The Surgical Repair of a Hyperimmunoglobulin E Syndrome Associated Ascending Aortic Aneurysm

Battaloğlu, Bektaş; Çolak, Cemil; Disli, Olcay Murat; Akça, Barış; Erdil, Nevzat; Karakurt, Cemşit

doi:10.1016/j.athoracsur.2016.06.021

Cited by 3 publications

(3 citation statements)

References 5 publications

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“…Он возникает при синдроме артериальной извитости (аномалия развития соединительной ткани с аутосомно-рецессивным типом наследования, характеризующаяся стенозами, извитостями и аневризмами магистральных сосудов) [6][7][8]; синдроме Лоуйса -Дитца и синдроме Марфана [9]; мутации гена FBLN4, кодирующего синтез белка экстрацеллюлярного матрикса -фибулина-4, который содержится в средних слоях больших артерий и вен [10]; пороках аортального клапана в сочетании с другими врожденными пороками или без них [11], синдроме Элерса -Данлоса, инфекционных поражениях аорты, аортитах различного генеза, травматическом повреждении [3]. Также артериальные аневризмы и аневризма аорты могут быть осложнениями синдрома гипериммуноглобулинемии Е, при этом утвержденной общей хирургической тактики в отношении таких пациентов нет [12]. Пренатальная диагностика при помощи фетальной эхокардиографии отчетливо выявляет аневризму восходящего отдела аорты у плода [13].…”

Section: послеоперационный периодunclassified

“…Осуществить пересадку корня аорты вместе с левой коронарной артерией было невозможно, поэтому решили протезировать участок аневризмы 28-миллиметровым протезом компании JOTEC GmbH (Хехинген, Германия) с предварительным укреплением стенки аорты на месте анастомозов двумя слоями полосок из войлока. Послеоперационный период протекал без осложнений, качество жизни в отдаленном периоде не ухудшилось [12].…”

Section: послеоперационный периодunclassified

See 1 more Smart Citation

Ascending aorta and aortic root replacement in an infant: a case report

Mironenko¹,

Rasumovsky²,

Свободов³

et al. 2021

PKiK

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We herein report the first clinical case of prosthetic replacement of the ascending aorta and aortic arch to repair a giant aneurysm in a 7-month-old child. The ascending aorta and arch replacement to the level of left subclavian artery was performed using a no. 16 Polymaille prosthesis, the brachiocephalic trunk was reimplanted into the vascular prosthesis and the kinked section of the left common carotid artery was removed, followed by reimplanting the left common carotid artery into the left subclavian artery. First, proximal anastomosis with the vascular prosthesis was created using a no. 16 Polymaille prosthesis and the vascular suture was strengthened with a Teflon strip. During circulatory arrest, the aortic arch was crossed between the orifice of the left common carotid artery and left subclavian artery, with the cut extended to the isthmus region along the small curvature of the arch. The brachiocephalic trunk was aligned and brought down, with subsequent implantation into the ascending aorta prosthesis 2 cm below the initial fixation point. In the final stage, the kinked section of the left common carotid artery was resected and the aligned left carotid artery was directly reimplanted into the left subclavian artery using end-to-side anastomosis. The patient developed tracheobronchitis and moderate heart failure during the postoperative period. The duration of mechanical ventilation was 16 hours. Infusion and antibacterial therapy were discontinued on postoperative day 8. On postoperative day 13, the patient was discharged and referred to the outpatient centre for further treatment and rehabilitation. A sufficiently large-sized prosthesis allows for further development in paediatric patients. This is facilitated by the preservation of the native aortic root with restored valve function and the formation of a bevelled distal anastomosis with a small unchanged aortic section in the isthmus region, which maintains growth potential. This first reported case of an infant demonstrates the possibility of combination interventions on the aortic arch and brachiocephalic artery during the first year of life.Received 30 January 2021. Revised 24 March 2021. Accepted 29 March 2021.Funding: The study did not have sponsorship.Conflict of interest: The authors declare no conflicts of interests.Contribution of the authors: The authors contributed equally to this article.

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Section: послеоперационный периодunclassified

Ascending aorta and aortic root replacement in an infant: a case report

Mironenko¹,

Rasumovsky²,

Свободов³

et al. 2021

PKiK

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“…One death occurred due to acute myocardial infarction with dilated coronary artery in a 13 year old male (20). Additional case reports describe symptomatic aortic and cerebral aneurysms in pediatric (21,22) and adults but with limited genetic and follow-up information. Subsequent cross-sectional studies within genetically-defined STAT3-HIES US (National Institute for Health) and French cohorts have provided convincing evidence for widespread "vasculopathy" in STAT3-HIES.…”

Section: What Is the Significance Of Vasculopathy In Stat3-hies?mentioning

confidence: 99%

Hematopoietic Stem Cell Transplantation and Vasculopathy Associated With STAT3-Dominant-Negative Hyper-IgE Syndrome

et al. 2020

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Dominant negative mutations in the transcription-factor STAT3 underlie the rare primary immunodeficiency Job's syndrome. Allogeneic Hematopoietic Stem Cell Transplantation (HSCT) has shown promise in correction of the underlying immunological defect, with one report suggesting HSCT can prevent development of wider connective tissue complications. Here, we report the case of a 26 year old male who developed an acute ST-elevation myocardial infarction due to coronary artery ectasia and thrombosis, occurring despite pediatric allogeneic HSCT for STAT3-HIES and a predicted 10-year conventional cardiovascular risk of 0.1%. Vasculopathy associated with STAT3-HIES may persist or arise following HSCT and can precipitate life-threatening complications. This has implications for counseling and vascular surveillance, and highlights the need for further studies to determine the risk, pathogenesis, and optimal management of the vasculopathy associated with STAT3-HIES.

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Orthognathic Surgical Treatment in a Patient With Hyperimmunoglobulin E Syndrome

Dibbs

Raghuram²,

Roy³

et al. 2020

Journal of Craniofacial Surgery

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Autosomal-dominant hyperimmunoglobulin E syndrome (HIES), or Job syndrome, is a rare, multisystem, primary immunodeficiency disorder. Additionally, patients may also suffer from connective tissue, dental, and bone malformations. While current management of HIES is directed at prophylactic antibiotics to prevent infections, there is limited work describing surgical considerations for these patients, particularly with respect to hardware placement. Here we report a case of a patient with HIES who underwent orthognathic surgery for maxillary advancement and mandibular setback to address his severe class III malocclusion. The patient's postoperative course was complicated by significant infection, requiring multiple operations and ultimately, hardware removal after bone healing. Although this patient ultimately had a good outcome, the role of orthognathic surgery with implant placement in patients with HIES should be approached with caution and careful consideration.

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The Surgical Repair of a Hyperimmunoglobulin E Syndrome Associated Ascending Aortic Aneurysm

Cited by 3 publications

References 5 publications

Ascending aorta and aortic root replacement in an infant: a case report

Ascending aorta and aortic root replacement in an infant: a case report

Hematopoietic Stem Cell Transplantation and Vasculopathy Associated With STAT3-Dominant-Negative Hyper-IgE Syndrome

Orthognathic Surgical Treatment in a Patient With Hyperimmunoglobulin E Syndrome

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