Vascular rings and slings: interesting vascular anomalies

2011 ◽  
Vol 125 (11) ◽  
pp. 1158-1163 ◽  
Author(s):  
E Phelan ◽  
S Ryan ◽  
H Rowley

AbstractIntroduction:A vascular ring refers to encirclement of the trachea and oesophagus by an abnormal combination of derivatives of the aortic arch system. These malformations can cause variable degrees of compression of the oesophagus, trachea or both. Symptoms can range from no effect to severe stridor, dyspnoea and/or dysphagia.Method and results:This study presents a case series of six patients treated over a six-year period (2003–2009), illustrating the features of four different types of vascular ring; these types are discussed in detail. The clinical presentation, radiology, and microlaryngoscopy and bronchoscopy findings are also discussed.Conclusion:The management of children with vascular rings requires a high index of clinical suspicion to ensure prompt diagnosis. As many of these children present with airway symptoms, the paediatric otolaryngologist plays a key role in identifying and assessing their anatomical anomalies.

2002 ◽  
Vol 12 (6) ◽  
pp. 1-7 ◽  
Author(s):  
Albert C. Cuetter ◽  
Russell J. Andrews

Object The prognosis for intraventricular neurocysticercosis (IVNCC) is poorer than that for parenchymal NCC, making prompt diagnosis and treatment especially important. Although active, viable intraventricular cysts produce no reaction in the host; they can cause noncommunicating hydrocephalus, the onset of which is frequently abrupt. With the death of the larva comes a local granulomatous ependymitis, generalized ventriculitis, and meningoencephalitis. The authors report on 18 patients with IVNCC (accrued from a larger case series of 62 patients with NCC treated over an 11-year period), detailing clinical presentation, neuroimaging findings, treatment, and outcome. Methods All patients presented with hydrocephalus and/or meningitis. The most valuable diagnostic tests were magnetic resonance imaging of the brain and EITB of serum and/or cerebrospinal fluid. Treatment included albenda-zole and steroid therapy in all cases, and when necessary in cases requiring urgent or emergency ventriculostomy and/or surgical removal of the obstructing cyst (followed by shunt placement if indicated). Conclusions An extensive review of the literature on IVNCC has been prepared, with the goal of providing the reader with the information necessary to diagnose and treat this complex and potentially fatal disease in a timely and effective manner.


Author(s):  
Alison U Kelly ◽  
Rajeev Srivastava ◽  
Ellie Dow ◽  
D Fraser Davidson

Neuroblastoma is the most common solid extracranial malignancy diagnosed in childhood. Clinical presentation is variable, and metastatic disease is common at diagnosis. Analyses of urinary catecholamines and their metabolites are commonly requested as a first-line investigation when clinical suspicion exists. Levodopa (L-Dopa) therapy is utilized as a treatment for a number of disorders in childhood, including Dopa-responsive dystonia. Neuroblastoma may mimic some of the clinical features of this disorder. L-Dopa can interfere with analysis of urinary catecholamines and their metabolites and complicate the interpretation of results. We present the cases of three children who were prescribed L-dopa at the time of analysis of urinary catecholamines and metabolites as a screen for neuroblastoma, but who did not have the disease. Comparison of their results with those from cases with true neuroblastoma reveal that it is impossible to reliably distinguish true neuroblastoma from L-Dopa therapy using these tests. We recommend that patients should be off L-dopa therapy, if possible when these tests are performed. These cases illustrate the importance of providing clinical details and drug history to the laboratory in order to avoid diagnostic confusion.


2018 ◽  
Vol 5 (6) ◽  
pp. 2341 ◽  
Author(s):  
Sneha Singh Dhruw ◽  
Abhishek Saini ◽  
Sourabh Singh

 Vascular rings are a group of anomalies caused by abnormal embryologic development of the branchial arch system. They can lead to variable degrees of respiratory problems or feeding difficulties by forming a complete or partial ring compressing the trachea, the bronchi, and the oesophagus. Vascular rings not being so common, a missed diagnosis is much more common. Hence a high degree of suspicion along with appropriate imaging study is required to make a diagnosis. This case report describes an uncommon acute presentation of a vascular ring in newborn as a brief resolved unexplained event (BRUE).


2002 ◽  
Vol 12 (2) ◽  
pp. 172-176 ◽  
Author(s):  
Annalisa Angelini ◽  
Konstantinos Dimopoulos ◽  
Carla Frescura ◽  
Piergiorgio Gamba ◽  
Roberto Lo Piccolo ◽  
...  

Vascular rings are rare vascular congenital anomalies causing oesophageal and tracheal compression. An aortoesophageal fistula is a devastating, in part iatrogenic, complication of vascular rings. It is seen with increasing frequency, and can be misleading, since differential diagnosis with other causes of haematemesis and melaena is often difficult, especially in infants. We report two infants with aortoesophageal fistulas secondary to double aortic arches forming a vascular ring. In both, the diagnosis was missed, and massive haemorrhage led to death. In both cases, the fissuration on the oesophageal and aortic sides of the fistula had sharp edges, highly suggestive of an iatrogenic laceration caused by manipulation of nasogastric tubes. The key for the diagnosis of vascular rings is, therefore, clinical suspicion and awareness of this condition. Prompt identification in infants with stridor, wheezing, or respiratory distress can prevent prolonged intubation, thus avoiding the formation of an aortoesophageal fistula and hopefully preventing a fatal outcome.


Author(s):  
Christopher Paul Bengel ◽  
Rifat Kacapor

Abstract Background Vaccination is the most important measure to control the coronavirus disease 2019 (COVID-19) pandemic. Myocarditis has been reported as a rare adverse reaction to COVID-19 vaccines. The clinical presentation of myocarditis in such cases can range from mild general symptoms to acute heart failure. Case summary We report the cases of two young men who presented with chest pain and dyspnoea following the administration of the mRNA COVID-19 vaccine. Cardiac investigations revealed findings typical of acute myocarditis. Discussion Myocarditis is a rare complication following mRNA COVID-19 vaccination. In this case series, the temporal proximity of the development of acute myocarditis and the administration of the mRNA COVID-19 vaccine was acknowledged. In the absence of other causative factors, myocarditis in these patients potentially occurred due to an adverse reaction to the mRNA COVID-19 vaccine. However, a causal relationship remains speculative. Clinical suspicion of myocarditis should be high if patients present with chest pain or dyspnoea after receiving COVID-19 vaccination.


2020 ◽  
Author(s):  
Julia Villalva ◽  
Belén Nieto-Ortega ◽  
Manuel Melle-Franco ◽  
Emilio Pérez

The motion of molecular fragments in close contact with atomically flat surfaces is still not fully understood. Does a more favourable interaction imply a larger barrier towards motion even if there are no obvious minima? Here, we use mechanically interlocked rotaxane-type derivatives of SWNTs (MINTs) featuring four different types of macrocycles with significantly different affinities for the SWNT thread as models to study this problem. Using molecular dynamics, we find that there is no direct correlation between the interaction energy of the macrocycle with the SWNT and its ability to move along or around it. Density functional tight-binding calculations reveal small (<2.5 Kcal·mol-1) activation barriers, the height of which correlates with the commensurability of the aromatic moieties in the macrocycle with the SWNT. Our results show that macrocycles in MINTs rotate and translate freely around and along SWNTs at room temperature, with an energetic cost lower than the rotation around the C−C bond in ethane.<br>


2019 ◽  
Vol 24 (5) ◽  
pp. 549-557
Author(s):  
Malia McAvoy ◽  
Heather J. McCrea ◽  
Vamsidhar Chavakula ◽  
Hoon Choi ◽  
Wenya Linda Bi ◽  
...  

OBJECTIVEFew studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.METHODSA retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017. The authors examined premorbid risk factors, clinical presentation, physical examination findings, type and duration of conservative management, indications for surgical intervention, complications, and postoperative outcomes.RESULTSOver the 19-year study period, 199 patients underwent LMD at the authors’ institution. The mean age at presentation was 16.0 years (range 12–18 years), and 55.8% were female. Of these patients, 70.9% participated in competitive sports, and among those who did not play sports, 65.0% had a body mass index greater than 25 kg/m2. Prior to surgery, conservative management had failed in 98.0% of the patients. Only 3 patients (1.5%) presented with cauda equina syndrome requiring emergent microdiscectomy. Complications included 4 cases of postoperative CSF leak (2.0%), 1 case of a noted intraoperative CSF leak, and 3 cases of wound infection (1.5%). At the first postoperative follow-up appointment, minimal or no pain was reported by 93.3% of patients. The mean time to return to sports was 9.8 weeks. During a mean follow-up duration of 8.2 years, 72.9% of patients did not present again after routine postoperative appointments. The total risk of reoperation was a rate of 7.5% (3.5% of patients underwent reoperation for the same level; 4.5% underwent adjacent-level decompression, and one patient [0.5%] ultimately underwent a fusion).CONCLUSIONSMicrodiscectomy is a safe and effective treatment for long-term relief of pain and return to daily activities among pediatric patients with symptomatic lumbar disc disease in whom conservative management has failed.


Medicina ◽  
2021 ◽  
Vol 57 (7) ◽  
pp. 660
Author(s):  
Csilla-Andrea Eötvös ◽  
Roxana-Daiana Lazar ◽  
Iulia-Georgiana Zehan ◽  
Erna-Brigitta Lévay-Hail ◽  
Giorgia Pastiu ◽  
...  

Among the different types, immunoglobulin light chain (AL) cardiac amyloidosis is associated with the highest morbidity and mortality. The outcome, however, is significantly better when an early diagnosis is made and treatment initiated promptly. We present a case of cardiac amyloidosis with left ventricular hypertrophy criteria on the electrocardiogram. After 9 months of follow-up, the patient developed low voltage in the limb leads, while still maintaining the Cornell criteria for left ventricular hypertrophy as well. The relative apical sparing by the disease process, as well as decreased cancellation of the opposing left ventricular walls could be responsible for this phenomenon. The discordance between the voltage in the frontal leads and precordial leads, when present in conjunction with other findings, may be helpful in raising the clinical suspicion of cardiac amyloidosis.


Author(s):  
Josee Paradis ◽  
Agnieszka Dzioba ◽  
Hamdy El-Hakim ◽  
Paul Hong ◽  
Frederick K. Kozak ◽  
...  

Abstract Background To evaluate the clinical presentation of choanal atresia (CA) in tertiary centers across Canada. Methods Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with CA at a participating center. Results The health charts of 215 patients (59.6% female) with CA were reviewed and included in this study. The mean age of patients at time of CA presentation was 0.4 months (range 0.1 to 7.2 months) for bilateral CA and 37.8 months (range 0.1 to 164.1 months) for unilateral cases. The most common presenting symptoms for bilateral CA in decreasing order were respiratory distress (96.4%), feeding difficulties (68.2%), and rhinorrhea (65.5%), and for unilateral cases in decreasing order were rhinorrhea (92.0%), feeding difficulties (24.7%), and respiratory distress (18.0%). For the majority of patients (73.2%), the obstruction comprised mixed bony and membranous tissue, with only 10.5% presenting with a purely membranous obstruction. Familial history of CA was confirmed in only 3.3% of cases. One half of patients with CA presented with one or more associated anomalies and 30.6% had a syndrome. Conclusions The present investigation is the first national multi-institutional study evaluating the clinical presentation of CA over three decades. The present cohort of CA patients presented with a breadth of co-morbidities with highly variable presentations, with bilateral cases being more severely affected than unilateral cases. Further investigation into hereditary linkages to CA development is warranted. Graphical abstract


Author(s):  
Elsayed Abdelkreem ◽  
Rofaida M. Magdy ◽  
Abdelrahim A. Sadek

AbstractObjectivesTo study the clinical and laboratory features, management, and outcome of pediatric non-diabetic ketoacidosis (NDKA).MethodsBetween May 2018 and April 2020, we prospectively collected children under 18 years who presented with ketoacidosis, defined as ketosis (urinary ketones ≥++ and/or serum β-hydroxybutyrate level ≥3 mmol/L) and metabolic acidosis (pH <7.3 and HCO3− <15 mmol/L). Children with HbA1c level ≥6.5% at initial presentation and those meeting the diagnostic criteria for DM during follow-up were excluded. Data were collected on demographics, clinical and laboratory features, management, and outcome.ResultsEleven children with 19 episodes of NDKA were identified. The median age was 12 months (range from 5 months to 5 years). They manifested dehydration and disturbed conscious level (all cases), convulsions (n=6), hypoglycemia (n=6), hyperglycemia (n=2) and significant hyperammonemia (n=4). Most cases required intensive care management. Death or neurodevelopmental impairment occurred in six cases. Seven cases had inborn errors of metabolism (IEMs). Other cases were attributed to starvation, sepsis, and salicylate intoxication.ConclusionsThis is the largest case series of pediatric NDKA. Ketoacidosis, even with hyperglycemia, is not always secondary to diabetes mellitus. IEMs may constitute a significant portion of pediatric NDKA. Increased awareness of this unfamiliar condition is important for prompt diagnosis, timely management, and better outcome.


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