Gross Anatomical Study of Congenital Meningoencephalocoele and Craniofacial Deformities in A Kid

Author(s):  
C. Lavanya ◽  
Sabiha H. Basha ◽  
S. Hamsa Yamini ◽  
A. Vijay ◽  
K.K. Ponnu Swamy

Background: Occurrence of congenital anomalies are less common in small ruminants, especially in goats compared to sheep. Most common cause for the congenital deformities in new born ruminants is due to the ingestion of toxic plants during gestation. Congenital meningoencephalocoele with associated craniofacial deformities is a rare occurrence in goats. Materials: A day old crossbred kid was presented with cranial defects and protrusion of brain through the defect. Detailed examination of the animal revealed cranioschisis in the frontal and parietal region along with presence of cleft lip, cleft palate and accessory ear lobe on the right side. Herniation of meninges and brain was exposed directly and was not covered by skin. There were two parietal cranial defects separated by a plate of bone. Result: Postmortem examination of the animal showed bilateral cleft lip and cleft palate with lateral deviation of nasal septum and deformation of premaxillary region. On reflection of skin over the frontal region, revealed frontal cranioschisis and the brain was situated subcutaneously. There were total of three cranial defects (two in the parietal and one in the frontal region) separated by small plates of bone in between them. This present study explains the anatomical aspects of cranioschisis, meningoencephalocoele, cleft lip, cleft palate and associated craniofacial abnormalities in a day old kid.

2021 ◽  
pp. 105566562110577
Author(s):  
Jaideep Singh Chauhan ◽  
Sarwpriya Sharma

Objective: To analyse the morphological presentation of orofacial clefts, gender, syndromes and systemic anomalies associated with them. Design: This was an epidemiological study performed in the patients who were registered for cleft lip and palate surgeries in our centre. The data was evaluated both retrospectively as well as prospectively. Patients/ Participants: The patients registered from November 2006 to April 2021 were studied. Out of 5276 patients, data of 5004 cases were analysed, rest 272 patients were excluded due to lack of information. Statistical analysis and Chi square test were applied. Results: Cleft deformities were more common in males than females. Cleft lip with palate was the commonest phenotype (52.2%). It was followed by isolated cleft lip (22.9%), isolated cleft palate (22.1%), rare clefts (1.62%) and syndromic clefts (1.18%). Unilateral variants were more frequent than bilateral. In unilateral, left side was more common than the right side. Among bilateral, most of the cases had premaxillary protrusion. In the present study, 3.46% of all the patients had associated anomalies affecting their other organs. Less common cleft phenotypes like microform cleft lip and submucous cleft palate ± bifid uvula showed frequency of 0.62% and 0.64% respectively. Conclusion: Thorough examination of cleft deformity should be done as it may appear as an isolated deformity or part of a syndrome and have associated systemic anomalies. This may help us to deliver comprehensive care to the patients and can prevent potential operative complications.


2013 ◽  
Vol 52 (192) ◽  
Author(s):  
Shankar Man Rai ◽  
Kiran Nakarmi ◽  
Surendra Basnet ◽  
Pramila Shakya ◽  
Krishna Nagarkoti ◽  
...  

Introduction: Timely repair of cleft lip and palate maximises the benefits of surgery. Developing countries have large number of adults with unrepaired clefts. The impact of a cleft program can be determined by observing the trends of lower age at surgery. Public Health Concern Trust, Nepal has been providing a comprehensive nationwide cleft service since 1999. This study was conducted to see any change in the age at surgery. Methods: A retrospective cross sectional study was conducted to analyse the data of all the individuals’ age at primary cleft surgery from July 1999 to June 2010. Mean and median age of individuals as well as the proportion of individuals operated on at the right age in different years were calculated and compared. Results: The median age for cleft lip surgery decreased from 100 to 24 months. Similarly the median age for cleft palate surgery decreased from 70 to 28 months. Proportion of surgeries carried out in the recommended age also increased. A change in the policy of the program reaching out to more remote areas and removing the age barrier for surgery resulted in older adults receiving surgery and increased median age especially for cleft palate repairs. Conclusions: A nationwide cleft program for a decade had a small impact on age at surgery. There are still many individuals who are missing the ideal age for surgery. The program needs to reach more remote areas. This information will be useful for governmental as well as non-governmental organizations working in the area of clefts. Keywords: age; cleft lip; cleft palate; Nepal.    


2020 ◽  
Vol 09 (04) ◽  
pp. 289-292
Author(s):  
Jong In You ◽  
Seul Ki Bang ◽  
Seung-Young Yu ◽  
Kyung Hyun Jin

AbstractWe describe a patient with oral-facial-digital syndrome (OFDS) with the following anomalies: cleft lip, cleft palate, micrognathia, hypertelorism, nasal septum deviation, thumb polydactyly in the right hand, and partial agenesis of the corpus callosum. In addition, the patient had optic disc coloboma in the left eye and subfoveal drusenoid deposit in the right eye, features of OFDS type IX. Subfoveal drusenoid deposit has not been previously reported in OFDS type IX. Evaluation of the fundus is necessary for diagnosis of OFDS.


2017 ◽  
Vol 1 (1) ◽  
pp. 1-3
Author(s):  
Gediminas Zekonis ◽  
Egle Zukauskaite ◽  
Vilte Masiliunaite ◽  
Agne Dzingute

Cleft palate and cleft lip are the most common congenital deformities involving the orofacial region. One of the most important problems with this deformation is difficulties with feeding due to lack of sufficient negative intraoral pressure which prevents regurgitation of food into the nasal cavity. Cleft lip and cleft palate can be surgically treated but repair of the palate is usually delayed until 6 months to 2 years of age. Early repair of the palate may have a negative effect on the growth and development of maxilla due to the resulting scar tissue. This article presents the Prosthodontic management of an infant with a cleft palate through fabricating a feeding plate (palatal obturator). Keywords: Cleft palate, Feeding plate, Palatal obturator


2021 ◽  
Author(s):  
Bum Jun Kim ◽  
Hitisha P Zaveri ◽  
Peter N Kundert ◽  
Valerie K Jordan ◽  
Tiana M Scott ◽  
...  

AbstractDeletions of chromosome 1p36 are the most common telomeric deletions in humans and are associated with an increased risk of orofacial clefting. Deletion/phenotype mapping, combined with data from human and mouse studies, suggests the existence of multiple 1p36 genes associated with orofacial clefting including SKI, PRDM16, PAX7 and GRHL3. The arginine–glutamic acid dipeptide (RE) repeats gene (RERE) is located in the proximal critical region for 1p36 deletion syndrome and encodes a nuclear receptor co-regulator. Pathogenic RERE variants have been shown to cause neurodevelopmental disorder with or without anomalies of the brain, eye or heart (NEDBEH). Cleft lip has previously been described in one individual with NEDBEH. Here we report the first individual with NEDBEH to have a cleft palate. We confirm that RERE is broadly expressed in the palate during mouse embryonic development, and we demonstrate that the majority of RERE-deficient mouse embryos on C57BL/6 background have cleft palate. We go on to show that ablation of Rere in cranial neural crest (CNC) cells, mediated by a Wnt1-Cre, leads to delayed elevation of the palatal shelves and cleft palate and that proliferation of mesenchymal cells in the palatal shelves is significantly reduced in Rereflox/flox; Wnt1-Cre embryos. We conclude that loss of RERE function contributes to the development of orofacial clefts in individuals with proximal 1p36 deletions and NEDBEH and that RERE expression in CNC cells and their derivatives is required for normal palatal development.


2014 ◽  
Vol 03 (04) ◽  
pp. 203-208
Author(s):  
Alpana Barman ◽  
B C Dutta ◽  
J K Sarkar

Abstract Background : Cleft lip and palate are some of the most common congenital deformities. They frequently occur as isolated deformities, but can be associated with other medical conditions and anomalies. Aim of the study: To study the distribution of different types of cleft lip and palate and associated anomalies. Materials and methods: Forty patients of cleft lip and palate were studied. Relevant history was taken, clinical examination done and recorded with photography with consent. The cases were classified as per Nagpur Classification. Results: In our study 24 cases (60%) were cleft lip and palate (type III) and 10 cases (25%) of cleft palate alone (type II) and 6 cases (15%) of cleft lip alone (type I). Males were found to predominate in type I and III. Females predominated in type II. Among the cleft lip, left side is found to be involved in most of the cases. In our study, out of 40 cases, 8 cases (20%) had other associated anomalies. Most of these were associated with cleft lip and palate (type III) and isolated cleft palate (type II). Conclusion: The typical universal distribution of cleft types and associated anomalies tallies with our results.


2004 ◽  
Vol 41 (4) ◽  
pp. 456-458 ◽  
Author(s):  
Kamile Marakoglu ◽  
E. Ferda Percin ◽  
Ismail Marakoglu ◽  
Ulvi Kahraman Gursoy ◽  
Fahrettin Goze

Objective Natal/neonatal teeth are very common in children with complete unilateral and bilateral cleft lip and palate. This article outlines a patient with intrauterine growth retardation, anencephaly, atrial septal defect, ventricular septal defect, two maxillary first natal incisor teeth, cleft palate, short neck, low-set ears, hypertelorism, retrognathia, and simian-line on the right hand. There is no conclusive evidence of a correlation between these findings and a known syndrome, suggesting that this case may be a hitherto undefined clinical combination with neonatal teeth.


QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Amr A Mabrouk ◽  
Iman M Alliethy ◽  
Niveen F Al Mahmoudy ◽  
Yara S El-sayed

Abstract Background Oro-facial clefts include a range of congenital deformities most commonly presenting as cleft lip with or without cleft palate (CLP) or isolated cleft palate (CP). CLP is the second most common congenital birth defect in the U.S. Aim of the Work to assess modified v-advancement vermilion flap combined with Tennison technique for vermilion repair in unilateral cleft lip regarding aesthetic outcome in comparison with repair of cleft lip by Tennison technique. Patients and methods This study was conducted in plastic, maxillofacial and reconstructive surgery department in Ain Shams University between September 2019 to February 2020. It included 20 patients divided into two group; the first group consisted of 10 patients who underwent repair of cleft lip by Tennison technique and the second group consisted of 10 patients who underwent repair by Tennison technique combined with v- advancement vermilion flap. Both groups were assessed postoperatively by Asher-McDade et. al. scoring system. Result The study showed statistically significant difference between the two groups as regard the white lip length which was more symmetrical in cases repaired by Tennison technique combined with modified v-advancement vermillion flap (P = 0.01). The bulge and the deficiency in the lateral vermilion were decreased in the group treated by Tennison technique with modified v-advancement vermilion flap. However the difference was statistically insignificant (P = 0.66). Conclusion The choice of a technique for surgical correction of UCL should be based on an evidence that this technique has the best functional and aesthetic outcomes. The findings of this study support the view that these two methods of cleft lip repair have their own advantages and disadvantages. Tennison technique combined with vadvancement vermilion flap achieved less bulge in the vermilion with no statistically significant difference.


2021 ◽  
Vol 15 (8) ◽  
pp. 2089-2092
Author(s):  
Muhammad Anwar ◽  
Gulam Mustafa ◽  
Zishan Haider

Objectives: To determine various types of cleft lip and palate, its geographical distribution and its association with cousin marriages, family history and other craniofacial syndromes in the Southern Pakistani population. Study design: Cross-sectional study Place and Duration of Study: Plastic Surgery Department of Sheikh Zayed Medical College / Hospital, Rahim Yar Khan and Hamza Medicare, Rahim Yar Khan from January 2020 to March 2021. Methodology: All patients who presented to the outpatients department with cleft deformities of lip and palate, irrespective of their age and gender were included in the study. Patients who were operated previously and those who were not agree with the study protocol were excluded from the study. The data were collected through a structured proforma as well as history and physical examination. The data were organized and analyzed through Statistical Package for Social Sciences version 23. Results: Total of 403 patients with cleft lip and palate deformities were included in this study, with an average age of 39±62 months. Out of total 239 59.3% were males with male to females ratio of 1.4:1. Cleft lip with cleft palate, cleft palate only and isolated cleft lip were found in 56.8%, 13.4% and 29.20% of cases respectively and 2 (0.5%) cases of median cleft. Out of total, 155 (38.5%) were from Southern Punjab, 50.6% from Sindh and 10.9% were from Baluchistan Province. In 386 (95.8%) cases parent had a History of cousin marriage, and family history of Cleft Lip & Cleft Palate was positive in 67 (16.6%) cases. Other congenital anomalies were found in 12.5% of cases. Conclusion: Cleft lip and palate deformities affected the male population more than females. Cleft lip in association with cleft palate is the commonest deformity. Isolated cleft palate mainly affected females. The high incidence of these anomalies in cousin marriages emphasizes educating the people to avoid cousin marriages. Key Words: Cleft Lip, Cleft Palate, Consanguinity, Family history. Congenital deformities


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