{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,24]],"date-time":"2026-01-24T12:26:05Z","timestamp":1769257565047,"version":"3.49.0"},"reference-count":29,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2026,1,20]],"date-time":"2026-01-20T00:00:00Z","timestamp":1768867200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dentistry Journal"],"abstract":"<jats:p>Background: Congenital Insensitivity to Pain and Anhidrosis (CIPA) is a rare autosomal recessive disorder characterized by congenital analgesia, anhidrosis, and multisystem involvement affecting the musculoskeletal, cutaneous, oral, and para-oral structures. This case report describes the oral phenotype and multidisciplinary clinical management of a child with CIPA. Case Description: A 9-year-old boy presented with poor oral hygiene, multiple severely damaged teeth, masticatory difficulty, limited mouth opening, impaired bolus control, and para-oral traumatic injuries. Medical and orthopedic history indicated recurrent painless fractures, self-inflicted injuries, cutaneous scarring, and recurrent hyperpyrexia. Oral self-injury associated with CIPA was suspected and supported by the Nociception Assessment Test and Minor\u2019s Iodine\u2013Starch Test. Although the clinical findings were suggestive of CIPA, the diagnosis remained presumptive due to the absence of confirmatory molecular or histopathological testing. Management: A wearable wireless continuous temperature-monitoring device was prescribed to assist in tracking hyperpyrexia associated with CIPA (RHA-CIPA). A conservative, staged, multidisciplinary treatment was planned rather than full-mouth extraction, emphasizing prevention of dental sepsis and mitigation of future self-injury. Dental procedures were performed under local anesthesia to manage discomfort related to tactile hyperesthesia. To reduce nocturnal biting and oral trauma, a hard acrylic occlusal protector was fabricated using an intraoral scanner and a 3D-printed cast. The patient was followed for 12 months. Outcomes: At the 12-month follow-up, clinical improvement was observed, with particularly notable gains in cheek elasticity and soft tissue resilience. Conclusions: This case highlights the considerable challenges involved in the interdisciplinary management of children with CIPA, including oral self-injury prevention, limited mouth opening, and the necessity of close coordination with medical specialties. These findings are descriptive observations of a single case and do not establish efficacy or generalizability of any intervention.<\/jats:p>","DOI":"10.3390\/dj14010068","type":"journal-article","created":{"date-parts":[[2026,1,20]],"date-time":"2026-01-20T17:20:42Z","timestamp":1768929642000},"page":"68","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Multidisciplinary Approach for Dental Management of Congenital Insensitivity to Pain with Anhidrosis: Clinical Case Report with 12-Month Follow-Up"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-4669-9112","authenticated-orcid":false,"given":"Almoataz B. A. T.","family":"Abdel-bari","sequence":"first","affiliation":[{"name":"Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Tanta University, Tanta 31527, Egypt"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohamed","family":"Fawzy","sequence":"additional","affiliation":[{"name":"Department of Endodontics, Faculty of Dentistry, Tanta University, Tanta 31527, Egypt"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Khaled A.","family":"Saad","sequence":"additional","affiliation":[{"name":"Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Tanta University, Tanta 31527, Egypt"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0390-418X","authenticated-orcid":false,"given":"Hatem A.","family":"Alhadainy","sequence":"additional","affiliation":[{"name":"Department of Endodontics, Faculty of Dentistry, Tanta University, Tanta 31527, Egypt"},{"name":"Division of Endodontics, Department of Dentistry, University of North Carolina, Chapel Hill, NC 27514, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,1,20]]},"reference":[{"key":"ref_1","first-page":"26330040241292378","article-title":"Congenital insensitivity to pain with anhidrosis: A literature review and the advocacy for stem cell therapeutic interventions","volume":"5","author":"Ikrama","year":"2024","journal-title":"Ther. Adv. Rare Dis."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/0887-8994(94)90091-4","article-title":"Congenital insensitivity to pain with anhidrosis (hereditary sensory and autonomic neuropathy type IV)","volume":"11","author":"Rosemberg","year":"1994","journal-title":"Pediatr. Neurol."},{"key":"ref_3","first-page":"589852","article-title":"Update review and clinical presentation in congenital insensitivity to pain and anhidrosis","volume":"2015","author":"Ricart","year":"2015","journal-title":"Case Rep. Pediatr."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Zhang, M., Cao, X., Li, N., Duan, G., and Zhang, X. (2022). Autism spectrum disorder in a boy with congenital insensitivity to pain with anhidrosis: A case report. BMC Pediatr., 22.","DOI":"10.1186\/s12887-022-03196-3"},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Yang, K., Xu, Y., Hu, H., Li, Y., Li, Q., Luan, Y., Liu, Y., Sun, Y., Feng, Z., and Yan, Y. (2021). Investigation of a novel NTRK1 variation causing congenital insensitivity to pain with anhidrosis. Front. Genet., 12.","DOI":"10.3389\/fgene.2021.763467"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1186\/1750-1172-2-39","article-title":"Hereditary sensory and autonomic neuropathies: Types II, III, and IV","volume":"2","author":"Axelrod","year":"2007","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"13","DOI":"10.4103\/0970-5333.128880","article-title":"Congenital insensitivity to pain: Review with dental implications","volume":"28","author":"Kumar","year":"2014","journal-title":"Indian. J. Pain."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1093\/hmg\/10.3.179","article-title":"Congenital insensitivity to pain with anhidrosis (CIPA): Effect of TRKA (NTRK1) missense mutations on autophosphorylation of the receptor tyrosine kinase for nerve growth factor","volume":"10","author":"Mardy","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"ref_9","unstructured":"Adam, M.P., Everman, D.B., Mirzaa, G.M., Pagon, R.A., Wallace, S.E., and Bean, L.J.H. (2020). Congenital insensitivity to pain overview. Gene Reviews, University of Washington."},{"key":"ref_10","first-page":"412","article-title":"Congenital insensitivity to pain and anhidrosis (CIPA) syndrome: A report of 4 cases","volume":"22","author":"Daneshjou","year":"2012","journal-title":"Iran J. Pediatr."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"105636","DOI":"10.1016\/j.clineuro.2019.105636","article-title":"Congenital insensitivity to pain with anhidrosis syndrome: A series from Jordan","volume":"189","author":"Masri","year":"2020","journal-title":"Clin. Neurol. Neurosurg."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"2443","DOI":"10.1038\/s41390-024-03565-x","article-title":"Living without pain: A 10-year study of congenital insensitivity to pain with anhidrosis","volume":"97","author":"Klaitman","year":"2025","journal-title":"Pediatr. Res."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1097\/01241398-199403000-00024","article-title":"Nociceptive role of substance-P in the knee joint of a patient with congenital insensitivity to pain","volume":"14","author":"Derwin","year":"1994","journal-title":"J. Pediatr. Orthop."},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Al Amroh, H.H., Reyes, A.L., Barret Austin Hillary, J., and Al Khaffaf, W.H. (2020). Painless: A case of congenital insensitivity to pain in a 5-year-old male. Oxf. Med. Case Rep., 2020.","DOI":"10.1093\/omcr\/omaa046"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"827","DOI":"10.1007\/s00776-014-0595-2","article-title":"Skeletal complications in congenital insensitivity to pain with anhidrosis: A case series of 14 patients and review of articles published in Japanese","volume":"19","author":"Zhang","year":"2014","journal-title":"J. Orthop. Sci."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.neubiorev.2018.01.013","article-title":"NGF-dependent neurons and neurobiology of emotions and feelings: Lessons from congenital insensitivity to pain with anhidrosis","volume":"87","author":"Indo","year":"2018","journal-title":"Neurosci. Biobehav. Rev."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1111\/j.1365-263X.1996.tb00223.x","article-title":"The congenital insensitivity-to-pain syndrome (analgesia congenita): Report of a case","volume":"6","author":"Rasmussen","year":"1996","journal-title":"Int. J. Paediatr. Dent."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"44","DOI":"10.18231\/j.ijmi.2019.012","article-title":"Bifid tongue in hereditary sensory and autonomic neuropathy type IV","volume":"5","author":"Lakshman","year":"2019","journal-title":"IP Int. J. Maxillofac. Imaging."},{"key":"ref_19","first-page":"47","article-title":"Congenital insensitivity to pain with anhidrosis: Case report","volume":"11","author":"Kouvelas","year":"1989","journal-title":"Pediatr. Dent."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1213\/00000539-200202000-00008","article-title":"Anesthesia for patients with congenital insensitivity to pain and anhidrosis: A questionnaire study in Japan","volume":"94","author":"Tomioka","year":"2002","journal-title":"Anesth. Analg."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1316","DOI":"10.1213\/ANE.0000000000000912","article-title":"Anesthetic management of patients with congenital insensitivity to pain with anhidrosis: A retrospective analysis of 358 procedures performed under general anesthesia","volume":"121","author":"Zlotnik","year":"2015","journal-title":"Anesth. Analg."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1046\/j.1460-9592.2003.01235.x","article-title":"The anesthetic management of patients with congenital insensitivity to pain with anhidrosis","volume":"14","author":"Rozentsveig","year":"2004","journal-title":"Paediatr. Anaesth."},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Shmueli, M., Ling, G., Elamour, S., and Weisel, Y. (2025). Improved Outcomes in Congenital Insensitivity to Pain with Anhidrosis (CIPA) via a Multidisciplinary Clinic Model. J. Clin. Med., 14.","DOI":"10.3390\/jcm14155258"},{"key":"ref_24","doi-asserted-by":"crossref","unstructured":"Yefet, E., Shmueli, M., Givol, N., and Pikovsky, A. (2025). Oral and Maxillofacial Management in Patients With CIPA. Spec. Care Dentist., 45.","DOI":"10.1111\/scd.70073"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1111\/j.1365-2842.2008.01887.x","article-title":"Orthodontic treatment and management of limited mouth opening and oral lesions in a patient with congenital insensitivity to pain: Case report","volume":"36","author":"Paduano","year":"2009","journal-title":"J. Oral. Rehabil."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"100335","DOI":"10.1016\/j.pdj.2024.11.006","article-title":"Dental approach to a pediatric patient with congenital insensitivity to pain and anhidrosis (CIPA) syndrome: A case report","volume":"35","author":"Tozar","year":"2025","journal-title":"Pediatr. Dent. J."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1046\/j.1365-263X.1998.00061.x","article-title":"The dental problems and management of a patient suffering from congenital insensitivity to pain","volume":"8","author":"Littlewood","year":"1998","journal-title":"Int. J. Paediatr. Dent."},{"key":"ref_28","doi-asserted-by":"crossref","unstructured":"Gagnier, J.J., Kienle, G., Altman, D.G., Moher, D., Sox, H., Riley, D., and CARE Group (2013). The CARE guidelines: Consensus-based clinical case reporting guideline development. BMJ Case Rep., 2013.","DOI":"10.1136\/bcr-2013-201554"},{"key":"ref_29","unstructured":"(2025, December 07). CARE Case Report Guideline. 2013 CARE Checklist. Available online: https:\/\/www.care-statement.org\/."}],"container-title":["Dentistry Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2304-6767\/14\/1\/68\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,23]],"date-time":"2026-01-23T05:25:09Z","timestamp":1769145909000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2304-6767\/14\/1\/68"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1,20]]},"references-count":29,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,1]]}},"alternative-id":["dj14010068"],"URL":"https:\/\/doi.org\/10.3390\/dj14010068","relation":{},"ISSN":["2304-6767"],"issn-type":[{"value":"2304-6767","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1,20]]}}}