choanal atresia

Summary

Summary: A congenital abnormality that is characterized by a blocked CHOANAE, the opening between the nose and the NASOPHARYNX. Blockage can be unilateral or bilateral; bony or membranous.

Top Publications

  1. Dupé V, Matt N, Garnier J, Chambon P, Mark M, Ghyselinck N. A newborn lethal defect due to inactivation of retinaldehyde dehydrogenase type 3 is prevented by maternal retinoic acid treatment. Proc Natl Acad Sci U S A. 2003;100:14036-41 pubmed
    ..Raldh3 knockout notably causes choanal atresia (CA), which is responsible for respiratory distress and death of Raldh3-null mutants at birth...
  2. Bosman E, Penn A, Ambrose J, Kettleborough R, Stemple D, Steel K. Multiple mutations in mouse Chd7 provide models for CHARGE syndrome. Hum Mol Genet. 2005;14:3463-76 pubmed
    ..of heterozygous mutant mice revealed a range of defects with reduced penetrance, such as cleft palate, choanal atresia, septal defects of the heart, haemorrhages, prenatal death, vulva and clitoral defects and ..
  3. Rodríguez Conesa A, Etxaniz Alvarez A, Rey Calvete A, Perez Gil J, Nieto Mouronte C. [Use of a metal guide in the working channel of a fiberoptic scope to insert a tracheal tube in an infant with Treacher Collins syndrome and choanal atresia]. Rev Esp Anestesiol Reanim. 2010;57:115-8 pubmed
    ..We report the case of a 5-day-old infant boy with Treacher Collins syndrome and bilateral choanal atresia in whom we used a flexible metal guide inserted into the working channel of a fiberoptic scope...
  4. Bernstein V, Denno L. Repetitive behaviors in CHARGE syndrome: differential diagnosis and treatment options. Am J Med Genet A. 2005;133A:232-9 pubmed
    ..The student, now 19 years old, has made progress in her educational program and has been able to experience more community integration...
  5. Hartshorne T, Hefner M, Davenport S. Behavior in CHARGE syndrome: introduction to the special topic. Am J Med Genet A. 2005;133A:228-31 pubmed
    ..And, finally, all papers point to behavior as communication, especially within relationships, where it is essential for maximizing intellectual and social outcomes...
  6. Blake K, Salem Hartshorne N, Daoud M, Gradstein J. Adolescent and adult issues in CHARGE syndrome. Clin Pediatr (Phila). 2005;44:151-9 pubmed
    ..The resulting body of information provides important insights into the prognosis and special needs of individuals with CHARGE, as well as further research questions...
  7. Ramsden J, Campisi P, Forte V. Choanal atresia and choanal stenosis. Otolaryngol Clin North Am. 2009;42:339-52, x pubmed publisher
    ..The surgical management of choanal atresia varies widely in different centers...
  8. Amiel J, Attieé Bitach T, Marianowski R, Cormier Daire V, Abadie V, Bonnet D, et al. Temporal bone anomaly proposed as a major criteria for diagnosis of CHARGE syndrome. Am J Med Genet. 2001;99:124-7 pubmed
    ..Classical diagnostic criteria include: 1) one major feature namely coloboma/microphthalmia or choanal atresia, and 2) four of the six features designated in the CHARGE acronym...
  9. Smith I, Nichols S, Issekutz K, Blake K. Behavioral profiles and symptoms of autism in CHARGE syndrome: preliminary Canadian epidemiological data. Am J Med Genet A. 2005;133A:248-56 pubmed
    ..Results are discussed with reference to the challenges inherent in the diagnosis of autism in individuals with sensory impairments, and to the implications for understanding the etiology of CHARGE syndrome and of ASD...

More Information

Publications70

  1. Doyle C, Blake K. Scoliosis in CHARGE: a prospective survey and two case reports. Am J Med Genet A. 2005;133A:340-3 pubmed
  2. Pauws E, Hoshino A, Bentley L, Prajapati S, Keller C, Hammond P, et al. Tbx22null mice have a submucous cleft palate due to reduced palatal bone formation and also display ankyloglossia and choanal atresia phenotypes. Hum Mol Genet. 2009;18:4171-9 pubmed publisher
    ..We also find persistent oro-nasal membranes or, in some mice a partial rupture, resulting in choanal atresia. Each of these defects can cause severe breathing and/or feeding difficulties in the newborn pups, which ..
  3. Chalouhi C, Faulcon P, Le Bihan C, Hertz Pannier L, Bonfils P, Abadie V. Olfactory evaluation in children: application to the CHARGE syndrome. Pediatrics. 2005;116:e81-8 pubmed
    ..function in children and use it to investigate a group of children with CHARGE (coloboma, congenital heart disease, choanal atresia, mental and growth retardation, genital anomalies, and ear malformations and hearing loss) syndrome.
  4. El Sawy H, Siddiq M, Anbarasu A. Bilateral choanal atresia and paranasal sinus hypoplasia in an adult patient with hypogammaglobulinaemia. Eur Arch Otorhinolaryngol. 2006;263:1136-8 pubmed
    We present an unusual case of a young female patient presenting with bilateral choanal atresia, which was probably acquired, associated with nasal and paranasal sinus hypoplasia and hypogammaglobulinaemia.
  5. Hengerer A, Brickman T, Jeyakumar A. Choanal atresia: embryologic analysis and evolution of treatment, a 30-year experience. Laryngoscope. 2008;118:862-6 pubmed publisher
    b>Choanal atresia (CA) is a congenital obstruction of the posterior nasal apertures. Multiple surgical techniques have been proposed to repair the atresia...
  6. Barbero P, Valdez R, Rodriguez H, Tiscornia C, Mansilla E, Allons A, et al. Choanal atresia associated with maternal hyperthyroidism treated with methimazole: a case-control study. Am J Med Genet A. 2008;146A:2390-5 pubmed publisher
    ..Several case reports have suggested a relationship between the prenatal use of methimazole (MMI) and choanal atresia in the offspring. However, two epidemiological studies did not find an increased teratogenic risk for MMI...
  7. Dobbelsteyn C, Marche D, Blake K, Rashid M. Early oral sensory experiences and feeding development in children with CHARGE syndrome: a report of five cases. Dysphagia. 2005;20:89-100 pubmed
    ..The nature of these difficulties and the potential contributory factors are discussed...
  8. Cheung R, Prince M. Comparison of craniofacial skeletal characteristics of infants with bilateral choanal atresia and an age-matched normative population: computed tomography analysis. J Otolaryngol. 2001;30:173-8 pubmed
    b>Choanal atresia (CA) results from the developmental failure of the posterior nasal cavity to communicate with the nasopharynx...
  9. Hartshorne T, Grialou T, Parker K. Autistic-like behavior in CHARGE syndrome. Am J Med Genet A. 2005;133A:257-61 pubmed
    ..However, the variance for CHARGE was larger than for the normative groups, and 27.5% of those with CHARGE could be classified as autistic. The pattern of subscale scores for those with CHARGE differed from the other normative groups...
  10. Issekutz K, Graham J, Prasad C, Smith I, Blake K. An epidemiological analysis of CHARGE syndrome: preliminary results from a Canadian study. Am J Med Genet A. 2005;133A:309-17 pubmed
    ..In older individuals, bilateral posterior choanal atresia (BPCA) was predictive of the presence of the three other major criteria and of aortic arch anomalies...
  11. Brown D. CHARGE syndrome "behaviors": challenges or adaptations?. Am J Med Genet A. 2005;133A:268-72 pubmed
  12. van Dijk J, de Kort A. Reducing challenging behaviors and fostering efficient learning of children with CHARGE syndrome. Am J Med Genet A. 2005;133A:273-7 pubmed
  13. Arrington C, Cowley B, Nightingale D, Zhou H, Brothman A, Viskochil D. Interstitial deletion 8q11.2-q13 with congenital anomalies of CHARGE association. Am J Med Genet A. 2005;133A:326-30 pubmed
    ..Within the deleted region, there are approximately 150 expressed genes, one or more of which may contribute to the manifestations of CHARGE association...
  14. Aslan S, Yilmazer C, Yildirim T, Akkuzu B, Yilmaz I. Comparison of nasal region dimensions in bilateral choanal atresia patients and normal controls: a computed tomographic analysis with clinical implications. Int J Pediatr Otorhinolaryngol. 2009;73:329-35 pubmed publisher
    To determine if any difference exists between a set of nasal dimension measurements in choanal atresia patients and in a control group.
  15. Wieczorek D, Gener B, González M, Seland S, Fischer S, Hehr U, et al. Microcephaly, microtia, preauricular tags, choanal atresia and developmental delay in three unrelated patients: a mandibulofacial dysostosis distinct from Treacher Collins syndrome. Am J Med Genet A. 2009;149A:837-43 pubmed publisher
    ..CHARGE syndrome was discussed because of ear anomalies, choanal atresia, and developmental delay in our patients...
  16. Collins W, Buchman C. Bilateral semicircular canal aplasia: a characteristic of the CHARGE association. Otol Neurotol. 2002;23:233-4 pubmed
  17. Chen E, Obolensky E, Rauen K, Shaffer L, Li X. Cytogenetic and array CGH characterization of de novo 1p36 duplications and deletion in a patient with congenital cataracts, hearing loss, choanal atresia, and mental retardation. Am J Med Genet A. 2008;146A:2785-90 pubmed publisher
    We describe a 14-year-old boy with congenital bilateral cataracts, blepharophimosis, ptosis, choanal atresia, sensorineural hearing loss, short, webbed neck, poor esophageal motility, severe growth and mental retardation, skeletal ..
  18. Barbero P, Ricagni C, Mercado G, Bronberg R, Torrado M. Choanal atresia associated with prenatal methimazole exposure: three new patients. Am J Med Genet A. 2004;129A:83-6 pubmed
    We report three patients with bilateral choanal atresia in children prenatally exposed to methimazole (MMI) in order to define a MMI embryopathy clinical pattern...
  19. Vissers L, van Ravenswaaij C, Admiraal R, Hurst J, de Vries B, Janssen I, et al. Mutations in a new member of the chromodomain gene family cause CHARGE syndrome. Nat Genet. 2004;36:955-7 pubmed
    ..Sequence analysis of genes located in this region detected mutations in the gene CHD7 in 10 of 17 individuals with CHARGE syndrome without microdeletions, accounting for the disease in most affected individuals...
  20. Graham J, Rosner B, Dykens E, Visootsak J. Behavioral features of CHARGE syndrome (Hall-Hittner syndrome) comparison with Down syndrome, Prader-Willi syndrome, and Williams syndrome. Am J Med Genet A. 2005;133A:240-7 pubmed
    ..syndrome, or Hall-Hitner syndrome (HHS), has been delineated as a common syndrome that includes coloboma, choanal atresia, cranial nerve dysfunction (particularly asymmetric facial palsy and neurogenic swallowing problems), ..
  21. Burrow T, Saal H, de Alarcon A, Martin L, Cotton R, Hopkin R. Characterization of congenital anomalies in individuals with choanal atresia. Arch Otolaryngol Head Neck Surg. 2009;135:543-7 pubmed publisher
    To review a tertiary care pediatric hospital's experience with choanal atresia and stenosis (CA/S) related to associated congenital anomalies (birth defects, including minor abnormalities) and genetic disorders.
  22. Vanzieleghem B, Lemmerling M, Vermeersch H, Govaert P, Dhooge I, Meire F, et al. Imaging studies in the diagnostic workup of neonatal nasal obstruction. J Comput Assist Tomogr. 2001;25:540-9 pubmed
    ..Four groups were recognized: Group I: choanal atresia (n = 5) and choanal stenosis (n = 1); Group II: congenital nasal pyriform aperture stenosis (CNPAS) (n = 3) ..
  23. Clifton Bligh R, Wentworth J, Heinz P, Crisp M, John R, Lazarus J, et al. Mutation of the gene encoding human TTF-2 associated with thyroid agenesis, cleft palate and choanal atresia. Nat Genet. 1998;19:399-401 pubmed
    ..of mouse TTF-2 (encoded by the Titf2 gene) and that two siblings with thyroid agenesis, cleft palate and choanal atresia are homozygous for a missense mutation (Ala65Val) within its forkhead domain...
  24. Thelin J, Fussner J. Factors related to the development of communication in CHARGE syndrome. Am J Med Genet A. 2005;133A:282-90 pubmed
    ..Other factors that may be related to the development of symbolic language are also discussed...
  25. Bird L, Sivagnanam M, Taylor S, Newbury R. A new syndrome of tufting enteropathy and choanal atresia, with ophthalmologic, hematologic and hair abnormalities. Clin Dysmorphol. 2007;16:211-21 pubmed
    ..siblings are reported with a syndrome of intractable diarrhea of infancy (owing to tufting enteropathy) and choanal atresia/stenosis...
  26. Tellier A, Cormier Daire V, Abadie V, Amiel J, Sigaudy S, Bonnet D, et al. CHARGE syndrome: report of 47 cases and review. Am J Med Genet. 1998;76:402-9 pubmed
    ..patients evaluated for the frequency of major anomalies, namely coloboma (79%), heart malformation (85%), choanal atresia (57%), growth and/or mental retardation (100%), genital anomalies (34%), ear anomalies (91%), and/or deafness ..
  27. Yasar H, Ozkul M. Bilateral congenital choanal atresia in a 51-year-old woman. Am J Rhinol. 2007;21:716-8 pubmed publisher
    A 51-year-old woman with bilateral congenital choanal atresia (CCA) is presented. She had episodes of respiratory distress that were relieved by crying and worsened by breastfeeding during her newborn period...
  28. Felisati G, Meazzini M, Messina F, Tavecchia M, Farronato G. Orthopedic palatal expansion in the treatment of bilateral congenital choanal atresia: an additional tool in the long term follow up of patients?. Int J Pediatr Otorhinolaryngol. 2010;74:99-103 pubmed publisher
    ..Aim of this study is the description of the effects produced by RPE in two patients affected by Bilateral Congenital Choanal Atresia (BCCA) operated in infancy and who were scheduled for a secondary endoscopical surgical correction.
  29. Agopian A, Cai Y, Langlois P, Canfield M, Lupo P. Maternal residential atrazine exposure and risk for choanal atresia and stenosis in offspring. J Pediatr. 2013;162:581-6 pubmed publisher
    To assess the relationship between estimated residential maternal exposure to atrazine during pregnancy and the risk for choanal atresia or stenosis in offspring.
  30. Lalani S, Safiullah A, Molinari L, Fernbach S, Martin D, Belmont J. SEMA3E mutation in a patient with CHARGE syndrome. J Med Genet. 2004;41:e94 pubmed
  31. Kubba H, Bennett A, Bailey C. An update on choanal atresia surgery at Great Ormond Street Hospital for Children: preliminary results with Mitomycin C and the KTP laser. Int J Pediatr Otorhinolaryngol. 2004;68:939-45 pubmed
    We present the results of transnasal choanal atresia correction in 46 children, as an update to the published Great Ormond Street Hospital series. Females outnumbered males 2-1, and half the cases in our series were bilateral...
  32. Searle L, Graham J, Prasad C, Blake K. CHARGE syndrome from birth to adulthood: an individual reported on from 0 to 33 years. Am J Med Genet A. 2005;133A:344-9 pubmed
    ..facial and ear abnormalities were obvious in early childhood, and bilateral retinal colobomata, left choanal atresia, right congenital hip dislocation, and hypogonadism were diagnosed during the first year...
  33. Satar B, Mukherji S, Telian S. Congenital aplasia of the semicircular canals. Otol Neurotol. 2003;24:437-46 pubmed
  34. Martin D, Probst F, Fox S, Schimmenti L, Semina E, Hefner M, et al. Exclusion of PITX2 mutations as a major cause of CHARGE association. Am J Med Genet. 2002;111:27-30 pubmed
    ..Together, these data indicate that PITX2 mutations are unlikely to be a major contributing cause of the multiple anomalies present in individuals with CHARGE...
  35. Fernandez Rebollo E, Pérez O, Martinez Bouzas C, Cotarelo Pérez M, Garin I, Ruibal J, et al. Two cases of deletion 2q37 associated with segregation of an unbalanced translocation 2;21: choanal atresia leading to misdiagnosis of CHARGE syndrome. Eur J Endocrinol. 2009;160:711-7 pubmed publisher
  36. Nanni L, Ming J, Du Y, Hall R, Aldred M, Bankier A, et al. SHH mutation is associated with solitary median maxillary central incisor: a study of 13 patients and review of the literature. Am J Med Genet. 2001;102:1-10 pubmed
    ..These anomalies include short stature, pituitary insufficiency, microcephaly, choanal atresia, midnasal stenosis, and congenital nasal pyriform aperture stenosis...
  37. Prasad M, Ward R, April M, Bent J, Froehlich P. Topical mitomycin as an adjunct to choanal atresia repair. Arch Otolaryngol Head Neck Surg. 2002;128:398-400 pubmed
    To evaluate the use of topical mitomycin in choanal atresia repair to reduce the development of granulation tissue and cicatrix.
  38. Friedman N, Mitchell R, Bailey C, Albert D, Leighton S. Management and outcome of choanal atresia correction. Int J Pediatr Otorhinolaryngol. 2000;52:45-51 pubmed
    Outcome analyses of factors that may either maximize success or predict a better outcome following choanal atresia correction.
  39. Sanlaville D, Romana S, Lapierre J, Amiel J, Genevieve D, Ozilou C, et al. A CGH study of 27 patients with CHARGE association. Clin Genet. 2002;61:135-8 pubmed
    CHARGE association is a non-random occurrence of congenital malformations including coloboma, heart disease, choanal atresia, retarded growth and/or retarded development, genital hypoplasia, ear anomalies and/or deafness...
  40. Wolf D, Foulds N, Daya H. Antenatal carbimazole and choanal atresia: a new embryopathy. Arch Otolaryngol Head Neck Surg. 2006;132:1009-11 pubmed
    Carbimazole embryopathy is a recently recognized and defined phenotype. Choanal atresia; gastrointestinal anomalies, particularly esophageal atresia; athelia or hypothelia; developmental delay; hearing loss; aplasia cutis; and dysmorphic ..
  41. Panda N, Simhadri S, Ghosh S. Bilateral choanal atresia in an adult: is it compatible with life?. J Laryngol Otol. 2004;118:244-5 pubmed
    Bilateral choanal atresia is potentially life-threatening. The condition almost always presents in the new born, with alternating phases of respiratory distress and apnoea relieved by crying...
  42. Lin A, Siebert J, Graham J. Central nervous system malformations in the CHARGE association. Am J Med Genet. 1990;37:304-10 pubmed
    ..The presence of CNS malformation was most strongly associated with choanal atresia. This review demonstrates a predominance of forebrain anomalies, particularly arhinencephaly and ..
  43. Uchida K, Konishi N, Inoue M, Otake K, Kusunoki M. A case of congenital jejunal atresia associated with bilateral athelia and choanal atresia: new syndrome spectrum. Clin Dysmorphol. 2006;15:37-8 pubmed
    We present a case of bilateral choanal atresia, cleft of the posterior palate. Cardiac, respiratory and central nervous system examinations were normal...
  44. Jongmans M, Admiraal R, van der Donk K, Vissers L, Baas A, Kapusta L, et al. CHARGE syndrome: the phenotypic spectrum of mutations in the CHD7 gene. J Med Genet. 2006;43:306-14 pubmed
    CHARGE syndrome is a non-random clustering of congenital anomalies including coloboma, heart defects, choanal atresia, retarded growth and development, genital hypoplasia, ear anomalies, and deafness...
  45. Leclerc J, Leclerc J, Bernier K. Choanal atresia: long-term follow-up with objective evaluation of nasal airway and olfaction. Otolaryngol Head Neck Surg. 2008;138:43-9 pubmed publisher
    ..The purpose of this study was to determine the status of nasal airway and olfaction of adult subjects born with choanal atresia (CA)...
  46. Uri N, Greenberg E. Endoscopic repair of choanal atresia: practical operative technique. Am J Otolaryngol. 2001;22:321-3 pubmed
    b>Choanal atresia is a well-known entity with various surgical approaches...
  47. Martin D, Knez I, Rigler B. Anomalous origin of the brachiocephalic trunk from the left pulmonary artery with CHARGE syndrome. Thorac Cardiovasc Surg. 2006;54:549-51 pubmed
    ..Anomalous origin of the brachiocephalic trunk is a rare congenital malformation. This is a report about a case of an anatomic isolation of the left carotid artery with CHARGE syndrome. ..
  48. Chaudhary N, Jain A, Kapoor R, Motwani G. Bilateral complete choanal atresia in an adult woman--managed with nasal endoscopes. J Indian Med Assoc. 2010;108:109-10 pubmed
    b>Choanal atresia usually presents at birth as respiratory distress and difficulty in feeding...
  49. Schoem S. Transnasal endoscopic repair of choanal atresia: why stent?. Otolaryngol Head Neck Surg. 2004;131:362-6 pubmed
    To analyze the outcome of transnasal endoscopic repair of choanal atresia in children without stenting. Retrospective review. Academic pediatric referral center...
  50. Hamilton R, Gonzalez P, Dudgeon J, Mactier H. Visual electrophysiological findings in CHARGE syndrome with bilateral colobomas: a case report. Doc Ophthalmol. 2010;121:63-7 pubmed publisher
    ..Electrophysiology can provide valuable information on visual function when behavioural testing is limited by the child's ability to co-operate, such as during the early weeks of life. ..
  51. Thatcher A, Yu J, Kuo K. High-volume bilateral chylothorax presenting with hypoxemia and shock in a pediatric patient following tracheostomy revision: a case report. J Med Case Rep. 2015;9:235 pubmed publisher
    ..An 18-month-old white baby girl with a complex past medical history including choanal atresia, atrioventricular septal defect, failure to thrive, developmental delay, and tracheostomy dependence ..
  52. Van den Abbeele T, Francois M, Narcy P. Transnasal endoscopic treatment of choanal atresia without prolonged stenting. Arch Otolaryngol Head Neck Surg. 2002;128:936-40 pubmed
    To analyze the outcome of transnasal endoscopic repair of choanal atresia (CA) in children without prolonged nasal stenting after surgery. Retrospective study. Academic tertiary care children's hospital...
  53. Wang Q, Wang S, Lin S, Chen H, Lu Y. Transnasal endoscopic repair of acquired posterior choanal stenosis and atresia. Chin Med J (Engl). 2008;121:1101-4 pubmed
    ..There are congenital and acquired choanal atresias and many approaches have been used for their repair. We assessed the clinical effect of power instrument, endoscopic repair of acquired choanal stenosis and atresia...
  54. Scheid S, Spector A, Luft J. Tracheal cartilaginous sleeve in Crouzon syndrome. Int J Pediatr Otorhinolaryngol. 2002;65:147-52 pubmed
    ..Tracheotomy and frequent airway endoscopy coupled with a keen understanding of the shifting sites of obstruction will permit longterm survival in patients with TCS and CS...
  55. Jeftha A, Stephen L, Morkel J, Beighton P. Crouzonodermoskeletal syndrome. J Clin Pediatr Dent. 2004;28:173-6 pubmed
    ..b>Choanal atresia and hydrocephalus are other features...
  56. Parikh S, Crawford A, Do T, Roy D. Popliteal pterygium syndrome: implications for orthopaedic management. J Pediatr Orthop B. 2004;13:197-201 pubmed
    ..The popliteal web is typically the most difficult orthopaedic problem. The purpose of this report is to discuss the orthopaedic management of popliteal pterygium in eight patients...
  57. Cedin A, Peixoto Rocha J, Deppermann M, Moraes Manzano P, Murao M, Shimuta A. Transnasal endoscopic surgery of choanal atresia without the use of stents. Laryngoscope. 2002;112:750-2 pubmed
  58. Chia S, Carvalho D, Jaffe D, Pransky S. Unilateral choanal atresia in identical twins: case report and literature review. Int J Pediatr Otorhinolaryngol. 2002;62:249-52 pubmed
    b>Choanal atresia is postulated to be secondary to an abnormality of the rupture of the buccopharyngeal membrane during the embryological period...
  59. Papadimitriou A, Karapanou O, Papadopoulou A, Nicolaidou P, Fretzayas A. Congenital bilateral amazia associated with bilateral choanal atresia. Am J Med Genet A. 2009;149A:1529-31 pubmed publisher
    ..The patient's medical history was positive for bilateral complete choanal atresia that was diagnosed and corrected soon after birth...
  60. James F, Parente E, Palmer J. Management of bilateral choanal atresia in a foal. J Am Vet Med Assoc. 2006;229:1784-9 pubmed
    ..A 1-day-old Standardbred foal with a history of extreme respiratory distress after birth consistent with upper airway obstruction was evaluated. A temporary tracheostomy tube was placed by the referring veterinarian...
  61. Marina M, Gendeh B. Acquired nasal posterior choanal atresia: postradiotherapy. Med J Malaysia. 2006;61:94-6 pubmed
    Reported cases of acquired posterior choanal atresia are very few in the English literature...