0General anesthesia in a patient with Hutchinson-Gilford progeria syndrome: two cases report of dental treatment in the one patient.*Correspondence: o.halushko@ukr.net1State Institution “D.F. Chebotarev Institute of Gerontology of the National Academy of Medical Sciences of Ukraine”, Kyiv, Ukraine.OriginalPaper11201812871B. Mankovskyy1, O. Halushko1*, Y. Zablotskyy2, M. Synytsyn32”Zablotskyy Clinic”, Kyiv, Ukraine.3Clinical Hospital “Feofania”, State Management of Affairs, Kyiv, Ukraine.Abstract.Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder which manifests itself as premature aging. Such patients have peculiarities of the structure of the facial skull, which makes tracheal intubation difficult. Age-related comorbidities increase the risk of general anesthesia. We describe two cases of dental treatment using general anesthesia in the child living with HGPS children. To our knowledge, this is the first report of two cases of general anesthesia with tracheal intubation in the one HGPS patient. We got the written informed consent from the patient parents for publication of this report including images.Keywords:General anesthesiaProgeria syndromeFiber optic airway management1. IntroductionHutchinson-Gilford Progeria Syndrome (HGPS) is the most well-studied accelerated aging disorder, first described by Jonathan Hutchinson in 1886 and independently by Hastings Gilford in 1897 [1]. HGPS is caused by a random mutation in the LMNA gene, which encodes the protein prelamin A [2]. Prelamin A is involved in the formation of the inner membrane (lamina) of the cell nucleus and plays an important role in deoxyribonucleic acid (DNA) replication and cell division. In the case of a mutation in the LMNA gene, its damaged analogue, progerin, is formed instead of prelamin A [3]. Progerin does not properly integrate into the lamina leading to significant disfigurement of the nucleus [4]. Mutant lamin A causes DNA damage, loss of heterochromatin, and premature aging [5].Nuclear lamina defects resulting from progerin accumulation have been directly linked to the twelve hallmarks of aging, including genomic instability, telomere attrition, epigenetic alterations, loss of proteostasis, disabled macroautophagy, deregulated nutrient sensing, mitochondrial dysfunction, cellular senescence, stem cell exhaustion, altered intercellular communication, chronic inflammation, and dysbiosis [6].Progeria is a rare disease. According to various authors, the incidence ranges from 1 in 4 million newborns [7] to 1 in 20 million newborns [8].According to the Progeria Research Foundation database, as of March 31, 2025, there are only 203 patients worldwide living with progeria and progeroid laminopathies in 51 countries. 149 of these children/young people have HGPS and the other 54 have progeroid laminopathies [9].Clinical symptoms usually appear in the first 18 months after birth, and include growth retardation, facial dysmorphic changes (long narrow nose, prominent outer ears, wrinkled skin), alopecia, loss of subcutaneous fat, bone and joint abnormalities and cardiovascular pathology [8].Children with progeria have a disproportionately small face compared to the head, abnormally protruding eyes, a narrow nasal bridge, a small jaw, malformed and crowded teeth, and micrognathia [10].Until recent years, there was no treatment for the syndrome. In the early 2000s, a novel farnesyltransferase inhibitor, lonafarnib, was found to prevent the accumulation of progerin and progerin-like proteins in the nucleus and cellular cytoskeleton. In November 2020, lonafarnib received its first approval in the United States to reduce the risk of mortality in Hutchinson-Gilford progeria syndrome [11].Without specific treatment with lonafarnib, death occurs at a median age of 14.5 years (range: 6–20 years). With specific treatment, the median life expectancy increases to approximately 18.7 years [12].In cases where patients with HGPS require surgical treatment, the anatomical and physiological characteristics of children with HGPS impose extraordinary demands on anesthetic management. We report our experience with two general anesthesia procedures in a child with HGPS who underwent general anesthesia twice for dental procedures.2. Case PresentationThe parents of patient Iryna Khymych, born in 2010, first visited the clinic in July 2018 for the treatment of multiple caries and its complications. The patient had typical symptoms for HGPS: abnormal dentation, retrognathia and micrognathia, mandibular hypoplasia, limited opening of the mouth, narrow glottic opening, high-arched palate, pointed beak-like nose due to its narrow shape and small nostrils. Skin changes were manifested by scleroderma and resulting in loss of elasticity. Joint stiffness with affected flexibility and mobility of neck alongside temporomandibular joints. The difficulty of tracheal intubation was classified as grade III of the Mallampati scale. The problem wasn’t represented by the airway difficulty only. The potential fragility of the bones required very delicate handling. The narrow mouth rim created the additional difficulties for dentists (Fig. 1). The patient had brain stroke in the past and have been taking antiplatelet therapy for secondary prevention. Despite of the condition-related changes and concomitant cerebral pathology, the patient remained a child in terms of her development stage. The girl is a talented artist and her paintings were successful in Europe.
Fig. 1Picture of patient (from open sources)
The preoperative assessments included general blood tests (complete blood count, comprehensive blood chemistry) and instrumental tests (ECG, echocardiography). All test results were within the normal diapason. The patient’s height was 100 cm and weight was 11 kg.The first treatment was performed on July 31st, 2018. Fiberoptic assistance of tracheal intubation was chosen as a method for general anesthesia. After induction anesthesia by inhalation of sevoflurane via a face mask, intravenous cannula G22 was placed. Direct laryngoscopy tracheal intubation was attempted and classified as grade III of the Cormack-Lehane scale. Fiberoptic tracheal intubation was used to prevent trauma of the facial bones. A special face mask with a hole for the fiberscope was used to support breathing during fiberoptic intubation (Fig. 2). A standard endotracheal tube size ID 5,5 mm with cuff was used to provide adequate ventilation. The local anesthetic spray was used for additional pain relief. Muscle relaxants were not used. Due to the narrow nasal passages, nasotracheal intubation was not possible. The size of the endotracheal tube that could be passed through the nose would not address the adequate ventilation needs.
Fig. 2A face mask with hole for the fiberscope
Figure 3 The patient’s monitored life parametersGeneral anesthesia was prolongated by propofol 10 mg/kg/h and fentanyl 200 µg. The operation last 4,5 hours. During the treatment, the following patient life parameters were monitoring: ECG, NIBP, SpO2, EtCO2, respiratory rate, body temperature. The indicators did not exceed the age norm (Fig. 3). The whole course of anesthesia had not any complications. After appropriate post-operative monitoring and checks, the child was discharged from the hospital and sent home accompanied by his parents.The second treatment took place on October 8th, 2020. The same method of general anesthesia was chosen (Fig. 4). The duration of anesthesia was two hours. Dental treatment and general anesthesia went without any complications.
Fig. 4Dental treatment under general anesthesia
3. DiscussionIn narrative review by Kristof Nijs et all. [13] the 13 cases of general anesthesia were described in patients living with Hutchinson-Gilford progeria syndrome. The authors point out the main problem of general anesthesia in these children – airway management due to the anatomical features of the facial skull (retrognathia and micrognathia, mandibular hypoplasia, narrow nasal passages ets.) Eight cases of general anesthesia were performed by tracheal intubation. In one case, direct laryngoscopy was accompanied by technical difficulties [14]. In one case, tracheal intubation was refused due to concerns of laryngeal edema [15].The use of laryngeal mask airway (LMA) is an alternative tracheal intubation. One general anesthesia was performed through the LMA, but had some difficulties [16]. LMA doesn’t provide complete airway protection compared to the traditional tracheal intubation. When comparing supraglottic airway protection devices, LMA of second generation (i-gel) is more convenient to use. But the i-gel occupies entire oral cavity and is unacceptable in a case of dental treatment.Two tracheal intubations were performed with fiberoptic assistance. Both cases involved dental extraction and restoration. Nasotracheal intubation was performed for the convenience of the dentists [17,18]. Fiberoptic-assisted tracheal intubation seems as an optimal method of general anesthesia when airway protection is required.ConclusionIn case of long-lasting dental treatment, general anesthesia with tracheal intubation in an optimal method for pain management.The use of fiberoptic assistance for tracheal intubation is the preferred option.To maintain ventilation during fiberoptic-assisted tracheal intubation, it is advisable to use a facial mask with a hole.Acknowledgement relating to this articleWe would like to thank the healthcare professionals and assistants of the “Zablotskyy Clinic” who participated in the treatment. Special thanks to pediatric dentist Natalia Mulko, dental surgeon Ihor Proshchyn and doctor-anesthesiologist Anatolii Serhiienko.Author ContributionB. Mankovskyy – research concept and design; final approval of the article; O. Halushko – collection and/or assembly of data; data analysis and interpretation; Y. Zablotskyy – data analysis and interpretation; critical revision of the article; M. Synytsyn – collection and/or assembly of data; writing the article.FundingNot applicable.Availability of data and materialsData and material are available from the corresponding author onreasonable request.Ethics approval and consent to participateNot applicable.Consent for publicationThe authors declare that written informed consent for publication of this Case report and any accompanying images was obtained from the patient’ family.Competing interestsThe authors declare that they have no competing interests.References:1. Cisneros B, García-Aguirre I, De Ita M, Arrieta-Cruz I, Rosas-Vargas H. Hutchinson-Gilford Progeria Syndrome: Cellular Mechanisms and Therapeutic Perspectives. Arch Med Res. 2023 Jul;54(5):102837. doi: 10.1016/j.arcmed.2023.06.002.2. Gonzalo S, Kreienkamp R, Askjaer P. Hutchinson-Gilford Progeria Syndrome: A premature aging disease caused by LMNA gene mutations. 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