Anorectal Malformation: An Atypical Association of Pierre Robin Sequence
Isolated Pierre Robin sequence (PRS) is characterized by underdeveloped jaw, backward displacement of the tongue and upper airway obstruction with or without cleft palate or be part of a known syndrome as VACTREL or CHARGE, or be associated with other malformations as congenital heart disease, lung...
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| Format: | Article |
| Language: | English |
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Cairo University, Faculty of Medicine, Department of Pediatrics
2022-01-01
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| Series: | Pediatric Sciences Journal |
| Subjects: | |
| Online Access: | https://cupsj.journals.ekb.eg/article_210833_f412fa779e2e4c8aa0c423e2e43c84bd.pdf |
| _version_ | 1797785211861204992 |
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| author | Manal M. Farouk Sherif N. Kaddah Magd Ahmed Kotb |
| author_facet | Manal M. Farouk Sherif N. Kaddah Magd Ahmed Kotb |
| author_sort | Manal M. Farouk |
| collection | DOAJ |
| description | Isolated Pierre Robin sequence (PRS) is characterized by underdeveloped jaw, backward displacement of the tongue and upper airway obstruction with or without cleft palate or be part of a known syndrome as VACTREL or CHARGE, or be associated with other malformations as congenital heart disease, lung malformations, musculoskeletal system anomalies, and hereditary progressive arthro-ophthalmopathy. We report a neonate with PRS who presented by choanal atresia, sepsis, single ventricle, large ventricular septal defect; large patent ductus arteriosus, anorectal malformation in the form of high imperforate anus and rectovesical fistula. Anorectal malformation was not previously reported in Pierre Robin sequence. We report that single ventricle and anorectal malformation are rare associations of PRS. Examination for anorectal malformation, prompt diagnosis and initiation of specific management is life-saving in any neonate and especially in those with structural malformations or deformities of PRS. |
| first_indexed | 2024-03-13T00:50:52Z |
| format | Article |
| id | doaj.art-f7ef90f0e09c462a8f96d9667c7aced4 |
| institution | Directory Open Access Journal |
| issn | 2805-279X 2682-3985 |
| language | English |
| last_indexed | 2024-03-13T00:50:52Z |
| publishDate | 2022-01-01 |
| publisher | Cairo University, Faculty of Medicine, Department of Pediatrics |
| record_format | Article |
| series | Pediatric Sciences Journal |
| spelling | doaj.art-f7ef90f0e09c462a8f96d9667c7aced42023-07-07T17:00:02ZengCairo University, Faculty of Medicine, Department of PediatricsPediatric Sciences Journal2805-279X2682-39852022-01-012110410710.21608/cupsj.2021.100238.1031Anorectal Malformation: An Atypical Association of Pierre Robin SequenceManal M. Farouk0https://orcid.org/0000-0001-6439-1790Sherif N. Kaddah1Magd Ahmed Kotb2https://orcid.org/0000-0003-2118-3793Department of Pediatric Surgery, Faculty of Medicine, Cairo University, EgyptDepartment of Pediatric Surgery, Faculty of Medicine, Cairo University, EgyptDepartment of Pediatrics, Faculty of Medicine, Cairo University, EgyptIsolated Pierre Robin sequence (PRS) is characterized by underdeveloped jaw, backward displacement of the tongue and upper airway obstruction with or without cleft palate or be part of a known syndrome as VACTREL or CHARGE, or be associated with other malformations as congenital heart disease, lung malformations, musculoskeletal system anomalies, and hereditary progressive arthro-ophthalmopathy. We report a neonate with PRS who presented by choanal atresia, sepsis, single ventricle, large ventricular septal defect; large patent ductus arteriosus, anorectal malformation in the form of high imperforate anus and rectovesical fistula. Anorectal malformation was not previously reported in Pierre Robin sequence. We report that single ventricle and anorectal malformation are rare associations of PRS. Examination for anorectal malformation, prompt diagnosis and initiation of specific management is life-saving in any neonate and especially in those with structural malformations or deformities of PRS. https://cupsj.journals.ekb.eg/article_210833_f412fa779e2e4c8aa0c423e2e43c84bd.pdfpierre robin sequenceanorectal malformationventricular septal defectcongenital heart diseaserectovesical fistula. |
| spellingShingle | Manal M. Farouk Sherif N. Kaddah Magd Ahmed Kotb Anorectal Malformation: An Atypical Association of Pierre Robin Sequence Pediatric Sciences Journal pierre robin sequence anorectal malformation ventricular septal defect congenital heart disease rectovesical fistula. |
| title | Anorectal Malformation: An Atypical Association of Pierre Robin Sequence |
| title_full | Anorectal Malformation: An Atypical Association of Pierre Robin Sequence |
| title_fullStr | Anorectal Malformation: An Atypical Association of Pierre Robin Sequence |
| title_full_unstemmed | Anorectal Malformation: An Atypical Association of Pierre Robin Sequence |
| title_short | Anorectal Malformation: An Atypical Association of Pierre Robin Sequence |
| title_sort | anorectal malformation an atypical association of pierre robin sequence |
| topic | pierre robin sequence anorectal malformation ventricular septal defect congenital heart disease rectovesical fistula. |
| url | https://cupsj.journals.ekb.eg/article_210833_f412fa779e2e4c8aa0c423e2e43c84bd.pdf |
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