{"id":"https://openalex.org/W7225620184","doi":"https://doi.org/10.1097/rc9.0000000000001188","title":"Prune perineum syndrome: an extremely rare congenital malformation \u2013 a case report emphasizing diagnostic challenges, limited therapeutic consensus, and fatal outcome","display_name":"Prune perineum syndrome: an extremely rare congenital malformation \u2013 a case report emphasizing diagnostic challenges, limited therapeutic consensus, and fatal outcome","publication_year":2026,"publication_date":"2026-11-09","ids":{"openalex":"https://openalex.org/W7225620184","doi":"https://doi.org/10.1097/rc9.0000000000001188"},"language":"en","primary_location":{"id":"doi:10.1097/rc9.0000000000001188","doi":"https://doi.org/10.1097/rc9.0000000000001188","is_oa":true,"landing_page_url":"https://doi.org/10.1097/rc9.0000000000001188","pdf_url":null,"source":{"id":"https://openalex.org/S5398819","display_name":"International Journal of Surgery Case Reports","issn_l":"2210-2612","issn":["2210-2612"],"is_oa":true,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","poland-40"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"International Journal of Surgery Case Reports","raw_type":"journal-article"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["crossref"],"open_access":{"is_oa":true,"oa_status":"gold","oa_url":"https://doi.org/10.1097/rc9.0000000000001188","any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5161330157","display_name":"Polisi Zawadi Consolate","orcid":"https://orcid.org/0009-0007-0710-0716","observed_orcids":["https://orcid.org/0009-0007-0710-0716"]},"institutions":[{"id":"https://openalex.org/I154840374","display_name":"Muhimbili University of Health and Allied Sciences","ror":"https://ror.org/027pr6c67","country_code":"TZ","type":"education","lineage":["https://openalex.org/I154840374"]},{"id":"https://openalex.org/I4210137662","display_name":"Muhimbili National Hospital","ror":"https://ror.org/02xvk2686","country_code":"TZ","type":"healthcare","lineage":["https://openalex.org/I4210137662"]}],"countries":["TZ"],"is_corresponding":false,"raw_author_name":"Polisi Zawadi Consolate","raw_affiliation_strings":["Department of Surgery, Hopital Pediatrique de Kalembelembe, Kinshasa, D.R. Congo","Department of Surgery, Muhimbili University of Health and Allied Science (MUHAS), Dar es Salaam, Tanzania","Department of Surgery, Pediatric Surgery Unit, Muhimbili National Hospital (MNH), Dar es Salaam, Tanzania"],"raw_orcid":"https://orcid.org/0009-0007-0710-0716","affiliations":[{"raw_affiliation_string":"Department of Surgery, Hopital Pediatrique de Kalembelembe, Kinshasa, D.R. Congo","institution_ids":[]},{"raw_affiliation_string":"Department of Surgery, Muhimbili University of Health and Allied Science (MUHAS), Dar es Salaam, Tanzania","institution_ids":["https://openalex.org/I154840374"]},{"raw_affiliation_string":"Department of Surgery, Pediatric Surgery Unit, Muhimbili National Hospital (MNH), Dar es Salaam, Tanzania","institution_ids":["https://openalex.org/I4210137662"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5056581757","display_name":"Neema Lala Bayyo","orcid":"https://orcid.org/0000-0001-5813-6531","observed_orcids":["https://orcid.org/0000-0001-5813-6531"]},"institutions":[{"id":"https://openalex.org/I154840374","display_name":"Muhimbili University of Health and Allied Sciences","ror":"https://ror.org/027pr6c67","country_code":"TZ","type":"education","lineage":["https://openalex.org/I154840374"]},{"id":"https://openalex.org/I4210137662","display_name":"Muhimbili National Hospital","ror":"https://ror.org/02xvk2686","country_code":"TZ","type":"healthcare","lineage":["https://openalex.org/I4210137662"]}],"countries":["TZ"],"is_corresponding":false,"raw_author_name":"Neema Lala Bayyo","raw_affiliation_strings":["Department of Surgery, Muhimbili University of Health and Allied Science (MUHAS), Dar es Salaam, Tanzania","Department of Surgery, Pediatric Surgery Unit, Muhimbili National Hospital (MNH), Dar es Salaam, Tanzania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Surgery, Muhimbili University of Health and Allied Science (MUHAS), Dar es Salaam, Tanzania","institution_ids":["https://openalex.org/I154840374"]},{"raw_affiliation_string":"Department of Surgery, Pediatric Surgery Unit, Muhimbili National Hospital (MNH), Dar es Salaam, Tanzania","institution_ids":["https://openalex.org/I4210137662"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5161334982","display_name":"Pahari Byamungu Kagenderezo","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I154840374","display_name":"Muhimbili University of Health and Allied Sciences","ror":"https://ror.org/027pr6c67","country_code":"TZ","type":"education","lineage":["https://openalex.org/I154840374"]},{"id":"https://openalex.org/I4210137662","display_name":"Muhimbili National Hospital","ror":"https://ror.org/02xvk2686","country_code":"TZ","type":"healthcare","lineage":["https://openalex.org/I4210137662"]}],"countries":["TZ"],"is_corresponding":false,"raw_author_name":"Pahari Byamungu Kagenderezo","raw_affiliation_strings":["Department of Surgery, Muhimbili University of Health and Allied Science (MUHAS), Dar es Salaam, Tanzania","Department of Surgery, Pediatric Surgery Unit, Muhimbili National Hospital (MNH), Dar es Salaam, Tanzania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Surgery, Muhimbili University of Health and Allied Science (MUHAS), Dar es Salaam, Tanzania","institution_ids":["https://openalex.org/I154840374"]},{"raw_affiliation_string":"Department of Surgery, Pediatric Surgery Unit, Muhimbili National Hospital (MNH), Dar es Salaam, Tanzania","institution_ids":["https://openalex.org/I4210137662"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5098981426","display_name":"Nimwindael Stephen Msangi","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I154840374","display_name":"Muhimbili University of Health and Allied Sciences","ror":"https://ror.org/027pr6c67","country_code":"TZ","type":"education","lineage":["https://openalex.org/I154840374"]},{"id":"https://openalex.org/I4210137662","display_name":"Muhimbili National Hospital","ror":"https://ror.org/02xvk2686","country_code":"TZ","type":"healthcare","lineage":["https://openalex.org/I4210137662"]}],"countries":["TZ"],"is_corresponding":false,"raw_author_name":"Nimwindael Stephen Msangi","raw_affiliation_strings":["Department of Radiology, Muhimbili National Hospital (MNH), Dar es Salaam, Tanzania","Department of Surgery, Muhimbili University of Health and Allied Science (MUHAS), Dar es Salaam, Tanzania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Radiology, Muhimbili National Hospital (MNH), Dar es Salaam, Tanzania","institution_ids":["https://openalex.org/I4210137662"]},{"raw_affiliation_string":"Department of Surgery, Muhimbili University of Health and Allied Science (MUHAS), Dar es Salaam, Tanzania","institution_ids":["https://openalex.org/I154840374"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5161313351","display_name":"Mohamed Sultan Salim","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I154840374","display_name":"Muhimbili University of Health and Allied Sciences","ror":"https://ror.org/027pr6c67","country_code":"TZ","type":"education","lineage":["https://openalex.org/I154840374"]},{"id":"https://openalex.org/I4210137662","display_name":"Muhimbili National Hospital","ror":"https://ror.org/02xvk2686","country_code":"TZ","type":"healthcare","lineage":["https://openalex.org/I4210137662"]}],"countries":["TZ"],"is_corresponding":false,"raw_author_name":"Mohamed Sultan Salim","raw_affiliation_strings":["Department of Surgery, Muhimbili University of Health and Allied Science (MUHAS), Dar es Salaam, Tanzania","Department of Surgery, Pediatric Surgery Unit, Muhimbili National Hospital (MNH), Dar es Salaam, Tanzania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Surgery, Muhimbili University of Health and Allied Science (MUHAS), Dar es Salaam, Tanzania","institution_ids":["https://openalex.org/I154840374"]},{"raw_affiliation_string":"Department of Surgery, Pediatric Surgery Unit, Muhimbili National Hospital (MNH), Dar es Salaam, Tanzania","institution_ids":["https://openalex.org/I4210137662"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5021775514","display_name":"Petronilla Joseph Ngiloi","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I154840374","display_name":"Muhimbili University of Health and Allied Sciences","ror":"https://ror.org/027pr6c67","country_code":"TZ","type":"education","lineage":["https://openalex.org/I154840374"]},{"id":"https://openalex.org/I4210137662","display_name":"Muhimbili National Hospital","ror":"https://ror.org/02xvk2686","country_code":"TZ","type":"healthcare","lineage":["https://openalex.org/I4210137662"]}],"countries":["TZ"],"is_corresponding":false,"raw_author_name":"Petronilla Ngiloi","raw_affiliation_strings":["Department of Surgery, Muhimbili University of Health and Allied Science (MUHAS), Dar es Salaam, Tanzania","Department of Surgery, Pediatric Surgery Unit, Muhimbili National Hospital (MNH), Dar es Salaam, Tanzania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Surgery, Muhimbili University of Health and Allied Science (MUHAS), Dar es Salaam, Tanzania","institution_ids":["https://openalex.org/I154840374"]},{"raw_affiliation_string":"Department of Surgery, Pediatric Surgery Unit, Muhimbili National Hospital (MNH), Dar es Salaam, Tanzania","institution_ids":["https://openalex.org/I4210137662"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":2,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":{"value":2090,"currency":"USD","value_usd":2090},"apc_paid":{"value":2090,"currency":"USD","value_usd":2090},"fwci":0.0,"has_fulltext":false,"cited_by_count":0,"citation_normalized_percentile":{"value":0.0,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":null,"issue":null,"first_page":null,"last_page":null},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T12237","display_name":"Congenital gastrointestinal and neural anomalies","score":0.8984000086784363,"subfield":{"id":"https://openalex.org/subfields/2746","display_name":"Surgery"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T12237","display_name":"Congenital gastrointestinal and neural anomalies","score":0.8984000086784363,"subfield":{"id":"https://openalex.org/subfields/2746","display_name":"Surgery"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11815","display_name":"Urological Disorders and Treatments","score":0.05040000006556511,"subfield":{"id":"https://openalex.org/subfields/2748","display_name":"Urology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T12716","display_name":"Congenital Anomalies and Fetal Surgery","score":0.04050000011920929,"subfield":{"id":"https://openalex.org/subfields/2746","display_name":"Surgery"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/congenital-malformations","display_name":"congenital malformations","score":0.8059999942779541},{"id":"https://openalex.org/keywords/anorectal-malformation","display_name":"anorectal malformation","score":0.9629999995231628},{"id":"https://openalex.org/keywords/perineal-mass","display_name":"perineal mass","score":0.8209999799728394},{"id":"https://openalex.org/keywords/hydronephrosis","display_name":"hydronephrosis","score":0.7799999713897705},{"id":"https://openalex.org/keywords/mri-diagnosis","display_name":"MRI diagnosis","score":0.4519999921321869},{"id":"https://openalex.org/keywords/bowel-herniation","display_name":"bowel herniation","score":0.5870000123977661},{"id":"https://openalex.org/keywords/ambiguous-genitalia","display_name":"ambiguous genitalia","score":0.6330000162124634},{"id":"https://openalex.org/keywords/pediatric-reconstructive-surgery","display_name":"pediatric reconstructive surgery","score":0.6430000066757202},{"id":"https://openalex.org/keywords/resource-limited-settings","display_name":"resource-limited settings","score":0.8479999899864197},{"id":"https://openalex.org/keywords/infant-mortality","display_name":"infant mortality","score":0.4749999940395355}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9408000111579895},{"id":"https://openalex.org/C2781163205","wikidata":"https://www.wikidata.org/wiki/Q105499","display_name":"Perineum","level":2,"score":0.89410001039505},{"id":"https://openalex.org/C141071460","wikidata":"https://www.wikidata.org/wiki/Q40821","display_name":"Surgery","level":1,"score":0.6168000102043152},{"id":"https://openalex.org/C2778766689","wikidata":"https://www.wikidata.org/wiki/Q595453","display_name":"Colostomy","level":2,"score":0.5885999798774719},{"id":"https://openalex.org/C143409427","wikidata":"https://www.wikidata.org/wiki/Q161238","display_name":"Magnetic resonance imaging","level":2,"score":0.510699987411499},{"id":"https://openalex.org/C116558999","wikidata":"https://www.wikidata.org/wiki/Q4131496","display_name":"Reconstructive surgery","level":2,"score":0.4406000077724457},{"id":"https://openalex.org/C2781244320","wikidata":"https://www.wikidata.org/wiki/Q813683","display_name":"Pelvic floor","level":2,"score":0.40610000491142273},{"id":"https://openalex.org/C527108885","wikidata":"https://www.wikidata.org/wiki/Q835153","display_name":"Physical examination","level":2,"score":0.3993000090122223},{"id":"https://openalex.org/C2776135927","wikidata":"https://www.wikidata.org/wiki/Q5242559","display_name":"Referral","level":2,"score":0.3840000033378601},{"id":"https://openalex.org/C529618451","wikidata":"https://www.wikidata.org/wiki/Q234904","display_name":"Ultrasonography","level":2,"score":0.37290000915527344},{"id":"https://openalex.org/C126838900","wikidata":"https://www.wikidata.org/wiki/Q77604","display_name":"Radiology","level":1,"score":0.351500004529953},{"id":"https://openalex.org/C2776813522","wikidata":"https://www.wikidata.org/wiki/Q1147182","display_name":"Fecal incontinence","level":2,"score":0.29789999127388},{"id":"https://openalex.org/C2777698361","wikidata":"https://www.wikidata.org/wiki/Q1779119","display_name":"Sacrococcygeal teratoma","level":4,"score":0.2802000045776367},{"id":"https://openalex.org/C2779973185","wikidata":"https://www.wikidata.org/wiki/Q497","display_name":"Anus","level":2,"score":0.27639999985694885},{"id":"https://openalex.org/C2781027925","wikidata":"https://www.wikidata.org/wiki/Q7161796","display_name":"Pelvic exenteration","level":2,"score":0.26750001311302185},{"id":"https://openalex.org/C3020547009","wikidata":"https://www.wikidata.org/wiki/Q1070952","display_name":"Histopathological examination","level":2,"score":0.26339998841285706},{"id":"https://openalex.org/C2781074409","wikidata":"https://www.wikidata.org/wiki/Q158716","display_name":"Rectum","level":2,"score":0.2621999979019165}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.1097/rc9.0000000000001188","doi":"https://doi.org/10.1097/rc9.0000000000001188","is_oa":true,"landing_page_url":"https://doi.org/10.1097/rc9.0000000000001188","pdf_url":null,"source":{"id":"https://openalex.org/S5398819","display_name":"International Journal of Surgery Case Reports","issn_l":"2210-2612","issn":["2210-2612"],"is_oa":true,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","poland-40"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"International Journal of Surgery Case Reports","raw_type":"journal-article"}],"best_oa_location":{"id":"doi:10.1097/rc9.0000000000001188","doi":"https://doi.org/10.1097/rc9.0000000000001188","is_oa":true,"landing_page_url":"https://doi.org/10.1097/rc9.0000000000001188","pdf_url":null,"source":{"id":"https://openalex.org/S5398819","display_name":"International Journal of Surgery Case Reports","issn_l":"2210-2612","issn":["2210-2612"],"is_oa":true,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","poland-40"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"International Journal of Surgery Case Reports","raw_type":"journal-article"},"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":0.98580002784729,"display_name":"Good health and well-being"}],"study_designs":[{"id":"https://openalex.org/study-designs/case-report","display_name":"Case Report"}],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":6,"referenced_works":["https://openalex.org/W2110448706","https://openalex.org/W2158546830","https://openalex.org/W3051169693","https://openalex.org/W3093935010","https://openalex.org/W3192680544","https://openalex.org/W4251655345"],"related_works":["https://openalex.org/W2315917490","https://openalex.org/W3051169693","https://openalex.org/W3036918361","https://openalex.org/W2141090212","https://openalex.org/W1996633851","https://openalex.org/W2050796528","https://openalex.org/W2407891728","https://openalex.org/W1993089150","https://openalex.org/W4324002130","https://openalex.org/W4407243523"],"abstract_inverted_index":{"Background:":[0],"Prune":[1],"perineum":[2],"syndrome":[3],"(PPS)":[4],"is":[5,31,204],"an":[6,44,54],"extremely":[7],"rare":[8],"congenital":[9],"malformation":[10],"characterized":[11],"by":[12],"a":[13,48,69,91,97,111,127,144,151,161,169,208],"large,":[14,49],"prune-like":[15,51],"perineal":[16,52,71,94,129,163],"mass":[17,95],"associated":[18,245],"with":[19,33,47,96,137,171,246],"complex":[20],"anorectal,":[21],"genitourinary,":[22],"and":[23,36,61,65,79,102,110,131,147,168,180,214,224,258],"sacrococcygeal":[24],"anomalies.":[25],"Recent":[26],"literature":[27],"suggests":[28],"that":[29],"survival":[30,265],"possible":[32],"timely":[34],"diagnosis":[35],"staged":[37],"reconstructive":[38,229,259],"surgery.":[39],"Case":[40],"presentation:":[41],"We":[42],"report":[43],"infant":[45,175],"born":[46],"redundant,":[50],"mass,":[53],"absent":[55],"anal":[56,105],"opening,":[57,106],"ambiguous":[58],"external":[59],"genitalia,":[60,109],"passage":[62],"of":[63,87,187,196,200,227,232,266],"urine":[64,101],"fecal":[66],"material":[67],"through":[68],"single":[70],"opening.":[72],"Referral":[73],"was":[74,141],"delayed":[75],"due":[76],"to":[77],"financial":[78],"logistical":[80],"limitations.":[81],"Upon":[82],"admission":[83],"at":[84,182,184],"2":[85],"months":[86,186],"age,":[88,188],"examination":[89],"demonstrated":[90,115,160],"massive,":[92],"wrinkled":[93],"large":[98,128,162],"cavity":[99,164],"draining":[100],"stool,":[103],"no":[104],"poorly":[107],"differentiated":[108],"non-palpable":[112],"coccyx.":[113],"Ultrasonography":[114],"bilateral":[116],"hydronephrosis.":[117],"Magnetic":[118],"resonance":[119],"imaging":[120,256],"(MRI)":[121],"revealed":[122],"bowel":[123,155],"loops":[124],"herniating":[125],"into":[126],"defect":[130],"dysplastic":[132],"pelvic":[133],"structures,":[134],"findings":[135],"consistent":[136],"PPS.":[138],"The":[139,174],"condition":[140],"misdiagnosed":[142],"as":[143],"cloacal":[145],"malformation,":[146],"the":[148,172,197,225,243,264],"patient":[149],"underwent":[150],"double-barrel":[152],"colostomy":[153],"for":[154,263],"diversion.":[156],"Examination":[157],"under":[158],"anesthesia":[159],"containing":[165],"ureteric":[166],"openings":[167],"communication":[170],"bowel.":[173],"later":[176],"developed":[177],"recurrent":[178],"infections":[179],"died":[181],"home":[183],"6":[185],"before":[189],"definitive":[190],"reconstruction.":[191],"Discussion:":[192],"PPS":[193,247],"represents":[194],"one":[195],"rarest":[198],"variants":[199],"anorectal":[201],"malformations.":[202],"Diagnosis":[203],"challenging.":[205],"MRI":[206],"plays":[207],"critical":[209],"role":[210],"in":[211,236,248],"anatomical":[212],"delineation":[213],"surgical":[215],"planning.":[216],"Survival":[217],"depends":[218],"on":[219],"early":[220,253],"recognition,":[221],"multidisciplinary":[222],"management,":[223],"availability":[226],"specialized":[228],"surgery,":[230],"all":[231],"which":[233],"remain":[234],"limited":[235],"low-resource":[237],"settings.":[238],"Conclusion:":[239],"This":[240],"case":[241],"highlights":[242],"challenges":[244],"resource-constrained":[249],"environments.":[250],"Increased":[251],"awareness,":[252],"referral,":[254],"improved":[255],"access,":[257],"services":[260],"are":[261],"essential":[262],"affected":[267],"children.":[268]},"counts_by_year":[],"updated_date":"2026-11-11T12:16:26.678958","created_date":"2026-11-10T00:00:00"}
