Pierścień naczyniowy
Diagnostyka i diagnoza

Pierścień naczyniowy to rzadka wrodzona wada serca (ok. 1% wad wrodzonych), w której aorta lub jej odgałęzienia tworzą strukturę uciskającą tchawicę i/lub przełyk, prowadząc do objawów oddechowych (np. stridor) i dysfagii. Najczęstsze typy to podwójny łuk aorty oraz prawy łuk aorty z aberrantną lewą tętnicą podobojczykową. Diagnostyka opiera się na wysokim podejrzeniu klinicznym, badaniu fizykalnym, zdjęciu rentgenowskim klatki piersiowej, badaniu kontrastowym przełyku (ezofagogram), echokardiografii, tomografii komputerowej (CT) i rezonansie magnetycznym (MRI). CT jest złotym standardem, umożliwiając dokładną ocenę anatomii i ucisku tchawicy oraz przełyku, a prenatalna diagnostyka echokardiograficzna i MRI pozwala na wczesne rozpoznanie i planowanie leczenia. Bronchoskopia i endoskopia górnego odcinka przewodu pokarmowego są stosowane w ocenie ciężkich objawów.

Diagnostyka pierścieni naczyniowych

Pierścień naczyniowy to wrodzona wada serca, w której aorta lub jej odgałęzienia tworzą strukturę otaczającą tchawicę i/lub przełyk, powodując ich ucisk, co może prowadzić do problemów z oddychaniem i połykaniem. Wady te stanowią około 1% wszystkich wrodzonych wad serca. Dwa najczęstsze typy pełnych pierścieni naczyniowych to podwójny łuk aorty oraz prawy łuk aorty z nieprawidłowym połączeniem lewej tętnicy podobojczykowej.12

Diagnoza pierścieni naczyniowych wymaga wysokiego poziomu podejrzenia klinicznego, ponieważ objawy mogą być zmienne i niespecyficzne. Wielu pacjentów nie wykazuje objawów lub ma łagodne dolegliwości. Pierścienie naczyniowe diagnozowane u pacjentów poniżej 6 miesięcy życia są zazwyczaj wynikiem występowania u pacjenta głośnego oddechu (stridor).34

Diagnostyka prenatalna

Coraz częściej pierścienie naczyniowe są diagnozowane prenatalnie dzięki poprawie technik obrazowania. Prenatalne rozpoznanie umożliwia zaplanowanie odpowiedniego postępowania po urodzeniu i potencjalnie wcześniejszą interwencję chirurgiczną.56

  • Echokardiografia płodowa – umożliwia wykrycie anomalii naczyniowych poprzez widok trzech naczyń i tchawicy (3VT). W badaniach wykazano wzrost wykrywalności pierścieni naczyniowych z 0% przed 2006 r. do 70% od 2013 r.78
  • Rezonans magnetyczny płodu (MRI) – może dostarczyć dodatkowych informacji, szczególnie w ocenie dróg oddechowych płodu. W przypadkach bez zwężenia tchawicy, światło tchawicy jest widoczne na całej długości, podczas gdy w przypadkach ze zwężeniem tchawicy jego światło jest niewyraźne, wykazując „utratę ciągłości”.9

Diagnoza prenatalna jest szczególnie ważna, ponieważ umożliwia wcześniejsze wykonanie operacji, co zmniejsza szkodliwe skutki ucisku pierścienia na tchawicę i przełyk przez dłuższy czas.1011

Diagnostyka postnatalna

W przypadku podejrzenia pierścienia naczyniowego po urodzeniu, lekarz przeprowadza badanie fizykalne i zadaje pytania dotyczące objawów. Badanie fizykalne może pomóc scharakteryzować „głośny oddech” i odróżnić go od innych, częstszych problemów, takich jak astma.1213

Badania obrazowe

Zdjęcie rentgenowskie klatki piersiowej jest często wykonywane jako część wstępnej oceny. Może ono pokazać, po której stronie ciała znajduje się łuk aorty oraz wszelkie zmiany w tchawicy, które mogą sugerować obecność pierścienia naczyniowego. Identyfikacja prawostronnego łuku aorty na zdjęciu rentgenowskim u dziecka z trudnościami w oddychaniu, dusznością lub dysfagią powinna zwrócić uwagę klinicysty na prawdopodobieństwo występowania pierścienia naczyniowego.1415

Należy jednak zauważyć, że zdjęcie rentgenowskie klatki piersiowej nie jest bardzo czułe w diagnostyce pierścieni naczyniowych. Około 95% pacjentów ma nieprawidłowości widoczne na zdjęciu rentgenowskim (uwięzienie powietrza, prawostronny łuk aorty), ale badania te są często interpretowane jako prawidłowe.1617

Badanie kontrastowe przełyku (ezofagogram) – większość ekspertów uważa, że jest to najważniejsze badanie u pacjentów z podejrzeniem pierścienia naczyniowego i jest diagnostyczne w zdecydowanej większości przypadków. Pacjenci z trudnościami w połykaniu powinni przejść badanie połykania baru jako część pierwszej oceny. Typowo pokaże ono nieprawidłowy ucisk środkowej części przełyku.1819

Badanie barowe wykazujące klasyczne cechy pierścienia naczyniowego, wraz ze zdjęciem rentgenowskim klatki piersiowej pokazującym prawostronny łuk aorty, czyni prawdopodobnym obecność pierścienia naczyniowego.2021

Tomografia komputerowa (CT) jest obecnie złotym standardem w ocenie i identyfikacji pierścieni naczyniowych. Badanie CT klatki piersiowej lub angiografia CT często są zlecane w celu ukazania relacji między naczyniami, tchawicą i przełykiem. Technika ta umożliwia lekarzom dokładną wizualizację anatomii naczyniowej dziecka.222324

Tomografia komputerowa ma tę zaletę, że zapewnia kompletną ocenę ucisku tchawicy i przełyku jednocześnie. Można ją przeprowadzić szybko, bez znieczulenia czy wstrzymywania oddechu, co jest szczególnie ważne u małych dzieci.2526

Rezonans magnetyczny (MRI) jest również cennym narzędziem diagnostycznym, które pokazuje położenie struktur naczyniowych, tchawiczno-oskrzelowych i przełykowych oraz ich wzajemne relacje. Jego zaletą jest brak narażenia na promieniowanie.2728

Echokardiografia jest coraz częściej stosowana w diagnostyce pierścienia naczyniowego. Badanie to ma jednak pewne ograniczenia diagnostyczne. Echokardiografia jest zawsze wskazana w celu wykluczenia współistniejących wad serca.2930

Echokardiografia może lokalizować pozycję łuku aorty i jego wzór rozgałęzienia. Lewy łuk aorty jest typowym wzorem. Prawy łuk aorty jest związany z wrodzonymi wadami serca. Podwójny łuk aorty jest rzadki, ale powoduje najwięcej objawów i prawie wszystkie wymagają operacji.31

Procedury endoskopowe

Bronchoskopia jest stosowana w ocenie dzieci z objawami niedrożności lub ucisku dróg oddechowych. Gdy objawy oddechowe są umiarkowane do ciężkich, może zostać wykonana bronchoskopia. Badanie to nie jest powszechnie wykonywane, nie ma jasno określonej czułości ani swoistości. Należy szukać tętniczych pulsacji, rumienia i obrzęku ściany tchawicy.323334

Endoskopia górnego odcinka przewodu pokarmowego – długa, elastyczna rurka z kamerą umożliwia lekarzowi zbadanie przełyku. Endoskop jest wprowadzany przez usta do gardła, a mała kamera na końcu rurki wysyła obrazy do monitora wideo.3536

Inne metody diagnostyczne

Cewnikowanie serca jest przydatne w przypadkach, gdy znane są lub podejrzewa się współistniejące nieprawidłowości serca.37

Cyfrowa angiografia subtrakcyjna (DSA) może być użytecznym narzędziem diagnostycznym, ponieważ ujawnia położenie struktur naczyniowych, tchawiczno-oskrzelowych i przełykowych oraz ich wzajemne relacje.38

Algorytm diagnostyczny

Nie istnieje znormalizowane podejście diagnostyczne. Skierowanie i obrazowanie opierają się na wzorcu objawów. Laryngologia jest często konsultowana wtórnie w związku z problemami z drogami oddechowymi i dysfagią.3940

Algorytm diagnostyczny często obejmuje:

  1. Badanie fizykalne i szczegółowy wywiad medyczny
  2. Zdjęcie rentgenowskie klatki piersiowej jako początkowe badanie obrazowe
  3. Badanie połykania baru, jeśli podejrzewa się pierścień naczyniowy
  4. Echokardiogram w celu wykluczenia towarzyszących wad serca
  5. CT lub MRI klatki piersiowej w celu dokładnego określenia anatomii i planowania leczenia chirurgicznego
  6. Bronchoskopia lub endoskopia górnego odcinka przewodu pokarmowego w zależności od głównych objawów

Diagnostyka różnicowa

Diagnoza różnicowa pierścieni naczyniowych może być trudna ze względu na niespecyficzne objawy. Należy rozważyć:41

  • Tracheomalacja wrodzona – osłabienie chrząstek tchawicy powodujące zapadanie się dróg oddechowych
  • Astma – może powodować podobne objawy oddechowe
  • Infekcje dróg oddechowych – mogą powodować stridor i trudności w oddychaniu
  • Refluks żołądkowo-przełykowy (GERD) – może powodować problemy z połykaniem i oddychaniem

Postępowanie po diagnozie

Po zdiagnozowaniu pierścienia naczyniowego, leczenie zależy od nasilenia objawów i anatomii pierścienia naczyniowego. W przypadku pacjentów objawowych, zwłaszcza z nasilonymi objawami oddechowymi, zazwyczaj zaleca się interwencję chirurgiczną.4243

Pierścienie naczyniowe, które uciskają tchawicę lub przełyk, wymagają operacji. Zabieg chirurgiczny w celu skorygowania pierścienia naczyniowego jest dość bezpieczny i często skuteczny. Operacja polega na podzieleniu pierścieni naczyniowych w celu zmniejszenia ucisku na przełyk lub tchawicę dziecka.4445

Decyzja o operacji jest wspólną decyzją wielu specjalistów. Wyniki chirurgiczne są generalnie bardzo dobre (70-90% ustąpienia objawów). Dysfagia prawie zawsze ustępuje, a utrzymujące się problemy po początkowej naprawie są często związane z anomaliami tchawicy.46

U pacjentów bezobjawowych można przyjąć podejście uważnego oczekiwania i ścisłego monitorowania w miarę wzrostu dziecka.47

Zindywidualizowane podejście diagnostyczne

Zindywidualizowane podejście diagnostyczne i terapeutyczne uwzględnia:4849

  • Wiek pacjenta – młodsi pacjenci mogą wymagać mniej inwazyjnych metod diagnostycznych
  • Ciężkość objawów – nasilenie objawów i czas prezentacji są bezpośrednio proporcjonalne do ciasnoty pierścienia
  • Typ pierścienia naczyniowego – podwójny łuk aorty często staje się objawowy, ponieważ pierścień wokół tchawicy i przełyku jest często ciasny, podczas gdy pierścienie naczyniowe spowodowane przez prawy łuk aorty z aberrantną lewą tętnicą podobojczykową mogą być bezobjawowe lub mieć tylko łagodne objawy, ponieważ pierścień jest stosunkowo luźny
  • Współistniejące anomalie – szczególnie wady serca

Znaczenie wczesnej diagnostyki

Wczesna i dokładna diagnoza pierścienia naczyniowego jest kluczem do leczenia. Im wcześniej zostanie postawiona diagnoza pierścienia naczyniowego i zastosowane odpowiednie leczenie chirurgiczne pacjentów z możliwym uciskiem, tym lepsze rokowanie.50

Kontakt z lekarzem w przypadku, gdy dziecko ma objawy pierścienia naczyniowego, jest kluczowy. Szybka diagnoza i leczenie mogą zapobiec poważnym powikłaniom.51

Operacja w przypadku bezobjawowych pacjentów skutkowała minimalną zachorowalnością szpitalną, co wspiera obecne zalecenie wczesnej naprawy chirurgicznej po skrupulatnej przedoperacyjnej ocenie związanych anomalii tchawiczo-oskrzelowych.52

Trendy i postępy w diagnostyce

Diagnostyka pierścieni naczyniowych ewoluowała na przestrzeni lat. Obecnie obserwuje się następujące trendy:5354

  • Wzrost diagnostyki prenatalnej – wprowadzenie prenatalnej diagnostyki pierścieni naczyniowych wpłynęło na praktykę kliniczną, ponieważ możemy identyfikować pacjentów, którzy pozostają bezobjawowi lub mają tylko łagodne, przejściowe objawy
  • Preferowanie badań CT i MRI – te techniki obrazowania zapewniają dokładniejsze informacje anatomiczne niż tradycyjne badania
  • Zmniejszenie roli konwencjonalnej angiografii – zastąpionej przez mniej inwazyjne metody
  • Wcześniejsza interwencja chirurgicznadiagnostyka prenatalna umożliwia wcześniejszą operację, co może poprawić długoterminowe wyniki

Wczesna interwencja chirurgiczna może być korzystniejsza ze względu na bardziej elastyczne naczynia u młodszych pacjentów oraz potencjalnie lepsze ustąpienie objawów.55

Wyzwania diagnostyczne

Mimo postępów w diagnostyce, wciąż istnieją pewne wyzwania:5657

  • Niespecyficzne objawy – mogą przypominać inne, częstsze schorzenia
  • Brak korelacji między objawami a nasileniem ucisku – podejmowanie decyzji jest dodatkowo skomplikowane przez fakt, że objawy niekoniecznie korelują z pozorną ciężkością ucisku w badaniach diagnostycznych
  • Różnorodne konfiguracje anatomiczne – różne warianty pierścieni naczyniowych wymagają indywidualnego podejścia
  • Decyzje dotyczące leczenia bezobjawowych pacjentów – obecnie większość klinicystów sugeruje, że pacjenci bez objawów nie powinni być poddawani naprawie

Diagnostyka pierścieni naczyniowych wymaga wielodyscyplinarnego podejścia, angażującego kardiologów, radiologów, chirurgów oraz, w razie potrzeby, pulmonologów i gastroenterologów.5859

Podsumowanie diagnostyki

Diagnostyka pierścieni naczyniowych wymaga wysokiego poziomu podejrzenia klinicznego i może być potwierdzona za pomocą różnych badań obrazowych i endoskopowych, takich jak tomografia komputerowa, rezonans magnetyczny, bronchoskopia, gastroskopia, echokardiografia, cewnikowanie serca i badania górnego odcinka przewodu pokarmowego.6061

Po zdiagnozowaniu pacjenta, leczenie chirurgiczne jest wymagane do dekompresji jako standardowa terapia. Podejście wielodyscyplinarne, angażujące kardiologów, radiologów, chirurgów i innych specjalistów, jest kluczowe dla optymalnego zarządzania tymi rzadkimi, ale potencjalnie poważnymi anomaliami naczyniowymi.62

Dokładne badanie i ocena anatomii są korzystne w odniesieniu do decyzji o tym, jak powinna być przeprowadzona operacja. Celem operacji jest złagodzenie ucisku tchawicy i/lub przełyku poprzez podzielenie przemieszczonego naczynia przy jednoczesnym zapewnieniu wystarczającego przepływu krwi w kierunku głowy. Wyniki po operacji są zadowalające i prowadzą do bezproblemowego długoterminowego przeżycia.63

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  1. 09.04.2026
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Materiały źródłowe

  • #1 Vascular Ring Anomaly | Symptoms, Diagnosis & Treatment
    https://www.cincinnatichildrens.org/health/v/vascular-ring
    A vascular ring is a type of congenital (present at birth) defect. This happens when normal vessels are in the wrong place. This may cause pressure on the esophagus or airway. […] The two most common types of true vascular rings are persistent double aortic arch and right aortic arch with abnormal connection of the left subclavian artery. […] Many patients do not have symptoms or have mild symptoms. Vascular rings diagnosed in patients less than 6 months of age are usually a result of a patient having noisy breathing (stridor). […] Diagnosis of Vascular Rings […] Physical examination may help characterize the „noisy breathing.” This may help separate it from other more common problems such as asthma. […] A chest X-ray is often done as a part of the initial evaluation. If the aortic arch is on the right side, a vascular ring should be suspected.
  • #2 Vascular Ring; Spectrum of Pathologies and Scenarios for Diagnosis and Management, International Journal of Cardiovascular and Thoracic Surgery, Science Publishing Group
    https://www.sciencepublishinggroup.com/article/10.11648/j.ijcts.20200606.12
    Vascular rings represent approximately 1% of all congenital cardiac anomalies, with Edwards classification being the first to outline them into a complete or partial vascular ring. A Complete ring is a combination of patent vessels, atretic vascular segments or ligamentous structures. […] We reviewed patients` records from our Pediatric Cardiac Surgery Unit, over 10 years. We encountered 63 patients from January 2009 to January 2019. Our patients were 57 (90.5%) complete vascular rings, 5 (7.9%) pulmonary artery slings, and 1 (1.6%) patient with innominate artery compression. […] Vascular ring is a rare anomaly that needs suspicion for diagnosis. The operative strategy relies on extensive dissection and division of the non-dominant arch and division of the ligamentum, freeing all fibrous bands between the arch and esophagus and trachea. For augmented results; we need collaboration from pediatricians and pulmonologists who must be aware of such anomalies.
  • #3 Vascular Ring Anomaly | Symptoms, Diagnosis & Treatment
    https://www.cincinnatichildrens.org/health/v/vascular-ring
    A vascular ring is a type of congenital (present at birth) defect. This happens when normal vessels are in the wrong place. This may cause pressure on the esophagus or airway. […] The two most common types of true vascular rings are persistent double aortic arch and right aortic arch with abnormal connection of the left subclavian artery. […] Many patients do not have symptoms or have mild symptoms. Vascular rings diagnosed in patients less than 6 months of age are usually a result of a patient having noisy breathing (stridor). […] Diagnosis of Vascular Rings […] Physical examination may help characterize the „noisy breathing.” This may help separate it from other more common problems such as asthma. […] A chest X-ray is often done as a part of the initial evaluation. If the aortic arch is on the right side, a vascular ring should be suspected.
  • #4 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology
    https://emedicine.medscape.com/article/426233-overview
    A vascular ring is an unusual congenital condition in which the anomalous configuration of the arch, associated vessels, or both surrounds the trachea and esophagus, forming a complete or incomplete ring around them. […] Prompt diagnosis and treatment of these congenital abnormalities can be lifesaving. […] For healthcare providers who look after pediatric patients, a high degree of suspicion, coupled with the appropriate imaging study, is required to make the diagnosis. A multimodality imaging approach (eg, with magnetic resonance imaging [MRI], computed tomography [CT], and esophagography) is often required for correct diagnosis (see Workup). […] Surgical division of a vascular ring is indicated in all symptomatic patients. To avoid serious complications, surgery should not be delayed, especially in patients with symptoms of airway compression. Individuals who have no symptoms from a vascular ring may not require surgical intervention. Surgical repairs in most cases can be performed successfully and without long-term morbidity (see Treatment).
  • #5 Editorial on “vascular ring diagnosis and management: notable trends over 25 years” – Savla – Translational Pediatrics
    https://tp.amegroups.org/article/view/14191/html
    Vascular rings occur due to some perturbation in the normal sequence of development and selective involution of paired aortic arches to form a definitive left or right aortic arch. The two most common types of vascular rings are double aortic arch (DAA) and right aortic arch with an aberrant left subclavian artery and a left patent ductus arteriosus or ligamentum arteriosum (RAA-ALS). The prevalence of vascular rings is unknown because many patients remain asymptomatic and are undiagnosed. […] A recently published study by Evans and colleagues reviewed the diagnosis and management of vascular rings before and after the introduction of prenatal diagnosis. […] As indicated in the study, after the three-vessel fetal echocardiography view was introduced in 2004, the rate of prenatal detection increased significantly from 0% before 2006, to 55% in 2010, and then up to 70% prenatal detection of diagnosed vascular rings since 2013.
  • #6 Expertise in the nuances of diagnosis, management and surgery for vascular rings – Mayo Clinic
    https://www.mayoclinic.org/medical-professionals/pediatrics/news/expertise-in-the-nuances-of-diagnosis-management-and-surgery-for-vascular-rings/mac-20530282
    Vascular rings are a group of diagnoses present at birth caused by abnormal development of the brachial arch system, which results in a ring of vessels encircling and constricting the airway and esophagus. […] Diagnosis of vascular rings requires: […] CT scan, on which a vascular ring would be evident. Frequently the diagnosis of a vascular ring occurs when a CT scan is performed for another indication. […] Increasingly, vascular rings are diagnosed during imaging conducted during pregnancy. „With a fetal diagnosis, we have the opportunity to perform surgery much earlier, reducing the deleterious effects of the ring compressing the trachea and esophagus for a longer time,” says Elizabeth H. Stephens, M.D., Ph.D., a pediatric and congenital heart surgeon at Mayo Clinic Children’s Center.
  • #7 Editorial on “vascular ring diagnosis and management: notable trends over 25 years” – Savla – Translational Pediatrics
    https://tp.amegroups.org/article/view/14191/html
    Vascular rings occur due to some perturbation in the normal sequence of development and selective involution of paired aortic arches to form a definitive left or right aortic arch. The two most common types of vascular rings are double aortic arch (DAA) and right aortic arch with an aberrant left subclavian artery and a left patent ductus arteriosus or ligamentum arteriosum (RAA-ALS). The prevalence of vascular rings is unknown because many patients remain asymptomatic and are undiagnosed. […] A recently published study by Evans and colleagues reviewed the diagnosis and management of vascular rings before and after the introduction of prenatal diagnosis. […] As indicated in the study, after the three-vessel fetal echocardiography view was introduced in 2004, the rate of prenatal detection increased significantly from 0% before 2006, to 55% in 2010, and then up to 70% prenatal detection of diagnosed vascular rings since 2013.
  • #8
    https://journals.lww.com/aopc/fulltext/2021/14030/prenatal_diagnosis_of_vascular_rings_and_outcome.16.aspx
    Vascular rings (VRs) present with varied symptoms and may result in significant morbidity before an accurate diagnosis is made. Prenatal diagnosis may be useful to plan surgery after birth. […] The purpose of the study was to see the feasibility of accurate diagnosis of VR during antenatal ultrasound examination and describe their outcome. […] Vascular rings were diagnosed on the basis of three vessel tracheal view and neck vessels arrangements on fetal echocardiogram. […] Fetal echocardiography enables prenatal diagnosis and planning of postnatal repair of VRs. […] The diagnosis of VR was confirmed in 96% of our postnatal cases. Prenatal diagnosis is possible if the fetal echocardiogram is performed meticulously by an expert. […] A systematic evaluation of three-vessel view and a high thoracic section of the fetus will give essential clues to the diagnosis. […] The prenatal diagnosis appears to be useful in the proper planning of surgical management before they become symptomatic.
  • #9 Tracheal stenosis due to vascular rings: its possible prenatal diagnosis based on four cases of vascular rings with or without eventual tracheal stenosis
    https://www.imrpress.com/journal/CEOG/46/5/10.12891/ceog4787.2019/htm
    The present authors confirmed the efficacy of the 3VT view to detect vascular rings. […] Fetal MRI may also provide another additional finding: in cases without tracheal stenosis, an intact tracheal lumen could be easily followed for its entire tracheal length, whereas in a case with tracheal stenosis, its lumen was obscure, showing „loss of continuity”.
  • #10 Expertise in the nuances of diagnosis, management and surgery for vascular rings – Mayo Clinic
    https://www.mayoclinic.org/medical-professionals/pediatrics/news/expertise-in-the-nuances-of-diagnosis-management-and-surgery-for-vascular-rings/mac-20530282
    Vascular rings are a group of diagnoses present at birth caused by abnormal development of the brachial arch system, which results in a ring of vessels encircling and constricting the airway and esophagus. […] Diagnosis of vascular rings requires: […] CT scan, on which a vascular ring would be evident. Frequently the diagnosis of a vascular ring occurs when a CT scan is performed for another indication. […] Increasingly, vascular rings are diagnosed during imaging conducted during pregnancy. „With a fetal diagnosis, we have the opportunity to perform surgery much earlier, reducing the deleterious effects of the ring compressing the trachea and esophagus for a longer time,” says Elizabeth H. Stephens, M.D., Ph.D., a pediatric and congenital heart surgeon at Mayo Clinic Children’s Center.
  • #11 Vascular ring: prenatal diagnosis and prognostic management based on sequential cross-sectional scanning by ultrasound | BMC Pregnancy and Childbirth | Full Text
    https://bmcpregnancychildbirth.biomedcentral.com/articles/10.1186/s12884-023-05637-y
    In terms of embryonic origin, vascular ring is a congenital anomaly in which the aortic arch and its branches completely or incompletely encircle and compress the trachea or esophagus. Early and accurate diagnosis of a vascular ring is the key to treatment. Prenatal diagnosis mainly relies on fetal echocardiography, but the rate of missed diagnosis and misdiagnosis is still very high, and the prognosis has not been evaluated. The aim of this study was to investigate the accuracy of prenatal diagnosis and to evaluate the prognosis semi-quantitatively according to the shape of the ring and the distance between the vessel and the trachea. […] A total of 418 cases of vascular rings were detected. There was no missed diagnoses or misdiagnoses by SCS. The vessels formed different shaped rings according to their origin and route. Grade I, and O rings have a poor prognosis and are associated with the highest risk of respiratory symptoms.
  • #12 Vascular Ring Anomaly | Symptoms, Diagnosis & Treatment
    https://www.cincinnatichildrens.org/health/v/vascular-ring
    A vascular ring is a type of congenital (present at birth) defect. This happens when normal vessels are in the wrong place. This may cause pressure on the esophagus or airway. […] The two most common types of true vascular rings are persistent double aortic arch and right aortic arch with abnormal connection of the left subclavian artery. […] Many patients do not have symptoms or have mild symptoms. Vascular rings diagnosed in patients less than 6 months of age are usually a result of a patient having noisy breathing (stridor). […] Diagnosis of Vascular Rings […] Physical examination may help characterize the „noisy breathing.” This may help separate it from other more common problems such as asthma. […] A chest X-ray is often done as a part of the initial evaluation. If the aortic arch is on the right side, a vascular ring should be suspected.
  • #13 Vascular rings – Overview – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/vascular-rings/cdc-20389579
    A vascular ring occurs when a part of the body’s main artery, called the aortic arch, or its branches form a ring around the trachea or esophagus. […] A vascular ring is a heart condition present at birth. That means it’s a congenital heart defect. In this condition, part of the body’s main artery or its branches form a complete ring around the windpipe and the food swallowing tube. […] A vascular ring can affect breathing and swallowing. Surgery is usually needed to treat a vascular ring. […] Congenital heart conditions such as vascular rings often are diagnosed before or soon after a child is born. […] To diagnose a vascular ring, a healthcare professional does a physical exam and asks questions about the symptoms. […] Tests to diagnose a vascular ring may include: Chest X-ray. A chest X-ray is a picture of the heart and lungs. It can show changes in the windpipe that might suggest a vascular ring. The test also can tell which side of the body the aortic arch is on. […] Surgery usually is done to treat a vascular ring that presses against the trachea or esophagus. Surgery also helps prevent complications.
  • #14 Vascular Ring Anomaly | Symptoms, Diagnosis & Treatment
    https://www.cincinnatichildrens.org/health/v/vascular-ring
    A vascular ring is a type of congenital (present at birth) defect. This happens when normal vessels are in the wrong place. This may cause pressure on the esophagus or airway. […] The two most common types of true vascular rings are persistent double aortic arch and right aortic arch with abnormal connection of the left subclavian artery. […] Many patients do not have symptoms or have mild symptoms. Vascular rings diagnosed in patients less than 6 months of age are usually a result of a patient having noisy breathing (stridor). […] Diagnosis of Vascular Rings […] Physical examination may help characterize the „noisy breathing.” This may help separate it from other more common problems such as asthma. […] A chest X-ray is often done as a part of the initial evaluation. If the aortic arch is on the right side, a vascular ring should be suspected.
  • #15 Vascular Rings Workup: Chest Radiography, Barium Esophagography, Echocardiography and Color-Flow Doppler
    https://emedicine.medscape.com/article/426233-workup
    Because children usually present with symptoms of respiratory difficulty, chest radiography is always the first and most commonly performed test. […] The identification of a right aortic arch on chest radiograph in a child with airway difficulties, respiratory distress or dysphagia should alert the clinician to the likelihood of a vascular ring. […] In general, chest radiography is not very sensitive in the diagnosis of vascular rings. […] Most authorities consider barium esophagography to be the most important study in patients with a suspected vascular ring, and it is diagnostic in the vast majority of cases. […] Echocardiographic studies have been increasingly used for the diagnosis of a vascular ring. […] This study has some diagnostic limitations. […] Computed tomography (CT), magnetic resonance imaging (MRI), and digital subtraction angiography (DSA) can be useful diagnostic tools because they reveal the positions of vascular, tracheobronchial, and esophageal structures and their relations to one another.
  • #16 Vascular Rings Workup: Chest Radiography, Barium Esophagography, Echocardiography and Color-Flow Doppler
    https://emedicine.medscape.com/article/426233-workup
    Because children usually present with symptoms of respiratory difficulty, chest radiography is always the first and most commonly performed test. […] The identification of a right aortic arch on chest radiograph in a child with airway difficulties, respiratory distress or dysphagia should alert the clinician to the likelihood of a vascular ring. […] In general, chest radiography is not very sensitive in the diagnosis of vascular rings. […] Most authorities consider barium esophagography to be the most important study in patients with a suspected vascular ring, and it is diagnostic in the vast majority of cases. […] Echocardiographic studies have been increasingly used for the diagnosis of a vascular ring. […] This study has some diagnostic limitations. […] Computed tomography (CT), magnetic resonance imaging (MRI), and digital subtraction angiography (DSA) can be useful diagnostic tools because they reveal the positions of vascular, tracheobronchial, and esophageal structures and their relations to one another.
  • #17 Vascular ring | Iowa Head and Neck Protocols
    https://medicine.uiowa.edu/iowaprotocols/vascular-ring
    All congenital vascular anomalies that cause compression of the trachea and/or esophagus. Complete vascular ring: Completely encircles trachea/esophagus. Incomplete ring: everything else. […] No standardized diagnostic work-up. Referral and imaging based on symptom pattern. Otolaryngology often consulted secondary to airway concerns and dysphagia. […] CXR 95% have abnormalities (air trapping, right sided aortic arch). Often read as normal. […] Barium Swallow Pro: Historical test of choice in combination with angiography. Lateral view will show indentations on the posterior wall of the esophagus. May rule out vascular ring. […] Echocardiogram Pro: Highly sensitive. No radiation may work well as initial imaging test. […] Prenatal echocardiogram (Evans 2016) Increasing importance in diagnosis.
  • #18 Vascular Ring Anomaly | Symptoms, Diagnosis & Treatment
    https://www.cincinnatichildrens.org/health/v/vascular-ring
    Patients with swallowing difficulties should complete a barium swallow study as part of the first evaluation. This will typically show abnormal compression of the middle part of the esophagus. […] A barium study that shows classic features of a vascular ring, along with a chest X-ray showing a right-sided aortic arch makes it likely that a vascular ring is present. […] A chest CT or MRI will often be ordered to show the relationship of the vessels, the trachea and the esophagus. […] When breathing symptoms are moderate to severe a bronchoscopy may be done.
  • #19 Vascular Rings Workup: Chest Radiography, Barium Esophagography, Echocardiography and Color-Flow Doppler
    https://emedicine.medscape.com/article/426233-workup
    Because children usually present with symptoms of respiratory difficulty, chest radiography is always the first and most commonly performed test. […] The identification of a right aortic arch on chest radiograph in a child with airway difficulties, respiratory distress or dysphagia should alert the clinician to the likelihood of a vascular ring. […] In general, chest radiography is not very sensitive in the diagnosis of vascular rings. […] Most authorities consider barium esophagography to be the most important study in patients with a suspected vascular ring, and it is diagnostic in the vast majority of cases. […] Echocardiographic studies have been increasingly used for the diagnosis of a vascular ring. […] This study has some diagnostic limitations. […] Computed tomography (CT), magnetic resonance imaging (MRI), and digital subtraction angiography (DSA) can be useful diagnostic tools because they reveal the positions of vascular, tracheobronchial, and esophageal structures and their relations to one another.
  • #20 Vascular Ring Anomaly | Symptoms, Diagnosis & Treatment
    https://www.cincinnatichildrens.org/health/v/vascular-ring
    Patients with swallowing difficulties should complete a barium swallow study as part of the first evaluation. This will typically show abnormal compression of the middle part of the esophagus. […] A barium study that shows classic features of a vascular ring, along with a chest X-ray showing a right-sided aortic arch makes it likely that a vascular ring is present. […] A chest CT or MRI will often be ordered to show the relationship of the vessels, the trachea and the esophagus. […] When breathing symptoms are moderate to severe a bronchoscopy may be done.
  • #21 What Are Vascular Rings?
    https://www.icliniq.com/articles/heart-circulatory-health/vascular-rings
    When normal vessels are positioned incorrectly, vascular rings occur, and as a result, the esophagus or airway may get compressed. […] The child’s doctor might recommend testing to see if they exhibit signs of a vascular ring, such as a weak pulse or noisy breathing. […] A physical examination could be useful in identifying „noisy breathing.” […] All that is required to proceed with surgery is a chest X-ray demonstrating a right-sided aortic arch and a barium study demonstrating the typical characteristics of a vascular ring. […] Computed tomography (CT) scanning is the gold standard for evaluating and identifying vascular rings. This method enables medical professionals to see the vascular structure of the child accurately. A diagnosis of vascular rings can be made at any age, even when the child is still in the womb.
  • #22 Vascular Ring Anomaly | Symptoms, Diagnosis & Treatment
    https://www.cincinnatichildrens.org/health/v/vascular-ring
    Patients with swallowing difficulties should complete a barium swallow study as part of the first evaluation. This will typically show abnormal compression of the middle part of the esophagus. […] A barium study that shows classic features of a vascular ring, along with a chest X-ray showing a right-sided aortic arch makes it likely that a vascular ring is present. […] A chest CT or MRI will often be ordered to show the relationship of the vessels, the trachea and the esophagus. […] When breathing symptoms are moderate to severe a bronchoscopy may be done.
  • #23 Five things parents should know about vascular rings – Boston Children’s Answers
    https://answers.childrenshospital.org/five-things-parents-should-know-about-vascular-rings/
    If your child has a high-pitched cough, breathes loudly, wheezes, has difficulty eating or swallowing, or always seems to be coming down with pneumonia or respiratory infections, the problem could be more complex than you realize. […] Although rare, abnormal formations of the blood vessels called vascular rings can compress the trachea, esophagus, or both. […] The gold standard for evaluating and identifying vascular rings is a computed tomography (CT) scan, says Dr. Jennings. This technique allows physicians to visualize your child’s vascular anatomy properly. Vascular rings can be diagnosed at any age, including while a child is still in the womb. […] Children who have symptoms from vascular rings usually need to surgery to relieve pressure on the airway and esophagus. […] Surgeons may also treat tracheomalacia at this time. […] Boston Children’s is the only hospital to address all of these problems in one comprehensive surgical repair.
  • #24 What Are Vascular Rings?
    https://www.icliniq.com/articles/heart-circulatory-health/vascular-rings
    When normal vessels are positioned incorrectly, vascular rings occur, and as a result, the esophagus or airway may get compressed. […] The child’s doctor might recommend testing to see if they exhibit signs of a vascular ring, such as a weak pulse or noisy breathing. […] A physical examination could be useful in identifying „noisy breathing.” […] All that is required to proceed with surgery is a chest X-ray demonstrating a right-sided aortic arch and a barium study demonstrating the typical characteristics of a vascular ring. […] Computed tomography (CT) scanning is the gold standard for evaluating and identifying vascular rings. This method enables medical professionals to see the vascular structure of the child accurately. A diagnosis of vascular rings can be made at any age, even when the child is still in the womb.
  • #25 Vascular ring | Iowa Head and Neck Protocols
    https://medicine.uiowa.edu/iowaprotocols/vascular-ring
    Conventional angiography Pro: High resolution. Complex relationships. Real time information. […] Computed tomography (CT) scan Pro: Complete assessment of tracheal and esophageal compression simultaneously. Can be performed fast without anesthesia of breath holding. […] Magnetic Resonance Angiography (MRA) Pro: No radiation exposure. Provides detailed relationships. […] Endoscopy Not universally performed, no clear sensitivity or specificity. Look for arterial pulsations, erythema and edema of the tracheal wall.
  • #26 Computed tomography in the evaluation of vascular rings and slings | Insights into Imaging | Full Text
    https://insightsimaging.springeropen.com/articles/10.1007/s13244-014-0343-3
    Vascular rings can be identified by several imaging examinations and occasionally multiple imaging tests may be required to make a diagnosis. […] CT has emerged as the preferred imaging examination for the diagnosis and characterisation of vascular rings. […] CT angiography (CTA) for the evaluation of vascular rings can be performed without any sedation and with quiet breathing since the latest CT scanners have fast gantry rotation times and high z axis coverage, as a result of which artefacts are minimal. […] A vascular ring is defined by encirclement of the trachea and oesophagus by the aorta, arch branch vessels, pulmonary artery, ductus arteriosus or ligamentum arteriosum. […] A ring is complete or true when there is encirclement on all sides, while it is incomplete or partial when at least one side is not involved.
  • #27 Vascular Rings Workup: Chest Radiography, Barium Esophagography, Echocardiography and Color-Flow Doppler
    https://emedicine.medscape.com/article/426233-workup
    Because children usually present with symptoms of respiratory difficulty, chest radiography is always the first and most commonly performed test. […] The identification of a right aortic arch on chest radiograph in a child with airway difficulties, respiratory distress or dysphagia should alert the clinician to the likelihood of a vascular ring. […] In general, chest radiography is not very sensitive in the diagnosis of vascular rings. […] Most authorities consider barium esophagography to be the most important study in patients with a suspected vascular ring, and it is diagnostic in the vast majority of cases. […] Echocardiographic studies have been increasingly used for the diagnosis of a vascular ring. […] This study has some diagnostic limitations. […] Computed tomography (CT), magnetic resonance imaging (MRI), and digital subtraction angiography (DSA) can be useful diagnostic tools because they reveal the positions of vascular, tracheobronchial, and esophageal structures and their relations to one another.
  • #28 Vascular ring | Iowa Head and Neck Protocols
    https://medicine.uiowa.edu/iowaprotocols/vascular-ring
    Conventional angiography Pro: High resolution. Complex relationships. Real time information. […] Computed tomography (CT) scan Pro: Complete assessment of tracheal and esophageal compression simultaneously. Can be performed fast without anesthesia of breath holding. […] Magnetic Resonance Angiography (MRA) Pro: No radiation exposure. Provides detailed relationships. […] Endoscopy Not universally performed, no clear sensitivity or specificity. Look for arterial pulsations, erythema and edema of the tracheal wall.
  • #29 Vascular Rings Workup: Chest Radiography, Barium Esophagography, Echocardiography and Color-Flow Doppler
    https://emedicine.medscape.com/article/426233-workup
    Because children usually present with symptoms of respiratory difficulty, chest radiography is always the first and most commonly performed test. […] The identification of a right aortic arch on chest radiograph in a child with airway difficulties, respiratory distress or dysphagia should alert the clinician to the likelihood of a vascular ring. […] In general, chest radiography is not very sensitive in the diagnosis of vascular rings. […] Most authorities consider barium esophagography to be the most important study in patients with a suspected vascular ring, and it is diagnostic in the vast majority of cases. […] Echocardiographic studies have been increasingly used for the diagnosis of a vascular ring. […] This study has some diagnostic limitations. […] Computed tomography (CT), magnetic resonance imaging (MRI), and digital subtraction angiography (DSA) can be useful diagnostic tools because they reveal the positions of vascular, tracheobronchial, and esophageal structures and their relations to one another.
  • #30 Case Based Pediatrics Chapter
    https://www.hawaii.edu/medicine/pediatrics/pedtext/s07c07.html
    Evaluation of suspected vascular rings can include a chest x-ray, esophagram, echocardiogram and a CT or MRI. […] Echocardiography is always indicated to exclude associated intracardiac defects. […] Vascular rings are surgically corrected if the patient is symptomatic. […] In summary, the diagnosis of a vascular ring or pulmonary sling requires a high index of suspicion. CXR and esophagram may be used for the initial work up, but further anatomic details are obtained by echocardiography, CT/MRI, and bronchoscopy for tracheal anomalies. Early surgical correction is safe with excellent outcomes.
  • #31 Vascular Ring: Does Your Child Have the Symptoms? – Urgent Heart Clinic for Kids and Adults- Dr. Nancy Hua Pediatric Cardiologist
    https://pedshappyhearts.com/2023/12/13/vascular-ring-what-parents-need-to-know/
    Healthcare professionals diagnose vascular rings through physical examination and imaging tests such as echocardiogram (heart ultrasound), X-rays, CT scans, or MRI scans. These tests help to identify the extent of the condition so that proper treatment can be administered. […] A chest X-ray is a simple and logical starting point for the imaging algorithm. It provides information regarding not only the position of the aortic arch but also the pulmonary complications of the anomaly. […] Healthcare professionals always indicate an echocardiogram with aortic arch anomalies to exclude any associated intracardiac anomaly. It can locate the aortic arch’s position and its branching pattern. The left aortic arch is the typical pattern. The right aortic arch is associated with congenital cardiac defects. Double aortic arch is rare but causes the most symptoms, and almost all require surgery.
  • #32 Vascular Ring Anomaly | Symptoms, Diagnosis & Treatment
    https://www.cincinnatichildrens.org/health/v/vascular-ring
    Patients with swallowing difficulties should complete a barium swallow study as part of the first evaluation. This will typically show abnormal compression of the middle part of the esophagus. […] A barium study that shows classic features of a vascular ring, along with a chest X-ray showing a right-sided aortic arch makes it likely that a vascular ring is present. […] A chest CT or MRI will often be ordered to show the relationship of the vessels, the trachea and the esophagus. […] When breathing symptoms are moderate to severe a bronchoscopy may be done.
  • #33 Vascular Rings Workup: Chest Radiography, Barium Esophagography, Echocardiography and Color-Flow Doppler
    https://emedicine.medscape.com/article/426233-workup
    At present, it is generally agreed that in the vast majority of cases, this study adds very little to the information obtained from barium esophagography. […] Cardiac catheterization is useful in cases where associated cardiac abnormalities are known or suspected. […] Bronchoscopy has been used in the evaluation of children with symptoms of airway obstruction or compression.
  • #34 Vascular ring | Iowa Head and Neck Protocols
    https://medicine.uiowa.edu/iowaprotocols/vascular-ring
    Conventional angiography Pro: High resolution. Complex relationships. Real time information. […] Computed tomography (CT) scan Pro: Complete assessment of tracheal and esophageal compression simultaneously. Can be performed fast without anesthesia of breath holding. […] Magnetic Resonance Angiography (MRA) Pro: No radiation exposure. Provides detailed relationships. […] Endoscopy Not universally performed, no clear sensitivity or specificity. Look for arterial pulsations, erythema and edema of the tracheal wall.
  • #35 Vascular rings
    https://www.mymlc.com/health-information/diseases-and-conditions/v/vascular-rings/?section=Diagnosis
    Barium swallow. In this test, your child swallows a substance called barium. X-rays are used to see how the barium moves inside of the esophagus. The barium allows the doctor to see whether or not there’s an indentation that may be caused by vascular rings. […] Upper endoscopy. A long, flexible tube with a camera allows your child’s doctor to examine the esophagus. The endoscope is inserted through the mouth into the throat as a tiny camera at the tip of the tube sends images to a video monitor. […] Bronchoscopy. A bronchoscopy may be done to evaluate the cause of symptoms and to determine the location and severity of compression against the trachea. In this test, a doctor inserts a small, flexible tube through the mouth or nose into the lungs. A light and a small camera attached to the bronchoscope allow the doctor to look inside the trachea and the lungs’ airways.
  • #36 Vascular rings – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube
    https://www.augustahealth.com/disease/vascular-rings/
    Upper endoscopy. A long, flexible tube with a camera allows your child’s doctor to examine the esophagus. The endoscope is inserted through the mouth into the throat as a tiny camera at the tip of the tube sends images to a video monitor. […] Bronchoscopy. A bronchoscopy may be done to evaluate the cause of symptoms and to determine the location and severity of compression against the trachea. In this test, a doctor inserts a small, flexible tube through the mouth or nose into the lungs. A light and a small camera attached to the bronchoscope allow the doctor to look inside the trachea and the lungs’ airways.
  • #37 Vascular Rings Workup: Chest Radiography, Barium Esophagography, Echocardiography and Color-Flow Doppler
    https://emedicine.medscape.com/article/426233-workup
    At present, it is generally agreed that in the vast majority of cases, this study adds very little to the information obtained from barium esophagography. […] Cardiac catheterization is useful in cases where associated cardiac abnormalities are known or suspected. […] Bronchoscopy has been used in the evaluation of children with symptoms of airway obstruction or compression.
  • #38 Vascular Rings Workup: Chest Radiography, Barium Esophagography, Echocardiography and Color-Flow Doppler
    https://emedicine.medscape.com/article/426233-workup
    Because children usually present with symptoms of respiratory difficulty, chest radiography is always the first and most commonly performed test. […] The identification of a right aortic arch on chest radiograph in a child with airway difficulties, respiratory distress or dysphagia should alert the clinician to the likelihood of a vascular ring. […] In general, chest radiography is not very sensitive in the diagnosis of vascular rings. […] Most authorities consider barium esophagography to be the most important study in patients with a suspected vascular ring, and it is diagnostic in the vast majority of cases. […] Echocardiographic studies have been increasingly used for the diagnosis of a vascular ring. […] This study has some diagnostic limitations. […] Computed tomography (CT), magnetic resonance imaging (MRI), and digital subtraction angiography (DSA) can be useful diagnostic tools because they reveal the positions of vascular, tracheobronchial, and esophageal structures and their relations to one another.
  • #39 Vascular ring | Iowa Head and Neck Protocols
    https://medicine.uiowa.edu/iowaprotocols/vascular-ring
    All congenital vascular anomalies that cause compression of the trachea and/or esophagus. Complete vascular ring: Completely encircles trachea/esophagus. Incomplete ring: everything else. […] No standardized diagnostic work-up. Referral and imaging based on symptom pattern. Otolaryngology often consulted secondary to airway concerns and dysphagia. […] CXR 95% have abnormalities (air trapping, right sided aortic arch). Often read as normal. […] Barium Swallow Pro: Historical test of choice in combination with angiography. Lateral view will show indentations on the posterior wall of the esophagus. May rule out vascular ring. […] Echocardiogram Pro: Highly sensitive. No radiation may work well as initial imaging test. […] Prenatal echocardiogram (Evans 2016) Increasing importance in diagnosis.
  • #40 Vascular ring | Iowa Head and Neck Protocols
    https://gme.medicine.uiowa.edu/iowaprotocols/vascular-ring
    All congenital vascular anomalies that cause compression of the trachea and/or esophagus. Complete vascular ring: Completely encircles trachea/esophagus. Incomplete ring: everything else. […] Severity of symptoms and time of presentation are directly proportional to the tightness of the ring. […] 95% have abnormalities (air trapping, right sided aortic arch). […] No standardized diagnostic work-up. Referral and imaging based on symptom pattern. Otolaryngology often consulted secondary to airway concerns and dysphagia. […] Historical test of choice in combination with angiography. […] Highly sensitive. No radiation may work well as initial imaging test. […] The decision for surgery is a joint decision between multiple actors. […] Surgical outcomes are generally very good (70-90% resolution). Dysphagia almost always resolves and persistent problems after initial repair are often related to tracheal anomalies.
  • #41 Vascular rings and slings | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/vascular-rings-and-slings?lang=us
    Vascular rings and slings refer to the congenital vascular encirclement of the esophagus and/or trachea by anomalous/aberrant vessels. […] Many vascular rings and slings are asymptomatic although they can present in pediatric patients with wheezing/stridor, recurrent respiratory infections, and/or dysphagia lusoria. […] Vascular rings can be classified as complete (mostly symptomatic) or incomplete (mostly asymptomatic). […] Radiographic features will differ depending on the underlying anomaly. […] On contrast esophagogram (and air tracheogram), the pattern of indentations on the frontal and lateral views helps to narrow the differential. […] CT/MRI allow precise demonstration of the anomaly. […] Possible considerations include congenital tracheomalacia.
  • #42 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology
    https://emedicine.medscape.com/article/426233-overview
    A vascular ring is an unusual congenital condition in which the anomalous configuration of the arch, associated vessels, or both surrounds the trachea and esophagus, forming a complete or incomplete ring around them. […] Prompt diagnosis and treatment of these congenital abnormalities can be lifesaving. […] For healthcare providers who look after pediatric patients, a high degree of suspicion, coupled with the appropriate imaging study, is required to make the diagnosis. A multimodality imaging approach (eg, with magnetic resonance imaging [MRI], computed tomography [CT], and esophagography) is often required for correct diagnosis (see Workup). […] Surgical division of a vascular ring is indicated in all symptomatic patients. To avoid serious complications, surgery should not be delayed, especially in patients with symptoms of airway compression. Individuals who have no symptoms from a vascular ring may not require surgical intervention. Surgical repairs in most cases can be performed successfully and without long-term morbidity (see Treatment).
  • #43 Get Vascular Rings Treatment | Cleveland Clinic Children’s
    https://my.clevelandclinic.org/pediatrics/services/vascular-rings-treatment
    If vascular rings press on your childs esophagus or trachea, theyll likely need surgery. Depending on how bad the symptoms are (and if they have a double aortic arch), our surgeons may operate right away. If they dont have symptoms, we may take a watchful waiting approach and keep a close eye on things as your child grows. […] Surgery for vascular rings […] Our surgeons do a thoracotomy to repair vascular rings. Theyll divide vascular rings to relieve pressure on your childs esophagus or trachea. They may also need to take out the abnormal arteries and then put them back in, or remove irregular parts in the the aorta. […] Lifelong care […] Your child may have symptoms for a few months after surgery, but these usually clear up within a year. Theyll need to have follow-up care and testing well into adulthood. So as they grow into adolescence and young adulthood, well help them transition comfortably to our adult providers.
  • #44 Vascular Rings: Causes and Symptoms
    https://my.clevelandclinic.org/health/diseases/23947-vascular-rings
    A vascular ring is a congenital birth defect involving the aorta. The condition can cause breathing or eating problems. A vascular ring is a congenital heart defect that can cause problems with breathing or eating. A vascular ring is a congenital defect, meaning it’s present at birth. The condition can be life-threatening, but surgery is usually successful. A vascular ring can also cause feeding/digestive issues. A healthcare provider may order some of the following tests to diagnose a vascular ring: Chest X-ray, CT scan, Echocardiogram. The healthcare provider may order other tests to evaluate your (or your baby’s) trachea or esophagus, such as: Barium swallow (esophagogram), Bronchoscopy, Upper endoscopy. Vascular rings that are pressing on your trachea or esophagus require surgery. Surgery to correct a vascular ring is quite safe and often successful. A vascular ring is an abnormal formation of the aorta that presses on the trachea, esophagus or both. The congenital defect can cause problems with breathing or eating. If you or your child has a vascular ring, surgery can correct the condition and eliminate symptoms.
  • #45 Get Vascular Rings Treatment | Cleveland Clinic Children’s
    https://my.clevelandclinic.org/pediatrics/services/vascular-rings-treatment
    If vascular rings press on your childs esophagus or trachea, theyll likely need surgery. Depending on how bad the symptoms are (and if they have a double aortic arch), our surgeons may operate right away. If they dont have symptoms, we may take a watchful waiting approach and keep a close eye on things as your child grows. […] Surgery for vascular rings […] Our surgeons do a thoracotomy to repair vascular rings. Theyll divide vascular rings to relieve pressure on your childs esophagus or trachea. They may also need to take out the abnormal arteries and then put them back in, or remove irregular parts in the the aorta. […] Lifelong care […] Your child may have symptoms for a few months after surgery, but these usually clear up within a year. Theyll need to have follow-up care and testing well into adulthood. So as they grow into adolescence and young adulthood, well help them transition comfortably to our adult providers.
  • #46 Vascular ring | Iowa Head and Neck Protocols
    https://gme.medicine.uiowa.edu/iowaprotocols/vascular-ring
    All congenital vascular anomalies that cause compression of the trachea and/or esophagus. Complete vascular ring: Completely encircles trachea/esophagus. Incomplete ring: everything else. […] Severity of symptoms and time of presentation are directly proportional to the tightness of the ring. […] 95% have abnormalities (air trapping, right sided aortic arch). […] No standardized diagnostic work-up. Referral and imaging based on symptom pattern. Otolaryngology often consulted secondary to airway concerns and dysphagia. […] Historical test of choice in combination with angiography. […] Highly sensitive. No radiation may work well as initial imaging test. […] The decision for surgery is a joint decision between multiple actors. […] Surgical outcomes are generally very good (70-90% resolution). Dysphagia almost always resolves and persistent problems after initial repair are often related to tracheal anomalies.
  • #47 Get Vascular Rings Treatment | Cleveland Clinic Children’s
    https://my.clevelandclinic.org/pediatrics/services/vascular-rings-treatment
    If vascular rings press on your childs esophagus or trachea, theyll likely need surgery. Depending on how bad the symptoms are (and if they have a double aortic arch), our surgeons may operate right away. If they dont have symptoms, we may take a watchful waiting approach and keep a close eye on things as your child grows. […] Surgery for vascular rings […] Our surgeons do a thoracotomy to repair vascular rings. Theyll divide vascular rings to relieve pressure on your childs esophagus or trachea. They may also need to take out the abnormal arteries and then put them back in, or remove irregular parts in the the aorta. […] Lifelong care […] Your child may have symptoms for a few months after surgery, but these usually clear up within a year. Theyll need to have follow-up care and testing well into adulthood. So as they grow into adolescence and young adulthood, well help them transition comfortably to our adult providers.
  • #48 Vascular Ring Surgery & Repair: Expert Care at Rady Children’s | Rady Children’s Hospital
    https://www.rchsd.org/programs-services/aerodigestive-vascular-ring-program/
    Vascular ring is a congenital aortic arch anomaly where the aorta and surrounding blood vessels develop abnormally, forming a ring-like structure that encircles the trachea and esophagus. A vascular ring can lead to breathing and swallowing difficulties as well as heart problems. […] At Rady Childrens, our specialists are here to help identify and treat vascular ringswhether its a complete vascular ring or an incomplete one. […] Vascular rings can be diagnosed based on symptoms or findings on a chest X-ray or echocardiogram. […] If a vascular ring is suspected in utero, you may meet with our Fetal Cardiology team to discuss possible impacts on pregnancy and delivery. Your provider may also recommend a CT scan or MRI of the chest and/or heart to examine the abnormal blood vessels, as well as the airway and esophagus. […] Evaluation and Diagnosis: Detailed assessments, including imaging (CT, MRI, or endoscopy), confirm the vascular anomaly. This helps identify any tracheoesophageal compression or additional heart defects.
  • #49 Vascular rings – what has changed, and what do I need to know as a practitioner? | Cardiology in the Young | Cambridge Core
    https://www.cambridge.org/core/journals/cardiology-in-the-young/article/vascular-rings-what-has-changed-and-what-do-i-need-to-know-as-a-practitioner/101C8D89EFAD9C234DD6B16851D497C1
    This has led to a younger age at diagnosis and repair. […] Conversely, knowledge of abnormal anatomy may result in stress to the family or even result in an operation in an otherwise normally developing child due to hypervigilance. […] To this point, one study noted an increased incidence of right aortic arch with aberrant left subclavian compared to other variants, concluding that this subset may be more common than previously identified, and thus, more likely to be asymptomatic and not require repair. […] Decision making is further complicated by the fact that symptoms do not necessarily correlate with the apparent severity of compression on diagnostic studies. […] In the incidentally discovered right-sided aortic arch, the patient and family should be questioned regarding symptoms.
  • #50 Vascular ring: prenatal diagnosis and prognostic management based on sequential cross-sectional scanning by ultrasound | BMC Pregnancy and Childbirth | Full Text
    https://bmcpregnancychildbirth.biomedcentral.com/articles/10.1186/s12884-023-05637-y
    SCS can accurately diagnose vascular rings before delivery, evaluate the shape and size of the rings to conduct prenatal monitoring of children until birth, which plays a guiding role in airway compression after birth. […] The purpose of this study was to investigate the value of prenatal ultrasound in the integrated management of vascular ring. […] The earlier the diagnosis of vascular ring disease and the timely surgical treatment of patients with possible compression, the better the prognosis. […] This study presents a method of scanning the fetal heart with continuous cross-sectional sequence scanning. The operator simply moves from a standard section of the abdominal circumference along the long axis of the body to the cephalic side. Following the cross-section of the great vessels of the heart until the mediastinum disappears, the method does not include the long axis section of the heart, which is less affected, convenient and easy to master, and easy to understand by tracing the origin of abnormal blood vessels through a continuous cross Sect.
  • #51 Vascular ring: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/007318.htm
    Vascular ring is an abnormal formation of the aorta, the large artery that carries blood from the heart to the rest of the body. It is a congenital problem, which means it is present at birth. […] The following tests can help diagnose vascular ring: Chest x-ray, Computed tomography (CT) scan of the heart and major blood vessels, Camera down the throat to examine the airways (bronchoscopy), Magnetic resonance imaging (MRI) of the heart and major blood vessels, Ultrasound examination (echocardiogram) of heart, X-ray of blood vessels (angiography), X-ray of the esophagus using a special dye to better highlight the area (esophagram or barium swallow). […] Contact your provider if your baby has symptoms of vascular ring. Getting diagnosed and treated quickly can prevent serious complications.
  • #52 Pediatric vascular ring outcomes for surgically repaired vs. unoperated children: a single-center experience – Ajdaa – Journal of Thoracic Disease
    https://jtd.amegroups.org/article/view/86251/html
    Overall, 79 patients (96%) underwent surgical repair. […] There was no in-hospital mortality. […] At a median follow-up of 54 (IQR, 23118) months, 20 patients (24%) still experienced some form of symptoms. […] Most patients with anatomic variants of vascular ring were symptomatic at the time of diagnosis, suffering predominantly from respiratory symptoms. […] Subtypes of vascular rings such as pulmonary slings or innominate artery compression syndromes require detailed preoperative evaluation and prompt surgical management. […] While 24% of patients remained symptomatic at long-term follow-up, half of those were asthma-related and medically controlled, resulting in nearly 90% of patients symptoms-free at mid-term follow-up. […] Finally, surgery in asymptomatic patients resulted in minimal hospital morbidity, supporting the current recommendation of early surgical repair after meticulous preoperative evaluation of associated trachea-bronchial anomalies.
  • #53 Editorial on “vascular ring diagnosis and management: notable trends over 25 years” – Savla – Translational Pediatrics
    https://tp.amegroups.org/article/view/14191/html
    Vascular rings occur due to some perturbation in the normal sequence of development and selective involution of paired aortic arches to form a definitive left or right aortic arch. The two most common types of vascular rings are double aortic arch (DAA) and right aortic arch with an aberrant left subclavian artery and a left patent ductus arteriosus or ligamentum arteriosum (RAA-ALS). The prevalence of vascular rings is unknown because many patients remain asymptomatic and are undiagnosed. […] A recently published study by Evans and colleagues reviewed the diagnosis and management of vascular rings before and after the introduction of prenatal diagnosis. […] As indicated in the study, after the three-vessel fetal echocardiography view was introduced in 2004, the rate of prenatal detection increased significantly from 0% before 2006, to 55% in 2010, and then up to 70% prenatal detection of diagnosed vascular rings since 2013.
  • #54 Vascular rings – what has changed, and what do I need to know as a practitioner? | Cardiology in the Young | Cambridge Core
    https://www.cambridge.org/core/journals/cardiology-in-the-young/article/vascular-rings-what-has-changed-and-what-do-i-need-to-know-as-a-practitioner/101C8D89EFAD9C234DD6B16851D497C1
    Vascular rings represent a heterogeneous set of aberrant great vessel anatomic configurations which can cause respiratory symptoms or dysphagia due to tracheal or oesophageal compression. […] More recently, many have been identified in patients without symptoms, including fetal echocardiogram, resulting in a conundrum for practitioners when attempting to determine who will benefit from surgical correction. […] A recent increase in diagnosis in asymptomatic patients due to more readily available cross-sectional imaging and fetal echocardiograms has led to additional challenges regarding who may benefit from surgical correction. […] Recently, there has been a substantial increase in the frequency of diagnosis in asymptomatic patients and on fetal echocardiogram. […] This diagnosis can prime the parents and practitioners with a possible anatomic basis for any symptoms which may potentially develop.
  • #55 Vascular rings – what has changed, and what do I need to know as a practitioner? | Cardiology in the Young | Cambridge Core
    https://www.cambridge.org/core/journals/cardiology-in-the-young/article/vascular-rings-what-has-changed-and-what-do-i-need-to-know-as-a-practitioner/101C8D89EFAD9C234DD6B16851D497C1
    In addition to surgical repair being less challenging in younger patients due to their size and more pliable vasculature, early evidence seems to suggest that it may result in more effective symptom resolution, thus the push for earlier repair. […] Finally, it is important to recognise that repair in adults is more complex and a higher-risk procedure.
  • #56 Vascular rings – what has changed, and what do I need to know as a practitioner? | Cardiology in the Young | Cambridge Core
    https://www.cambridge.org/core/journals/cardiology-in-the-young/article/vascular-rings-what-has-changed-and-what-do-i-need-to-know-as-a-practitioner/101C8D89EFAD9C234DD6B16851D497C1
    This has led to a younger age at diagnosis and repair. […] Conversely, knowledge of abnormal anatomy may result in stress to the family or even result in an operation in an otherwise normally developing child due to hypervigilance. […] To this point, one study noted an increased incidence of right aortic arch with aberrant left subclavian compared to other variants, concluding that this subset may be more common than previously identified, and thus, more likely to be asymptomatic and not require repair. […] Decision making is further complicated by the fact that symptoms do not necessarily correlate with the apparent severity of compression on diagnostic studies. […] In the incidentally discovered right-sided aortic arch, the patient and family should be questioned regarding symptoms.
  • #57
    https://link.springer.com/article/10.1007/s00247-005-1529-0
    Vascular ring is a term given to a combination of vascular and often ligamentous structures that encircle the trachea and esophagus. The diagnosis can be difficult because clinical symptoms can be variable and nonspecific, and because vascular arrangements that result in vascular rings in some patients do not form vascular rings in others. […] The diagnosis of vascular ring can be made by various imaging modalities. Therefore it is imperative that pediatric radiologists be familiar with the anatomic variants that can result in a symptomatic ring needing surgical repair, their imaging appearance and the appropriate imaging algorithm. […] The goals of this manuscript are to describe common and uncommon types of vascular rings, to simplify the differential diagnosis, and to outline the imaging options for accurate diagnosis.
  • #58 Vascular Ring; Spectrum of Pathologies and Scenarios for Diagnosis and Management, International Journal of Cardiovascular and Thoracic Surgery, Science Publishing Group
    https://www.sciencepublishinggroup.com/article/10.11648/j.ijcts.20200606.12
    Vascular rings represent approximately 1% of all congenital cardiac anomalies, with Edwards classification being the first to outline them into a complete or partial vascular ring. A Complete ring is a combination of patent vessels, atretic vascular segments or ligamentous structures. […] We reviewed patients` records from our Pediatric Cardiac Surgery Unit, over 10 years. We encountered 63 patients from January 2009 to January 2019. Our patients were 57 (90.5%) complete vascular rings, 5 (7.9%) pulmonary artery slings, and 1 (1.6%) patient with innominate artery compression. […] Vascular ring is a rare anomaly that needs suspicion for diagnosis. The operative strategy relies on extensive dissection and division of the non-dominant arch and division of the ligamentum, freeing all fibrous bands between the arch and esophagus and trachea. For augmented results; we need collaboration from pediatricians and pulmonologists who must be aware of such anomalies.
  • #59 Get Vascular Rings Treatment | Cleveland Clinic Children’s
    https://my.clevelandclinic.org/pediatrics/services/vascular-rings-treatment
    During this visit, your childs provider will do a physical exam and order more tests to find out whats going on with their trachea, esophagus, chest, abdomen (belly) and heart. […] Testing for vascular rings […] Because vascular rings can cause many different symptoms, your child may have several tests to help their providers pinpoint a diagnosis. These may include: […] Meet our vascular rings team […] Cleveland Clinic Childrens team-based approach to care means your child will get a care team of different providers from different specialties. These experts are handpicked to meet your childs unique needs and diagnosis. […] Treating Vascular Rings at Cleveland Clinic Childrens […] We cant prevent vascular rings from developing. But we can treat them. Your childs treatment plan will depend on how the rings are affecting things like swallowing, breathing and your childs overall health.
  • #60 Vascular ring – Wikipedia
    https://en.wikipedia.org/wiki/Vascular_ring
    A vascular ring is a congenital defect in which there is an abnormal formation of the aorta and/or its surrounding blood vessels. The trachea and esophagus are completely encircled and sometimes compressed by a „ring” formed by these vessels, which can lead to breathing and digestive difficulties. […] Diagnosis requires a high degree of clinical suspicion and can be confirmed with barium contrast esophagogram for those with esophageal symptoms, bronchoscopy, or CT or MRI.
  • #61 Assessment of Surgical Correction at Vascular Ring Anomalies – Journal of Updates in Cardiovascular Medicine
    https://www.jucvm.com/articles/assessment-of-surgical-correction-at-vascular-ring-anomalies/doi/ejcm.18.00389
    Aim: In this retrospective study, we present our fifteen years of experience in surgical treatment in vascular rings. Thus, we aimed to determine the factors that causing improved both early and late term complications. […] The diagnosis can be confirmed using various imaging and endoscopic methods such as computer assisted tomography (CT), magnetic resonance imaging (MRI), bronchoscopy, gastroscopy, echocardiography, cardiac catheterization, and upper GI series. […] Once the patient was diagnosed, surgical treatment is required for decompression as a standard therapy. […] Vascular rings may manifest in various symptoms and different ages, thus requires awareness for diagnosis. Once the patient was diagnosed surgical treatment is essential to prevent irreversible harmful effects. A profound examination and assessment of the anatomy are beneficial regarding the decision of how the surgery should be performed. The goal of the surgery is to relieve the compression over tracheal and/or esophagus by dividing the dislocated vessel while providing sufficient blood flow towards the head. The outcomes after the surgery are satisfactory and lead to uneventful long-term survival.
  • #62 Assessment of Surgical Correction at Vascular Ring Anomalies – Journal of Updates in Cardiovascular Medicine
    https://www.jucvm.com/articles/assessment-of-surgical-correction-at-vascular-ring-anomalies/doi/ejcm.18.00389
    Aim: In this retrospective study, we present our fifteen years of experience in surgical treatment in vascular rings. Thus, we aimed to determine the factors that causing improved both early and late term complications. […] The diagnosis can be confirmed using various imaging and endoscopic methods such as computer assisted tomography (CT), magnetic resonance imaging (MRI), bronchoscopy, gastroscopy, echocardiography, cardiac catheterization, and upper GI series. […] Once the patient was diagnosed, surgical treatment is required for decompression as a standard therapy. […] Vascular rings may manifest in various symptoms and different ages, thus requires awareness for diagnosis. Once the patient was diagnosed surgical treatment is essential to prevent irreversible harmful effects. A profound examination and assessment of the anatomy are beneficial regarding the decision of how the surgery should be performed. The goal of the surgery is to relieve the compression over tracheal and/or esophagus by dividing the dislocated vessel while providing sufficient blood flow towards the head. The outcomes after the surgery are satisfactory and lead to uneventful long-term survival.
  • #63 Assessment of Surgical Correction at Vascular Ring Anomalies – Journal of Updates in Cardiovascular Medicine
    https://www.jucvm.com/articles/assessment-of-surgical-correction-at-vascular-ring-anomalies/doi/ejcm.18.00389
    Aim: In this retrospective study, we present our fifteen years of experience in surgical treatment in vascular rings. Thus, we aimed to determine the factors that causing improved both early and late term complications. […] The diagnosis can be confirmed using various imaging and endoscopic methods such as computer assisted tomography (CT), magnetic resonance imaging (MRI), bronchoscopy, gastroscopy, echocardiography, cardiac catheterization, and upper GI series. […] Once the patient was diagnosed, surgical treatment is required for decompression as a standard therapy. […] Vascular rings may manifest in various symptoms and different ages, thus requires awareness for diagnosis. Once the patient was diagnosed surgical treatment is essential to prevent irreversible harmful effects. A profound examination and assessment of the anatomy are beneficial regarding the decision of how the surgery should be performed. The goal of the surgery is to relieve the compression over tracheal and/or esophagus by dividing the dislocated vessel while providing sufficient blood flow towards the head. The outcomes after the surgery are satisfactory and lead to uneventful long-term survival.