CASE OF VACTERLASSOCIATION WITHOUT THE "V AND L": ANAESTHETIC CHALLENGES

2021 ◽  
pp. 12-13
Author(s):  
Kumari Sneha ◽  
Gunjan Singh ◽  
Kalyan Kumar Saha ◽  
Vanita R Mhaske ◽  
Bikram Gupta
Keyword(s):  
Cleft Palate ◽  
Atrial Septal Defect ◽  
Congenital Malformations ◽  
Cleft Lip ◽  
Septal Defect ◽  
Renal Agenesis ◽  
Tube Placement ◽  
Renal Anomaly ◽  
Endotracheal Tube Placement ◽  
Vertebral Defects

Aim & Objective: To highlight the importance of anesthetic management in a small neonate with cleft lip, cleft palate, single kidney, atrial septal defect, posted for trachea-oesophageal stula repair. Case description:Afemale baby born at 35 weeks of gestation to a 23-year-old primigravida mother by caesarean section in view of foetal distress with breech presentation .Baby had difculty in breathing and froathing from mouth. Cleft lip and cleft palate was present. Anasogastric tube was not going beyond 11 cm per oral route. Chest x ray showed coiling of tube in upper esophagus.F/S/O Type C Tracheo esophageal stula. . Plain Xray abdomen showed presence of bowel gas. Ultrasonography of the abdomen showed right renal agenesis. The left kidney was normal. Echocardiography showed presence of atrial septal defect with left to right shunt, tiny patent ductusarteriosus, mild pulmonary arterial hypertension, mild tricuspid regurgitation. Based on the presence of tracheoesophagealstula, atrialseptal defect, unilateral renal agenesis and absence of features, suggestive of alternative diagnosis infant, meet criteria of vacteral association. Discussion: VACTERL is a cluster of congenital malformations based on the non-random association of various congenital malformations in a single patient. Here “V” denotes vertebral defects or vascular anomalies (single umbilical artery), “A” anal atresia, “C” cardiac abnormalities, “TE” tracheoesophageal stula, “R"renal (kidney) abnormalities and “L” for limb anomalies) Diagnosis of VACTERL association is done only when at least three of the above mentioned congenital malformations are identied in a patient. Although 80% of these cases have vertebral defects, our case is unique as patient does not have one of the commonest occuring association i.e., vertebral anomalies. The other highlight of this case is although reports say that VACTERL babies with ipsilateral renal disorder have the same side limb defects, our case has a renal anomaly with no limb anomaly. Conclusion: Anaesthetic challenges were difcult airway, endotracheal tube placement, low respiratory reserve, small maximum allowable blood loss, long duration of surgery, risk of hypothermia, aspiratedlungs, risk of right to left shunt, difculty in securing intravenous line and intra arterialline. this case needs continuous monitoring of ECG, invasive blood.

Association of cleft lip and atrial septal defect in mice: A preliminary report

Teratology ◽  
1975 ◽  
Vol 11 (3) ◽  
pp. 321-324 ◽  
Author(s):  
F. Clarke Fraser ◽  
Joel Rosen
Keyword(s):  
Atrial Septal Defect ◽  
Preliminary Report ◽  
Cleft Lip ◽  
Septal Defect

JOB LEWIS SMITH ON MATERNAL IMPRESSIONS AS A CAUSE OF CLEFT LIP AND PALATE (1890)

PEDIATRICS ◽  
1981 ◽  
Vol 68 (4) ◽  
pp. 483-483
Author(s):  
T. E. C.
Keyword(s):  
New York ◽  
Cleft Palate ◽  
Congenital Malformations ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Seventh Edition ◽  
Pediatric Society ◽  
Firm Believer

Job Lewis Smith (1827-1897), a founder of the American Pediatric Society and who, with Abraham Jacobi, established pediatrics as a specialty in our country, was a firm believer that strong mental impressions during pregnancy might be a cause of congenital malformations. He gave the following explanation of the cause of cleft lip and palate in the seventh edition of his textbook, published in 1890.1 Mrs. D[unknown], Eighth avenue, New York, seven months before the birth of her child, when visiting at a distance, accidentally broke the plate of a full set of upper teeth. The line of fracture was antero-posterior and through the centre of the plate. Being away from home, she was much annoyed by the accident and retained the fragments of the plate in situ by pressure with the tongue. As she could not open her mouth without the plate falling out, except it was retained by pressure with the tongue, her mind was dwelling almost constantly on the accident during the few days of her visit. Her boy, born seven months subsequently, had a hare-lip and cleft palate. The mother stated that the deficiency in the lip and palate corresponded precisely to the location of the fracture in the plate.

scholarly journals PREVALENCE OF CARDIAC DISEASES

2015 ◽  
Vol 22 (11) ◽  
pp. 1443-1448
Author(s):  
Lubna Latif ◽  
Usman Javed Iqbal
Keyword(s):  
Atrial Septal Defect ◽  
Cleft Lip ◽  
Septal Defect ◽  
Case Series ◽  
First Trimester ◽  
Fetal Outcome ◽  
Cardiac Diseases ◽  
Pregnant Females ◽  
Cardiology Department ◽  
Time Of Presentation

Objectives: The objective of this study was to find the prevalence of cardiacdisease among pregnant females and its impact on feto-maternal outcome. Study Design:Descriptive case series. Setting: Cardiology department Gulab Devi Chest Hospital LahoreDuration: April 2013 to April 2014. Patients & Methods: All pregnant females with cardiacdisease at any gestation with booked or un-booked statutes were included in this study. Patientswere admitted for thorough evaluation and investigations. Labor was monitored intensively. Dataregarding maternal outcomes were noted down on pre-formed questionnaire. Intra partum andpostpartum details were also noted down along with fetal outcome. The results were analyzedusing SPSS version 16.0.. Results: The total number of females presented with cardiac diseasewas 2650, out of which only 35 women were reported as pregnant. The duration of pregnancyat the time of presentation was as follows: 05 (14.2%) females presented in first trimester, 20(57.1%) in second trimester, 08 (22.8%) in third trimester and 02 (5.7%) patients presented inpostpartum period. There were 08 (22.8%) patients who had preterm labor. In terms of fetaloutcome 04 babies had birth weight of less than 1.5 kg, 12 had 1.5-2.0 kg, 15 were in rangeof 2-2.5 kg and 04 were more than 2.5 kg. 27 (77.1%) were term and 08 (22.8%) were pretermbabies. Cleft lip and atrial septal defect were the only two identified congenital anomalies.Conclusion: The overall prevalence of cardiac diseases during pregnancy was found to be1.3% in this study. Most common affected age group was of 20-25 years. Most common cardiacdisease found in our patient was mitral stenosis. 02 pregnancies ended in intrauterine fetaldeath. 08 babies were born preterm. Cleft lip and atrial septal defect were the only two identifiedcongenital anomalies in newborn delivered by our pregnant patients. Every effort should madeto create awareness regarding pre-pregnancy counseling, so that associated fetal and maternalmorbidity can be reduced.

scholarly journals Study of Anomalies Associated With Labiopalatine Clefting

2012 ◽  
Vol 01 (01) ◽  
pp. 014-018
Author(s):  
Pradipta Ray Choudhury ◽  
K.L. Talukdar ◽  
J. Sarma ◽  
Prabahita Baruah
Keyword(s):  
Heart Disease ◽  
Cleft Palate ◽  
Pediatric Surgery ◽  
Congenital Malformations ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
External Ear ◽  
Orofacial Cleft ◽  
Genitourinary System ◽  
Medical College

Abstract Background and aims: Cleft lip with or without cleft palate are the most common congenital malformations of the head and neck accounting for about 65% of all anomalies of this region. Approximately 70% of cleft cases are non-syndromic and occur as isolated condition, while remaining 30% cases are associated with systemic defects involving cardiovascular, musculoskeletal, genitourinary system and other structural anomalies. The purpose of this study is to assess the various anomalies associated with labiopalatine clefting. Materials and methods: Sixty four cases of cleft lip with or without cleft palate attending the OPD of Department of Plastic Surgery and Pediatric Surgery, Gauhati Medical College and Hospital, were studied. Among them three cases were found to be associated with other anomalies. Results: One case of cleft lip and palate presented with multiple anomalies like tongue tie, malformed external ear and micrognathia. Another one had acyanotic heart disease associated with cleft lip. A case of cleft lip and palate was also found to be associated with hydrocephalus. Conclusion : Proper knowledge and details of anomalies associated with orofacial cleft will help to provide necessary treatment and improve survival of these children.

Atrial Septal Defect in Cyanotic CL/Fr Mice

1998 ◽  
Vol 35 (1) ◽  
pp. 58-64
Author(s):  
Shingo Kadowaki ◽  
Michiko Sakamoto ◽  
Hiroshi Kamiishi ◽  
Takashi Tanimura
Keyword(s):  
Atrial Septal Defect ◽  
Airway Obstruction ◽  
Cleft Lip ◽  
Septal Defect ◽  
Cleft Lip And Palate ◽  
Atrial Septum ◽  
Palatal Shelf ◽  
Icr Mice ◽  
Septum Primum ◽  
Septal Development

Objective It is widely known that some newborn CL/Fr mice with cleft lip and palate (CLP) also have cyanotic symptoms, which have been thought to depend on an atrial septal defect (ASD). In a previous study, we found that cyanotic mice tended to have more severe types of CLP. We hypothesize that the mechanical airway obstruction due to a poorly developed palatal shelf and unmoved tongue in CLP(+) mice might be related to the occurrence of cyanosis. The purpose of this study was to examine the relationships between ASD and cyanosis in CLP(+) newborns. Method The newborn hearts from CLP(-), noncyanotic CLP(+), cyanotic CLP(+), CL/Fr mice and ICR mice were examined histologically, and the incidence and size of ASD was determined on neonatal day (ND) 0. In CLP(-) newborns, similar procedures were performed from ND 1 to ND 4. Furthermore, in CLP(+) newborns, development of the palatal shelf was examined. Results While all the ICR mice had a well-developed atrial septum, and the incidence of ASD was 0%, about 80% of CL/Fr mice had ASD, irrespective of the presence or absence of CLP and cyanosis. On ND 0, the septum primum was significantly shorter in cyanotic CLP(+) mice than in CLP(-) mice. It also tended to be shorter in CLP(+) mice than in CLP(-) mice. Between the cyanotics and noncyanotics, there were no significant differences in the incidences of ASD and the rate of septal development. In CLP(-) mice, the septum primum developed well later and no ASD was observed on ND 4. Cyanotic newborns had significantly less developed palatal shelves than did noncyanotics. Conclusions Cyanosis may not be related to ASD and the rate of septal development, but may be related to the occurrence of CLP in this strain. Furthermore, we confirmed that some relationship exists between the development of the palatal shelf and cyanosis. The present study supports our hypothesis concerning the cause of cyanosis in CL/Fr mice.

scholarly journals The Impact of Technology on the Diagnosis of Congenital Malformations

2019 ◽  
Vol 188 (11) ◽  
pp. 1892-1901 ◽  
Author(s):  
Loreen Straub ◽  
Krista F Huybrechts ◽  
Brian T Bateman ◽  
Helen Mogun ◽  
Kathryn J Gray ◽  
...  
Keyword(s):  
Risk Factors ◽  
Ventricular Septal Defect ◽  
Patent Ductus Arteriosus ◽  
Atrial Septal Defect ◽  
Congenital Malformations ◽  
Septal Defect ◽  
Ductus Arteriosus ◽  
Secundum Atrial Septal Defect ◽  
The Impact ◽  
Patent Ductus

Abstract As technology improves and becomes more widely accessible, more subclinical congenital malformations are being detected. Using a cohort of 1,780,156 pregnant women and their offspring nested in the 2000–2013 US Medicaid Analytic eXtract, we contrasted time trends in malformations which do not necessarily present with overt clinical symptoms early in life and are more likely to be diagnosed via imaging (secundum atrial septal defect, patent ductus arteriosus, ventricular septal defect, pulmonary artery anomalies, pulmonary valve stenosis, hydrocephalus) with trends in malformations that are unlikely to escape clinical diagnosis (tetralogy of Fallot, coarctation of the aorta, transposition of the great vessels, hypoplastic left heart syndrome, oral cleft, abdominal wall defect). Logistic regression was used to account for trends in risk factors while assessing the impact of increased screening intensity. Prevalence of the diagnosis of secundum atrial septal defect rose from 2.3‰ in 2000–2001 to 7.5‰ in 2012–2013, of patent ductus arteriosus from 1.9‰ to 4.1‰, and of ventricular septal defect from 3.6‰ to 4.5‰. Trends were not explained by changes in the prevalence of risk factors but were attenuated when accounting for screening tests. The other malformations showed no temporal trends. Findings suggest that increased screening partially explains the observed increase in diagnosis of milder cases of select common malformations.

Preferential Associations between Oral Clefts and Other Major Congenital Anomalies

2008 ◽  
Vol 45 (5) ◽  
pp. 525-532 ◽  
Author(s):  
Monica Rittler ◽  
Jorge S. López-Camelo ◽  
Eduardo E. Castilla ◽  
Eva Bermejo ◽  
Guido Cocchi ◽  
...  
Keyword(s):  
Spina Bifida ◽  
Cleft Palate ◽  
Congenital Anomalies ◽  
Cleft Lip ◽  
Heart Defects ◽  
Strong Association ◽  
Renal Dysplasia ◽  
Oral Clefts ◽  
Underlying Mechanisms ◽  
Vertebral Defects

Objectives: To identify preferential associations between oral clefts (CL  =  cleft lip only, CLP  =  cleft lip with cleft palate, CP  =  cleft palate) and nonoral cleft anomalies, to interpret them on clinical grounds, and, based on the patterns of associated defects, to establish whether CL and CLP are different conditions. Design And Settings: Included were 1416 cleft cases (CL  =  131, CLP  =  565, CP  =  720), among 8304 live- and stillborn infants with multiple congenital anomalies, from 6,559,028 births reported to the International Clearinghouse for Birth Defects Surveillance and Research by 15 registries between 1994 and 2004. Rates of associated anomalies were established, and multinomial logistic regressions applied to identify significant associations. Results: Positive associations with clefts were observed for only a few defects, among which anencephaly, encephaloceles, club feet, and ear anomalies were the most outstanding. Anomalies negatively associated with clefts included congenital heart defects, VATER complex (vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia), and spina bifida. Conclusion: The strong association between all types of clefts and anencephaly seems to be attributable to cases with disruptions; the association between CP and club feet seems to be attributable to conditions with fetal akinesia. Some negative associations may depend on methodologic factors, while others, such as clefts with VATER components or clefts with spina bifida, may depend on biological factors. The different patterns of defects associated with CL and CLP, indicating different underlying mechanisms, suggest that CL and CLP reflect more than just variable degrees of severity, and that distinct pathways might be involved.

scholarly journals A Child with Lung Hypoplasia, Congenital Heart Disease, Hemifacial Microsomia, and Inguinal Hernia: Ipsilateral Congenital Malformations

2015 ◽  
Vol 2015 ◽  
pp. 1-3
Author(s):  
Chengming Fan ◽  
Can Huang ◽  
Jijia Liu ◽  
Jinfu Yang
Keyword(s):  
Inguinal Hernia ◽  
Atrial Septal Defect ◽  
Congenital Malformations ◽  
Septal Defect ◽  
Heart Function ◽  
Facial Asymmetry ◽  
First Grade ◽  
Lung Hypoplasia ◽  
Indirect Inguinal Hernia ◽  
The Right

A 3-year-old Chinese boy was diagnosed with ipsilateral congenital malformations: right lung hypoplasia, dextroversion of heart, atrial septal defect, hepatic vein drainage directly into the right atrium, facial asymmetry, right microtia and congenital deafness, and indirect inguinal hernia. He underwent indirect inguinal hernia repair at the age of 2. Although without any facial plastic surgery performed, he underwent a repair of atrial septal defect and recovered uneventfully. At 6-month follow-up, the patient was free from any symptom of dyspnea; his heart function returned to the first grade.

scholarly journals Hypoplasia of ribs associated with cleft palate, cleft lip, and unilateral renal agenesis in a neonate dog of undefined breed

2019 ◽  
Vol 40 (1) ◽  
pp. 497
Author(s):  
Keylla Helena Nobre Pacifico Pereira ◽  
Caio Henrique Paganini Burini ◽  
Elton Luís Ritir Oliveira ◽  
Lucas Emanuel Ferreira Canuto ◽  
Luiz Eduardo Cruz Dos Santos Correia ◽  
...  
Keyword(s):  
Cleft Palate ◽  
Autosomal Recessive ◽  
Cleft Lip ◽  
Renal Agenesis ◽  
Present Report ◽  
Autosomal Recessive Inheritance ◽  
Normal Function ◽  
Congenital Defects ◽  
Unilateral Renal Agenesis ◽  
First Case

Congenital defects can cause changes in the normal function or morphology of organs, thus contributing to neonatal mortality. Malformations in dogs occur as a result of genetic factors or by the action of teratogenic agents during pregnancy. Genetic defects can be inherited from one or both parents. These defects are more common in purebred puppies or can even be the result of consanguinity. Teratogenic agents, such as toxins, drugs, infectious diseases, mechanical influences, and irradiation, may affect the litters during gestational development. Hypoplasia of ribs has been described in human newborns. It is a rare and lethal malformation of autosomal recessive inheritance that prevents thoracic expansion and reduces pulmonary compliance, causing respiratory failure. A pregnant bitch of undefined breed was submitted to caesarean section. At birth, a neonate exhibited respiratory distress, and the palpation of the thorax indicated absence of ribs. In addition, the newborn had cleft palate and cleft lip, which led to perform the euthanasia of the animal. Post-mortem examination indicated hypoplasia of ribs and unilateral renal agenesis. As in the canine neonate, hypoplasia of ribs in human newborns is also associated with other malformations, such as cleft lip, cleft palate, and urogenital defects. The present report describes the first case of hypoplasia of ribs associated with other malformations in a canine neonate, the cause being possibly related to a genetic hereditary factor.

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