoma as possible. Using an esophageal balloon, we devised a treatment plan to slowly expand the oral diameter and allow for placement of a dynamic oral splint without performing a surgical procedure. The outcome would have depended on the compliance of the patient s caretakers in continued use of the splint. REFERENCES Boraz RA, Hiebert JM, Thomas M. Congenital micrognathia and microglossia: an experimental approach to treatment. ASDC J Dent Child. 1985;52(1): Cervenka J, Figalová P, Gorlin RJ. Cranio-carpo-tarsal dysplasia or the whistling face syndrome: II. Oral intercommissural distance in children. Am J Dis Child. 1969;117: Dougherty ME, Warden GD. A thirty-year review of oral appliances used to manage microstomia, 1972 to JBurnCareRehabil. 2003;24: Escoda-Francolí J, Sánchez-Garcés MA, Gay-Escoda C. Oral implant rehabilitation in a patient with Moebius syndrome. Med Oral Patol Oral Cir Bucal. 2009;14:E295 E298. Ferreira LM, Minami E, Andrews Jde M. Freeman-Sheldon syndrome: surgical correction of microstomia. Br J Plast Surg. 1994;47: Guyuron B, Winkler PA. Craniocarpotarsal dysplasia: the whistling face syndrome. Ann Plast Surg. 1988;20: Hartford CE, Kealey GP, Lavelle WE, Buckner H. An appliance to prevent and treat microstomia from burns. J Trauma. 1975;15: Heinle JA, Kealey GP, Cram AE, Hartford CE. The microstomia prevention appliance: 14 years of clinical experience. J Burn Care Rehabil. 1988;9: Koymen R, Gulses A, Karacayli U, Aydintug YS. Treatment of microstomia with commissuroplasties and semidynamic acrylic splints. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009;107: La Trenta GS, Grant RT, Haworth RD, Madden M, Hoffman LA. Functional reconstruction for severe postburn microstomia. Ann Plast Surg. 1992;29: Langstein HN, Robb GL. Lip and perioral reconstruction. Clin Plast Surg. 2005;32: Mehra P, Caiazzo A, Bestgen S. Bilateral oral commissurotomy using buccal mucosa flaps for management of microstomia: report of a case. J Oral Maxillofac Surg. 1998;56: Naylor WP, Douglass CW, Mix E. The nonsurgical treatment of microstomia in scleroderma: a pilot study. Oral Surg Oral Med Oral Pathol. 1984;57: Ortiz-Monasterio F, Factor R. Early definitive treatment of electric burns of the mouth. Plast Reconstr Surg. 1980;65: Pons J, Bouhours G. Treatment of microstomia [in French]. Rev Stomatol Chir Maxillofac. 1969;70: Taylor L, Walker J. A review of selected microstomia prevention appliances. Pediatr Dent. 1997;19: Temperley A, Walker P. Blind nasal intubation by monitoring capnography in a neonate with congenital microstomia. Anaesth Intensive Care. 1995;23: Yamada A, Konno N, Imai Y, Saitou C, Kochi S. Treatment of hypoglossia-hypodactyly syndrome without extremeity anomalies. Plast Reconstr Surg. 2000;106: