Background: The prevalence of congenital heart defects (CHD) in children with cleft lips (CLs) and palate (CP) is reportedly higher than the general population. Several studies have described diverse rate of CHD in different localities. Aims and Objectives: To the authors' knowledge, the frequency and pattern of CHD among children with CLs and CP has not described in this geopolitical zone. Materials and Methods: This is a retrospective study of the echocardiographic records of all cases of CL and CP seen over a 4 years period, between November, 2010 and October, 2013. Data were analyzed and expressed using frequency tables and percentages. Results: A total 30 children were referred for echocardiographic screening (ECGS) during the period. There were 17 males and 13 females, giving a male:female of about 1:1. The age range was between 15 days and 12 years with a median age of 4 months. There were 6 (20%) cases of CHDs. Conclusions: The relatively high rate of CHD among children with oral clefts in this study justifies the need for ECGS for these categories of children.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Background: Intra-oral surgeries such as cleft palate repair essentially require holding of the jaws in the open position to facilitate access to the oral cavity, which is mostly achieved with the use of mouth gags. Materials and Methods: The objective of this article is to present an account of various types and modifications of gags as surgeons, anesthetists, and innovators attempt to make that ideal mouth gag. The authors present this compendium of development of mouth gags using articles sourced from Medline, surgical catalogs, museums, ancient manuscripts, original quotes, techniques, and illustrations. Results: This article describes notable types and variants of mouth gags with an attempt to classify them. Conclusions: The huge number of modifications done and reports of newer variants appearing at regular intervals goes to prove that we are still far from developing the ideal mouth gag.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Classification of the cleft has evolved over a century. Many descriptive, diagrammatic, and coding systems have been proposed to be used. However, there are only few which have stood the test of time. One of them is Indian classification. Indian classification of cleft lip (CL) and palate proposed in 1975 is a popular classification in India presently. There are numerous combinations of cleft deformities, and we found that some of them could not be classified appropriately with the original classification. The clefts are classified in three groups: CL as Group 1, cleft palate as Group 2 and Group 3 for combined CL, alveolus and palate in continuity. Originally right, left, midline, and alveolus were abbreviated. To make the classification wholesome, the original classification has been revisited and presented with additional features. The basic classification in three groups remains as original. Additional abbreviations have been added to classify the special situations. Partial, submucosal, Simonart's band, protruding premaxilla, and microform have been added to the list of abbreviations. This classification has been used for over 30 years by the author in over 4000 cleft patients. We find it simple to use, versatile enough to classify almost all possible cleft combinations, easy for communication during discussion and convenient to write as diagnosis in patients' files. Easy computer archiving and efficient retrieval of the data are the special features of this classification.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Osteopetrosis also known as "marble bone disease" is a group of rare genetic disorders caused by osteoclast failure, which ranges widely in severity. Osteopetrosis presents with a spectrum of craniofacial abnormalities such as frontal bossing, macrocephaly, hydrocephaly, and cranial hyperostosis. Osteopetrosis is caused by failure of osteoclast development or function and mutations in at least 10 genes have been identified as causative in humans, accounting for 70% of all cases. These conditions can be inherited as autosomal recessive, dominant or X-linked traits with the most severe forms being autosomal recessive. We present a rare case of osteopetrosis in a 10-year-old boy who reported with an unhealed socket after tooth extraction. The characteristic clinical and radiographic findings were suggestive of intermediate osteopetrosis.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Craniofacial clefts cause severe facial disfigurement even in minor forms. The surgical reconstruction is imperative to restore function and appearance of facial structures. The presentation of Tessier number: 0 cleft patient may vary from minimal changes on median facial structures such lip, vermilion and nose, and nose to wide clefts dividing all median craniofacial structures. The variability of expression of the unusual orofacial clefts can be challenging for the surgeon, while reconstructing affected facial structures. In this report, we present the surgical management of the case with severe bifid nose. A 27-year-old male presented with congenital midfacial disfigurement with hypertelorism. The patient had a flat nasal dorsum and a deep groove between the two alar domes. The nose was short and bifid. The patient did not accept facial bipartition surgery. We performed de-epithelialization on the skin groove between the two alar domes. We repaired lower one-third part of the nose with native nasal tissues. We reconstructed upper two-third part of the nose with the osteocartilage frame harvested from the calvarium and the nasal septum. Two superiorly based-nasolabial flaps were designed with sufficient length to provide external cover of the osteocartilage frame. In case of the severe bifid nose, osteocartilaginous and soft tissue structures of the nose must be restored separately. Following reconstruction of the osteocartilaginous framework with nasal tissues or grafts, remaining nasal soft tissue, and local flaps can be used to cover the soft tissue.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Diprosopus or craniofacial duplication is an extremely rare craniofacial anomaly. It is the rarest form of conjoined twins. One of the types of incomplete facial duplication is the duplication of nose known as diprosopus dirrhinus, of which there is only a single case report from India to date. In the present article, the authors describe a 5-year-old boy having diprosopus dirrhinus with severe hypertelorism, right cleft 12 and right upper eyelid coloboma, a combination of anomalies which have not been reported to date.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

A new era in cleft lip repair began when Dr. Ralph D Millard introduced his technique of rotation and advancement. In 1987, Mohler described a variation of Millard's unilateral repair that included a columellar extension. In 2005, Fisher introduced the principle of anatomic subunit closure. These techniques can work very well across the spectrum of unilateral clefts of the lip, though challenges arise with wide clefts and those where there is a large discrepancy between the greater lip height and lesser lip height. In cases when the discrepancy is high, the surgeon often tries to gain the necessary lip height on the cleft side by shifting the Noordhoff's point more laterally, sacrificing excessive tissue. This often results in a lateral lip that is hypoplastic, giving an unnatural look to the repaired upper lip. Focus has traditionally been on getting the necessary lip height, sacrificing fullness of the lateral lip element. This paper describes several strategies to optimally manage the lateral lip element in rotation-advancement technique for unilateral cleft lip repair. The results with this technique are encouraging and can be utilized to offset the drawbacks of rotation-advancement technique in very wide and short cleft lips.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

The congenital anomaly of cleft lips and palates is probably more misunderstood than most. The attitude of National Governments, medical professionals and society at large often ranges from unsympathetic to callous. Luckily this is changing and bringing more cleft affected people back into the mainstream than at any other time in history. But there's still a long way to go.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Introduction: The multi-disciplinary approach to cleft care has been a reality since the beginning of the last century, but there is a paucity of literature discussing or recommending specific cleft protocols. This is understandable due to the significant difference in cleft protocols around the world and the controversies surrounding each of them. Material and Methods: The Indian Society of Cleft Lip Palate and Craniofacial Anomalies in its 13 ^th Annual Conference discussed threadbare the different protocols around the world and propose a guideline to Indian surgeons delivering cleft care. Results and Discussion: These guidelines though not binding, are supposed to be pointers to a generally accepted standard for cleft care, considering the unique circumstances and limitations of cleft care providers in developing countries like India. It is a "best practice" indicator which, if adhered to by all the cleft care providers, will soon bring about uniformity in cleft care deliverance and allow us to evaluate our results on a much larger scale than has hitherto been possible.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Background: Cleft lip and palate is one of the major congenital malformations in the newborn. Folic acid or folate has been postulated to have a protective effect on the occurrence of cleft lip and palate if taken in the periconceptional period. Aims: The aim of this study is to evaluate and compare the serum folate levels of mothers of cleft children with that of mothers with healthy children and women of childbearing age. Materials and Methods: A total of 44 subjects were included in the study of which 28 formed the study group comprising of women who had delivered children with cleft lip and palate and 8 were women who had delivered healthy children and formed Control Group I. Control Group II consisted of 8 women of childbearing age. Serum folate levels were assayed using an automated chemiluminescence assay. Results: We observed no significant difference in the serum folate levels of the study group as compared with the control groups. However, the mean serum folate level in the study group was marginally lower than in Control Group I. Conclusions: Since, in our study, folic acid deficiency could not be demonstrated in the serum of women with cleft children, our results suggest that serum folate levels may not be the only reason for clefting. Furthermore, the range of folate in a population is usually narrow; therefore, a significant difference in the serum folate levels may not be demonstrable.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Facial clefts are very common facial anomalies encountered in practice. However, lower facial clefts are very rare. Tessier has grouped these defects as cleft no. 30. Only 65 cases have been reported in the literature until 1996. Median cleft of the lower lip, mandible and bifid tongue with ankyloglossia has been reported in this anomaly. We report this rare cleft associated with bifid tongue with foregut duplication cyst over the floor of mouth.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

We present a case report of a 23-year-old female with syndromic face. She presented to us with typical facial features of Crouzon syndrome. Her childhood was uneventful except for the deformed face. Craniofacial surgeries to correct the deformation were not done in childhood due to risk and complications. Patient requested for the correction of facial deformity without major craniofacial surgery. She underwent two staged procedure. In the first stage, autofat grafting of forehead, left supraorbital rim and malar areas were performed with cheek fat removal. Chin and mid-face augmentation with costal cartilage grafts were done. Septorhinoplasty was done as a second stage procedure. She has got excellent outcome by this camouflage surgical approach. This approach can be used in selective cases with minimal risk. Excellent esthetic results can be achieved by thorough analysis and involvement of the patient and parents in planning.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

Facial pain is a complex disorder to diagnose and to manage. Problems in the craniofacial area may present with conflicting and blurred symptomatology that may result in an obscured clinical picture where a single etiological factor may seem unlikely. This is a report of a case of chronic palatal pain in a patient who was previously surgically managed for a cleft soft palate and partial cleft hard palate.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

Isolated congenital alar defects are extremely rare. In most cases, alar defects are acquired because of burns, trauma, and tumor excisions. We present two cases of congenital alar cleft reconstructed with transposition of ala, conchal cartilage support and use of composite chondrocutanous graft. The described technique attempts to avoid drawbacks of other methods such as rotation flap, and z-plasty technique. Absolute symmetry maintained with no retraction of alar rim on long-term follow-up with good esthetic results.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

A Submucous Cleft of the Palate presents a surgical challenge to improve function in an apparently normal area. Surgery itself should not add to the morbidity. Traditional approaches leave extensive or significant scarring and sometimes fistulae. A technique, termed as Minimal Access Palatoplasty (MAP) was designed to avoid these sequelae and facilitate surgery. 28 consecutive patients with submucous clefts were operated using this technique by a single surgeon over a period of 4 years. Patients were reviewed for surgical outcome. Follow up ranged from 4 weeks to 3 years. None of the patients had a fistula. Results are presented. This technique takes less time, is easy for even a novice and can be used in combination with Levator muscle dissection or Furlows technique as desired.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]