Cleft Maxillary Hypoplasia: Comparison of Techniques and Proposal of a Novel Treatment Protocol for Management.

This study was designed to compare different techniques of managing cleft maxillary hypoplasia and to propose a treatment protocol taking patient's age of presentation, amount of maxillary hypoplasia, and presence/absence of velopharyngeal insufficiency (VPI) into consideration. Five treatment modalities, viz. facemask therapy (Group I), anterior maxillary distraction (Group II), total maxillary distraction osteogenesis using rigid external distraction devices (Group III) and internal distraction devices (Group IV), and conventional orthognathic surgery (Group V), were assessed retrospectively using lateral cephalograms (taken at 3 intervals) and speech records (studied pre- and post-operatively). The results were subjected to statistical analysis. A P value of less than 0.05 was considered statistically significant. The mean advancement achieved in groups I, II, III, IV, and V was 4.2±1.54, 9.03±2.62, 11.82±1.18, 10.41±1.42, and 7.24±2.44 mm, respectively. The mean horizontal relapse noted in Groups I, II, III, IV, and V was 2.3 mm (n=8), 2.1 mm (n=14), 3.4 mm (n=10), 1.4 mm (n=5), and 2.4 mm (n=24), respectively. Compared to other groups, group II had fewer patients exhibiting relapse, a statistically significant observation. Similarly, statistically significant improvement in speech outcomes was found only in group II compared to other groups. Based on the results, a treatment protocol was proposed. The authors conclude that the proposed protocol would enable surgeons to decide the most appropriate treatment modality paying attention to not only the age of presentation and amount of advancement but also presence/absence of VPI which is instrumental in deciding upon the most appropriate treatment.

Giant Juvenile Xanthogranuloma of face in an adult: A rare complexity.

Juvenile xanthogranuloma, a form of non-Langerhans cell histiocytosis can be defined as the proliferation of cells with macrophage like characteristics. It has been described as a benign, asymptomatic and common self-healing disorder of non-Langerhans cell histiocytosis (LCH), affecting mostly infants, children and rarely adults. We have documented a case of a 40 year old male who presented to us with extensive insidious papulonodulous growth over the face. The facial disfigurement caused was catastrophic. An extensive surgical excision and reconstruction was performed followed by histopathological evaluation. Microscopic study and immune histochemistry revaled Juvenile Xanthogranuloma of adult. The article highlights the presentation, diagnosis and management of this mammoth, rare disease.

The Silicone Turkish Delight for Dorsal Augmentation in Rhinoplasty.

ABSTRACT: Rhinoplasties are the most challenging facial aesthetic procedures with the majority of the cases requiring dorsal augmentation either in the form of autogenous or alloplastic materials. Although autogenous grafts are the gold standard, these grafts have their innate issues such as donor site morbidity, occasional displacement, resorption, tendency to warp or develop sharp edges over time with increased perceptibility and clinical visibility of the graft with time. These issues were addressed by Erol in 2000 who advocated the use of finely diced autologous cartilage wrapped in a monolayer of surgical and termed it Turkish delight. This technique has been extensively modified by surgeons across the world with an attempt to forgo the use of autogenous grafts preventing donor site morbidity and replacing it with alloplastic materials. Silicon's inert nature, resorption resistance, absence of a secondary surgical site and economical value make it a widely utilized allograft augmentation option in Asian countries. The authors hereby present a modification of the original Turkish delight technique by utilizing diced silicone pieces amalgamated with 1 to 2 ml of autologous blood and wrapped in monolayer of surgicel (oxidized cellulose). The technique produced clinically stable results in 27 patients who were followed up for a period of 24 to 60 months (average 30 months). Improved pliability, negated risk of graft extrusion with no donor site morbidity are the key advantages of this technique over conventional autologous grafts and silicon block augmentation techniques. These results point towards the viability of the technique for performing dorsal augmentation in rhinoplasty.

A retrospective analysis of complications associated with tooth-borne anterior maxillary distraction for managing cleft maxillary hypoplasia: A 12-year experience.

PURPOSE: To evaluate the intra- and post-operative complications of tooth-borne anterior maxillary distraction in management of cleft maxillary hypoplasia over a 12-year period, and to establish whether the procedure involves a learning curve. MATERIALS AND METHODS: All patients with cleft maxillary hypoplasia satisfying the inclusion and exclusion criteria and undergoing anterior maxillary distraction with a tooth-borne palatal distractor between June 2007 to June 2018 were retrospectively studied by retrieving their individual medical records, which included radiographic (lateral cephalogram analysis at three different intervals) and speech assessment records. The presence of intra- and post-operative complications developing in this study period was noted. Furthermore, the frequency of intra- and post-operative complications occurring from years 2007-2018 were evaluated to study whether there was a decrease in the frequency of complications with increasing time. This was done to establish whether there was a learning curve associated with the surgical procedure. The results thus obtained were tabulated using SPSS 25.0 (SPSS Inc., Chicago, IL) and subjected to statistical analysis using the chi-squared test. A p value of less than 0.05 was considered statistically significant. RESULTS: Out of the 458 patients included in the study, 78.82 % (n = 361) had no complications. In the remaining 97 patients, a total of 137 complications (46 intra-operative and 91 post-operative) were observed, making up for a complication rate of 21.17%. The intra-operative complications noted were appliance dislodgement (n = 19), palatal mucosal tears (n = 12), hemorrhage (n = 8), dental injuries (n = 6) and inadvertent fracture of the anterior segment (n = 1). The post-operative complications noted were anterior open bite (n = 24), gingival inflammation (n = 14), appliance dislodgement (n = 13), dental injuries (n = 10), relapse (n = 8), hemorrhage (n = 5), soft tissue infections (n = 4), palatal fistula (n = 3), speech impairment (n = 3), wound dehiscence not related to infection (n = 3), need for re-operation (n = 2), avascular necrosis of the anterior segment (n = 1) and maxillary sinusitis (n = 1). A statistically significant decrease (p < 0.001) in the frequency of complications with each passing year from 2007 to 2018 was observed. Most complications were seen to occur in the first 4 years (i.e. 2007-2010; a total of 74 complications in 54 patients), making up 11.79% of the complications. In the following 8 years (i.e. 2011-2018), 63 complications were noted in 43 patients, making up the remaining 9.38 % of the complications. These observations indicate that a learning curve was involved before carrying out the procedure without many complications. CONCLUSION: Tooth-borne anterior maxillary distraction in managing cleft maxillary hypoplasia is associated with a wide range of complications: some avoidable and related to surgeon's experience; some related to the surgical procedure itself; and some unavoidable and related to the distractor appliance. Understanding potential complications allows the surgeon to ensure safe care through early intervention and to correctly inform the patient in the pre-operative discussion. The procedure involves a learning curve before it can be accomplished safely without many complications.

Modified two flap palatoplasty in asymptomatic transsphenoidal encephalocele: a case report.

About one-third of patients with transsphenoidal basal encephaloceles have associated congenital anomalies, including cleft palate. Moreover, they are often plagued by symptomatic exacerbations in the form of upper respiratory obstructions, cerebrospinal fluid leaks, meningitis, etc., with few patients being asymptomatic. We herein present a rare asymptomatic case of transsphenoidal basal encephalocele in an 18-month-old child with cleft palate and highlight a modified version of two-flap palatoplasty.

A comprehensive management protocol to treat cleft maxillary hypoplasia.

AIM: To describe a comprehensive management protocol to treat cleft maxillary hypoplasia specific to the patient's age, degree of hypoplasia and presence or absence of velopharyngeal incompetence (VPI). MATERIALS AND METHODS: A total of 359 patients suffering from cleft maxillary hypoplasia were retrospectively studied from January 2004 till June 2015. Lateral cephalograms were taken to assess the degree of deformity and advancement achieved at three intervals. Patients were treated by four treatment modalities: Facemask therapy, Anterior maxillary advancement, Total maxillary osteogenesis and LeFort I advancement. RESULTS: 359 patients of cleft maxillary hypoplasia were treated and followed up for a mean of 25 ± 3.6 months. Group I had 20 patients, all in the pre-pubertal age group with less than 11 mm discrepancy. 25% of patients in this group had relapse; Group II: 196 patients who were all above 11 years of age and included all grades of hypoplasia, only 6.25% patients had relapse; Group III had 36 patients all with severe form of hypoplasia and were above 5 years of age. Relapse in this group was 16.6%; Group IV had 102 patients who were above 16 years of age and had mildmoderate severity of hypoplasia. Relapse rate was 18.75%. CONCLUSION: Extensive literature search and our institutional study has helped us formulate a protocol that delineates the most appropriate treatment modality for a specific age group and degree of hypoplasia also considering the effect of treatment on velopharyngeal incompetence.

Van der Woude syndrome presenting as a single median lower lip pit with associated dental, orofacial and limb deformities: a rare case report.

Although it is a rare developmental malformation, van der Woude syndrome is the most common form of syndromic orofacial clefting, accounting for approximately 2% of all cleft cases. The lower lip pits with or without a cleft lip or palate is characteristic of the syndrome. Findings, such as hypodontia, limb deformities, popliteal webs, ankylogossia, ankyloblepheron, and genitourinary and cardiovascular abnormalities, are rarely associated with the syndrome. This paper reports a rare case of van der Woude syndrome in a 10-year-old male patient with a single median lower lip pit and a repaired bilateral cleft lip and cleft palate that were associated with microstomia, hypodontia, and clubbing of the left foot with syndactyly of the second to fifth lesser toes of the same foot.

Amniotic constriction band: a report of two cases with unique clinical presentations.

Amniotic constriction band is a rare clinical entity with varied manifestations that range from a combination of congenital malformations to isolated malformations that are unique to each patient. The etiology of this entity remains unknown. Herein, we highlight two cases of amniotic constriction band that presented to our unit with unique clinical characteristics. To the best of our knowledge, an isolated circumferential band of scarring on the face with ocular involvement, as demonstrated by the first case, and a combination of bilateral complete cleft lip and palate with bilateral microphthalmia, auto-amputation of the right thumb, and a constriction band on the left thumb, as demonstrated by the second case, are extremely rare presentations of amniotic constriction band that were not previously reported in the literature and therefore necessitate a special mention. We discuss potential etiologies for these cases and review the existing literature on this entity.

A large osteoid osteoma of the mandibular condyle causing conductive hearing loss: a case report and review of literature.

Osteoid osteomas are benign skeletal neoplasms that are commonly encountered in the bones of the lower extremities, but are exceedingly rare in jaw bones with a prevalence of less than 1%. This unique clinical entity is usually seen in younger individuals, with nocturnal pain and swelling as its characteristic clinical manifestations. The size of the lesion is rarely found to be more than 2 cm. We hereby report a rare case of osteoid osteoma originating from the neck of the mandibular condyle that grew to large enough proportions to result in conductive hearing loss in addition to pain, swelling and restricted mouth opening. In addition, an effort has been made to review all the documented cases of osteoid osteomas of the jaws that have been published in the literature thus far.

Use of repeat anterior maxillary distraction to correct residual midface hypoplasia in cleft patients.

OBJECTIVES: The study was designed to evaluate the efficacy of performing a second, repeat anterior maxillary distraction (AMD) to treat residual cleft maxillary hypoplasia. MATERIALS AND METHODS: Five patients between the ages of 12 to 15 years with a history of AMD and with residual cleft maxillary hypoplasia were included in the study. Inclusion was irrespective of gender, type of cleft lip and palate, and the amount of advancement needed. Repeat AMD was executed in these patients 4 to 5 years after the primary AMD procedure to correct the cleft maxillary hypoplasia that had developed since the initial procedure. Orthopantomogram (OPG) and lateral cephalograms were taken for evaluation preoperatively, immediately after distraction, after consolidation, and one year postoperatively. The data obtained was tabulated and a Mann Whitney U-test was used for statistical comparisons. RESULTS: At the time of presentation, a residual maxillary hypoplasia was observed with a well maintained distraction gap on the OPG which ruled out the occurrence of a relapse. Favorable movement of the segments without any resistance was seen in all patients. Mean maxillary advancement of 10.56 mm was achieved at repeat AMD. Statistically significant increases in midfacial length, SNA angle, and nasion perpendicular to point A distance was achieved (P=0.012, P=0.011, and P=0.012, respectively). Good profile was achieved for all patients. Minimal transient complications, for example anterior open bite and bleeding episodes, were managed. CONCLUSION: Addressing the problem of cleft maxillary hypoplasia at an early age (12-15 years) is beneficial for the child. Residual hypoplasia may develop in some patients, which may require additional corrective procedures. The results of our study show that AMD can be repeated when residual deformity develops with the previous procedure having no negative impact on the results of the repeat procedure.

Tooth-Borne Anterior Maxillary Distraction for Cleft Maxillary Hypoplasia: Our Experience With 147 Patients.

PURPOSE: To evaluate the results of anterior maxillary distraction for its efficacy and long-term stability in the management of cleft maxillary hypoplasia in a large series of patients with a long-term follow-up extending to 4 years. MATERIALS AND METHODS: One hundred sixty-four patients at least 10 years old with cleft maxillary hypoplasia who presented to the authors' unit from January 2009 through October 2014 were evaluated retrospectively, irrespective of gender, type of cleft lip and palate, and amount of advancement needed. Anterior maxillary distraction using a tooth-borne distractor appliance was carried out in all patients and all patients were followed up to 4 years (range, 1 to 4 yr) to evaluate the stability of the procedure and to document any relapse using digitalized lateral cephalograms taken before distraction, immediately after distraction (T2), and at the last follow-up visit (T3; range, 1 to 4 yr). Seventeen patients were subsequently lost to follow-up; therefore, a complete set of records was available for 147 patients. In a subset of 50 patients, perceptual speech assessment was carried out preoperatively and 6 months postoperatively by 2 speech pathologists using the Perkins scoring system that allowed the evaluation of 5 parameters (velopharyngeal insufficiency, resonance, nasal air emission, articulation, and intelligibility). None of these patients underwent speech therapy during the course of evaluation. The development of complications intra- or postoperatively was noted. The data were tabulated and analyzed. RESULTS: An advancement ranging from 4.0 to 13.1 mm (mean, 9.42 mm) was achieved in all patients. One hundred forty patients (95.23%) showed stable results on lateral cephalograms and when T2 values were compared with T3 values. Seven patients (4.76%) exhibited skeletal relapse in various linear and angular measurements assessed on lateral cephalograms. At 6-month follow-up, improvements of 62% (n = 31), 64% (n = 32), 50% (n = 25), 68% (n = 34), and 70% (n = 35) in velopharyngeal insufficiency, resonance, nasal air emission, articulation, and intelligibility, respectively, were observed, with worsening of all parameters in 1 patient (2%). An overall complication rate of 25.17% (n = 37) was noted, with bleeding being the most common intra- and postoperative complication noted in 15 patients (10.2%). No serious consequences related to any complication were noted. CONCLUSION: Anterior maxillary distraction can be considered a suitable treatment option for the management of mild to moderate cleft maxillary hypoplasia because the anteroposterior deficiency can be addressed at a young age, immediately after the eruption of the maxillary second premolars. Stable long-term results with negligible skeletal relapse are possible with this technique, with an added advantage of unhampered or even improved velopharyngeal function.

Management of Lip Pits in Van der Woude Syndrome: A Clinical Classification With Difficulty Index.

PURPOSE: Numerous case reports have been published on lip pits in Van der Woude syndrome explaining the morphology and genetics in detail; however, thus far, no article has focused on the classification of lip pits as an aid in surgical management. Although the procedure for lip pits in Van der Woude syndrome appears straightforward, even in the best of hands, the excision can be very challenging with no guarantee of esthetically desirable results. Therefore, we have devised a classification based on a difficulty index in the management of lower lip pits to assist in predicting the treatment outcome before surgery, as well as to offer the choice of a particular technique in a specific situation. MATERIALS AND METHODS: We reviewed 19 cases of Van der Woude syndrome having lower lip pits that were operated on at our unit from May 2005 to June 2015 with a minimum follow-up of at least 6 months. The data analyzed included the patient's age and gender, location of the lip pits with regard to their proximity to the white skin roll, number of lip pits, presurgical depth of the lip pits, and discharge of mucous secretion from the pits, as well as timing of lip pit excision. Four techniques of excision were performed via routine excision, modified routine excision, vertical wedge excision, and inverted-T lip reduction. The data were tabulated and analyzed. On the basis of our experience in managing lip pits, a clinically relevant classification with a difficulty index was then proposed. RESULTS: Among the 12 patients having preoperative involvement of the white skin roll, 8 had distortion of the white skin roll when operated on by either routine excision (n = 2), modified routine excision (n = 3), or inverted-T lip reduction (n = 3). The remaining 4 patients had no distortion of the white skin roll after surgery when the vertical wedge excision technique was performed. The 7 patients who had no distortion of the white skin roll preoperatively presented with esthetic results when operated on by either the routine excision, modified routine excision, or inverted-T lip reduction technique. In 2 patients with a presurgical pit depth greater than 6 mm, mucocele formation was observed after surgery. Using the data obtained, we proposed a classification based on 2 parameters: involvement of the white skin roll and presurgical depth. A difficulty index also was proposed using these same variables. CONCLUSIONS: Classification and evaluation of the difficulty of lip pit excision are essential in planning the surgical treatment to give improved esthetic results. This proposed classification and difficulty index will provide the operating surgeon with a standardized scheme to evaluate the difficulty of the excision as well as to predict the overall outcome of the procedure before surgery.

Use of regenerative tissue matrix as an oral layer for the closure of recalcitrant anterior palatal fistulae: a pilot study.

OBJECTIVES: To evaluate the effectiveness of regenerative tissue matrix (Alloderm) as an oral layer for difficult anterior palatal fistula closure. MATERIALS AND METHODS: The authors have tested the feasibility of a novel surgical technique of adding a regenerative tissue matrix (Alloderm) as an oral layer for closure of recalcitrant large anterior palatal fistulae and report the outcome of the first 12 patients in this pilot study. Patients with recurrent large fistula who otherwise would require either a local pedicled flap, free flap, or an obturator were treated with this technique and followed up for at least 6 months to monitor the progress of healing. RESULTS: Of the 12 patients, 8 patients (66.7%) had complete closure of the fistula, and 2 patients (16.7%) showed reduction in size of the fistula to the extent that symptoms were eliminated, for an overall success rate of 83.3% (10/12 patients). Premature graft loss and recurrence of the fistula were noted in 2 patients (16.7%). CONCLUSION: Alloderm provided an adequate barrier allowing healing to occur unimpeded and allowed closure of the palatal fistula. In our experience, this new technique using regenerative tissue matrix as an adjunct to the oral layer in large anterior palatal fistula has an advantage compared to other more invasive complex procedures and has been shown to provide satisfactory results.

Perceptual Speech Assessment After Anterior Maxillary Distraction in Patients With Cleft Maxillary Hypoplasia.

PURPOSE: To assess speech outcomes after anterior maxillary distraction (AMD) in patients with cleft-related maxillary hypoplasia. MATERIALS AND METHODS: Fifty-eight patients at least 10 years old with cleft-related maxillary hypoplasia were included in this study irrespective of gender, type of cleft lip and palate, and amount of required advancement. AMD was carried out in all patients using a tooth-borne palatal distractor by a single oral and maxillofacial surgeon. Perceptual speech assessment was performed by 2 speech language pathologists preoperatively, before placement of the distractor device, and 6 months postoperatively using the scoring system of Perkins et al (Plast Reconstr Surg 116:72, 2005); the system evaluates velopharyngeal insufficiency (VPI), resonance, nasal air emission, articulation errors, and intelligibility. The data obtained were tabulated and subjected to statistical analysis using Wilcoxon signed rank test. A P value less than .05 was considered significant. RESULTS: Eight patients were lost to follow-up. At 6-month follow-up, improvements of 62% (n = 31), 64% (n = 32), 50% (n = 25), 68% (n = 34), and 70% (n = 35) in VPI, resonance, nasal air emission, articulation, and intelligibility, respectively, were observed, with worsening of all parameters in 1 patient (2%). The results for all tested parameters were highly significant (P ≤ .001). CONCLUSION: AMD offers a substantial improvement in speech for all 5 parameters of perceptual speech assessment.

Management of human bite injury of the upper and lower eyelids: a rare case report.

Human bite injury to the eyelid is extremely rare and poses a significant challenge in surgical reconstruction. We report an extremely rare case of human bite injury to the eyelid in a 43-year-old male with approximately 60% full thickness loss of the upper eyelid and 80% to 90% full thickness loss of the lower eyelid and its successful reconstruction using the local advancement cheek flap.

Palatal fistulae: a comprehensive classification and difficulty index.

INTRODUCTION: Palatal fistula formation is a known complication of palatoplasty. Numerous classifications have been proposed that help in identifying the location of fistula and systematically arrange data for record keeping. They do not assess the difficulty level of the fistula. Management of fistulae can be very tricky and a definitive success cannot be guaranteed even in the best of hands. Hence we devised a classification system and a difficulty index to help evaluate the difficulty level and plan the treatment accordingly to predict the prognosis prior to surgery. MATERIALS AND METHODS: We reviewed 610 cases of palatal fistula operated at our center with a minimum follow-up of 6 months from May 2003 to May 2010. They were classified according to our classification. Difficulty index was also assessed. The data was tabulated and analysed. RESULTS: Longitudinal fistulae showed a recurrence rate of 7.87 % whereas transverse fistulae showed a recurrence rate of 19.66 %. Total recurrence rate was 11.31 %. Unilateral clefts with fistulae showed a recurrence of 6.55 % whereas bilateral clefts with fistulae showed a recurrence of 14.17 %. A total of 220 Grade 1 and 390 Grade 2 fistulae were managed. Out of these, 7 (3.18 %) Grade 1 and 62 (15.90 %) Grade 2 fistulae recurred. 90 % of failed fistulae showed decrease in the size of the fistula. CONCLUSION: Classification and evaluation of difficulty of palatal fistula is essential to plan the surgical treatment so as to give better results. Bidimensional fistulae in the anterior hard palate are associated with higher recurrence rate. Also, fistulae in bilateral clefts are more difficult to close than those in unilateral clefts. Classification of fistulae according to the difficulty index helps in pre-operative judgment of the outcome.

Anterior maxillary distraction using a tooth-borne device for hypoplastic cleft maxillas-a pilot study.

PURPOSE: The management of maxillary retrusion in a patient with cleft lip and palate is performed using Le Fort I advancement or distraction osteogenesis using an external or an internal device. Distraction mostly involves movement of the entire maxilla at the Le Fort I level and is characterized by a higher relapse rate and a hampering of speech. Hence, distraction of the anterior maxilla was performed using a tooth-borne palatal distractor similar to the one proposed by Gunaseelan et al (J Oral Maxillofac Surg 65:1044, 2007). However, the technique was modified by placing the distractor preoperatively before performing the osteotomy cut. The main advantage of this modification is that more control over the vector can be achieved and chances of cement failure caused by contamination and an inability to achieve isolation is drastically decreased. This study investigated the efficacy and stability of anterior maxillary distraction in the management of cleft maxillary retrognathia and any resulting complications. MATERIALS AND METHODS: Fourteen patients older than 12 years with cleft maxillary retrognathia were included in the study irrespective of gender, type of cleft lip and palate, and the amount of advancement needed. The patients were evaluated using Orthopantomogram and lateral cephalograms preoperatively and at 3 months postoperatively (ie, before appliance removal) and at 6 months postoperatively. The distractor was fabricated extraorally on a cast and cemented into the patient's mouth the day before surgery. The initial deficiency, amount of advancement achieved, and relapses at 6 months, if any, were studied. The data were tabulated and analyzed. RESULTS: Only 1 of the 14 patients showed a relapse, of 2 mm, because this patient was lost to follow-up and thus delayed tooth replacement. CONCLUSIONS: Anterior maxillary distraction with a tooth-borne device is a feasible modality for the management of cleft maxillary retrognathia, with stable results.