AR Buche

DD Alfery

JK Tomlinson

MG Gartlan

R Bali

Smriti Panda

Kapil Sikka

Jyotsna Punj

Suresh C. Sharma

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CASE REPORT Open AccessBilateral congenital alveolar synechiae—arare cause of trismusSmriti Panda1*, Kapil Sikka1, Jyotsna Punj2 and Suresh C. Sharma1AbstractCongenital alveolar synechiae is a rare anomaly mostly presenting in association with cleft palate. Owing to reducedmouth opening, feeding difficulties, and compromised airway in extreme cases along with presentation in early neonatalperiod, these patients present unique challenges to the surgeon as well as the anesthetist. Here, we discuss the surgicaland anesthetic management of this entity in a 12-month-old female child.BackgroundBilateral congenital alveolar synechiae is a rare clinicalentity frequently described in association with othercraniofacial anomalies [1]. This condition is recognizedin the early neonatal period due to feeding difficulties,restricted mouth opening, and in extreme cases, presentingwith respiratory distress. Early surgical intervention istherefore warranted to address the neonates’ nutritionalneeds and optimal growth. The surgical, anesthetic, andairway challenges that these patients present with havebeen reviewed in this case report.Case presentationA 1-year-old female was referred to our department withcomplaints of decreased mouth opening since birth. Thechild was born out of a non-consanguineous marriage.The mother had a full-term vaginal delivery. Birth weightwas 2.5 kg. Apgar scores were unknown. The perinatalperiod was uneventful. The clinician attending the child,however, made note of the reduced mouth opening andthe presence of bands adhering the upper and loweralveolus. Family history was also not contributory. Thechild attained age appropriate milestones and had noother systemic manifestations. The patient had no feedingor breathing difficulties and sought medical help forreduced mouth opening at the age of 12 months.On examination, there were no dysmorphic features.Cardiovascular and neurological assessments were normal.No other musculoskeletal anomalies were found. Detailedoral cavity examination could not be performed due to tris-mus. Evaluation of oral cavity was performed with a nasalendoscope and two fibrous bands could be identifiedbetween upper and lower alveolus of thickness of 3 to4 mm approximately, about 3 mm from the oral com-missure on the left and 5 mm from the commissure onthe right (Fig 1). The patient also had a concomitantbifid uvula. Bony ankylosis of temporomandibular jointwas ruled out on computed tomography.Operative detailsPatient was planned for laser release under generalanesthesia. Owing to trismus, fiberoptic naso trachealintubation was planned with an uncuffed 4-mm endo-tracheal tube. The child was premedicated with xylome-tazoline nasal drop (Otrivin 0.1 % w/w, Novartis, India)and injection glycopyrrolate (Vagolate 0.2 mg, 0.2 mg/ml,Abbott Health Care Pvt Ltd, India). Nasotracheal intub-ation was done with a size 4 uncuffed endotracheal tubewith flexible endoscopic guidance, and after confirmationof correct position of the endotracheal tube, muscle re-laxation was achieved with atracurium (Artacil—100,0.9 % /10 mg /10 ml, Neon Laboratories Ltd, India).Nasopharynx and larynx were found to be normal anddevoid of any fibrous bands or synechiae. Under endo-scopic guidance, the oral synechiae were released withdiode laser, 980 nm (ARC Diode Laser, FOX 10 W,Nurnberg, Germany) pulsed mode at 7.5 W power(Fig. 2). After release, a Boyle Davis mouth gag couldbe applied and a 3-cm mouth opening could beachieved (Fig. 3).* Correspondence: smriti.panda.87@gmail.com1Department of ENT, Teaching Block, All India Institute of Medical Sciences,4th Floor, East Ansari Nagar, New Delhi 110029, IndiaFull list of author information is available at the end of the article© 2016 Panda et al. Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, andreproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link tothe Creative Commons license, and indicate if changes were made.Panda et al. Maxillofacial Plastic and Reconstructive Surgery  (2016) 38:8 DOI 10.1186/s40902-016-0056-2Patient had an uneventful postoperative recovery. Oralfeeds were initiated immediately after recovery fromanesthesia and was tolerated well by the patient.DiscussionCongenital intraoral synechiae is a rare congenital anomalywith approximately 60 cases documented in the literature[2]. It rarely presents in isolation. Of the 50 cases reportedby Gartlan [3], only seven patients presented with iso-lated alveolar fusion. Associated anomalies include Vander Woude syndrome, popliteal pterygium syndrome,and orofacial digital syndrome [3]. The most commonassociation is with cleft palate (cleft palate lateral syne-chiae syndrome) [1, 3].These patients present in the early neonatal period dueto restriction of mouth opening interfering with feedingand airway [1, 2, 4]. Our patient, however, presented latedue to absence of gross craniofacial anomalies, absence ofsevere trismus owing to posterior location of the bands.Bifid uvula was the only concomitant anomaly found.Various theories have been put forth to explain the as-sociation between cleft palate and intraoral synechiae.The most convincing is the sequence of cleft palate pre-disposing to increased mucosal contact between tongueand developing alveolus [1]. Other possible mechanismsFig. 1 Preoperative findings. a Preoperative mouth opening and presence of a single central incisor. b Endoscopic view showing presence ofsynechiae on the left sideFig. 2 Intraoperative image. a Diode laser being used to release synechiae. b Minimal damage to mucosa after application of laser with limited charringPanda et al. Maxillofacial Plastic and Reconstructive Surgery  (2016) 38:8 Page 2 of 4like persistence of buccopharyngeal membrane and localischemia of amniotic bands causing pressure on the firstbranchial arch also explain the rare occurrence of iso-lated alveolar synechiae. Airway management presentsunique challenges to the anesthesia team. Blind nasal in-tubation was attempted in the past [5]. Tracheostomy is,generally, reserved as a last resort in these patients withonly one article quoting the need for tracheostomy in a38-week-old male infant presenting with marked desa-turations [3]. Nasotracheal intubation with flexiblefiberoptic bronchoscope as performed in our case isconsidered the gold standard [1, 5].Treatment consists of dividing the bands as early asthe child’s general condition permits to optimize feeding,craniofacial development, and prevent fibrous ankylosisdue to disuse. Diode laser has been used in our patientto minimize blood loss, post operative pain, prevent col-lateral damage, and hence hasten the healing processand prevent reformation of the adhesions.ConclusionsTrismus in a neonate presents unique challenges to thetreating physician for airway and feeding management.Timely referral to oral and maxillofacial center withgood anesthetic expertise can circumvent the problemsof failure to thrive and poor development. Flexible fiber-optic intubation can avoid the additional morbiditycaused by tracheostomy in such young infants. The useof diode laser not only reduced surgical time but alsoplayed a significant role in expediting healing processand helped in early initiation of oral feeds.ConsentWritten informed consent was obtained from the patientfor publication of this case report and any accompanyingimages. A copy of the written consent is available forreview by the Editor-in-Chief of this journal.Competing interestsThe authors declare that they have no competing interests.Authors’ contributionsSP prepared the manuscript, assisted the procedure, and did the patientwork-up and follow-up. KS and SCS are the main operating surgeons.JP was involved with the anesthetic and airway management. All authorsread and approved the final manuscript.AcknowledgementsThe authors thank the participant for the informed consent to share the resultsof this procedure. SP thanks Shradha Panda and Praveen Dhanalakota for theirexcellent processing of clinical photography.Author details1Department of ENT, Teaching Block, All India Institute of Medical Sciences,4th Floor, East Ansari Nagar, New Delhi 110029, India. 2Department ofAnesthesiology, Teaching Block, All India Institute of Medical Sciences, 5thFloor, East Ansari Nagar, New Delhi 110029, India.Fig. 3 Postoperative mouth opening. After synechiae release, Boyle Davis mouth gag could be insertedPanda et al. Maxillofacial Plastic and Reconstructive Surgery  (2016) 38:8 Page 3 of 4Received: 9 January 2016 Accepted: 15 February 2016References1. Tomlinson JK, Liam NT, Savarirrayan R, Meara JG (2006) Isolated andsyndromic syngnathia: management, implications and genetics. Ann PlastSurg 57:231–2352. Buche AR, Borade VR, Suman N, Chintale S, Jaiswal SA (2014) Congenitaloral synechiae. A case report and review. Int J Rec Trends Sc Technol10:184–1863. Gartlan MG, Davies J, Smith RJ (1993) Congenital oral synechiae. Ann OtolRhinol Laryngol 102(186):1974. Bali R, Sharma P, Jain S, Thapar D (2010) Congenital fibrousmaxillomandibular fusion. J Maxillofac Oral Surg 9:271–2795. Alfery DD, Ward CF, Harwood IR, Mannino FL (1979) Airway managementfor a neonate with congenital fusion of the jaws. Anesthesiol 51:340–342Submit your manuscript to a journal and beneﬁ t from:7 Convenient online submission7 Rigorous peer review7 Immediate publication on acceptance7 Open access: articles freely available online7 High visibility within the ﬁ eld7 Retaining the copyright to your article    Submit your next manuscript at 7 springeropen.comPanda et al. Maxillofacial Plastic and Reconstructive Surgery  (2016) 38:8 Page 4 of 4

Maxillofacial Plastic and Reconstructive Surgery

Bilateral congenital alveolar synechiae—a rare cause of trismus

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Bilateral congenital alveolar synechiae—a rare cause of trismus

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