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Congenital mitral stenosis is a rare anomaly. We report successful surgical management of congenital mitral stenosis in a patient with Kabuki syndrome. A 15-month-old male with Kabuki syndrome was referred and echocardiography showed isolated congenital mitral stenosis. The valve was not repairable as it was showing severe stenotic features in multilevel fashion. Valve was replaced with mechanical valve. Postoperative course was uneventful without any rhythm problem and he has been doing well clinically during the 25 months follow-up with regular international normalized ratio checks.
Valvular diseases are classified either as obstructing outflow (i.e., stenosis) or inadequately closing/coapting (i.e., regurgitation). Many mild–moderate valve lesions exist in a compensated or well-tolerated state; the tenets of hemodynamic management become particularly important in the severe or acute setting.
Frontal sinus stents have been introduced to reduce frontal sinus re-stenosis after surgery and to improve outcomes.
Method
This study was a retrospective analysis of 19 patients who had endoscopic sinus surgery with approach to the frontal sinus and insertion of a soft sinus stent.
Results
The frontal recess was patent in 78.9 per cent and stenosed in 21.1 per cent of patients; no completely closed recesses were observed. Mean follow up was 20.7 months, and time period of stenting was 9.8 months on average; complications were observed in 47.4 per cent of the patients, with post-operative sinonasal infection being the most common.
Conclusion
In the authors’ experience, indications for frontal sinus stenting include recalcitrant chronic rhinosinusitis after multiple functional endoscopic sinus surgeries (especially in chronic rhinosinusitis with nasal polyps), patients with history of important craniofacial surgery or trauma, and recurrent mucoceles. The stent was overall well tolerated as only minor complications were observed. Close clinical follow up is mandatory.
Frontal sinus surgery is challenging as the frontal recess is prone to re-stenosis and there is subsequent occlusion of the frontal sinus outflow tract. In an attempt to maintain the frontal recess calibre and reduce frontal sinus re-stenosis, frontal sinus stents have been used with different materials and varying results.
Objective
This paper presents the technique of using a modified Montgomery T-tube as a frontal sinus stent.
Results and conclusion
The use of a soft, self-retaining and non-absorbable stent that can be used for stenting of the frontal sinus is described. Our technique is safe, effective, inexpensive and well tolerated.
Laryngotracheal stenosis management remains largely discretionary in surgical practice. Duration of stenting remains variable following open reconstruction procedures in absence of clearly established differences. The current study evaluates successful decannulation after short-term periods compared with longer periods.
Method
A comparative study over 18 months evaluated differences in successful decannulation between short- and long-term stent groups. Patients with grade II, III or IV laryngotracheal stenosis were placed on Montgomery T-tube for a short-term period (n = 15), and decannulation rates were compared with age-, sex- and diagnosis-matched patients (n = 15) from historical cases with long-term stent placement.
Results
Thirty patients were included. There was no difference between the two groups at baseline. Nine patients (30 per cent) were successfully decannulated, and there was no difference in rates of decannulation between the two groups (p = 0.8). Granulations at the proximal end of tube (38.7 per cent), superior migration of tube (16.1 per cent) and dysphonia (12.9 per cent) were common complications.
Conclusion
Decannulation was not more successful after placing Montgomery T-tubes for longer periods of time when compared with removal within the first three months. Early removal after proper case selection and planning may be considered for a successful outcome in laryngotracheal stenosis.
Conduit stenosis is a major, albeit rare, complication following the Fontan palliation. A single-baffle conduit with polytetrafluoroethylene is widely used for an extracardiac type Fontan palliation. A polyethylene terephthalate conduit (Dacron) is sometimes used for the conduit when more flexibility is required. A Y-shaped conduit is rarely used, but it may reduce the energy loss and achieve better hepatic flow distribution. Data on the long-term patency and complications when using a Y-shaped Dacron conduit is lacking. We report a case of a severely stenotic Y-shaped Dacron conduit in a patient who underwent extracardiac Fontan palliation.
The incidence of recurrent stenosis after cricotracheal resection is 3–9.5 per cent. Management of such patients is challenging. This study aimed to review our experience in revision cricotracheal resection.
Methods
The study was conducted in the Otorhinolaryngology Department, Mansoura University Hospitals, Egypt, on nine patients with recurrent stenosis following cricotracheal resection. Revision cricotracheal resection was performed in all patients. Surgiflo was applied on the site of anastomosis to enhance healing.
Results
No intra-operative complications were recorded. Minor post-operative complications occurred in two patients (surgical emphysema and temporary choking); no major complications were reported. Re-stenosis occurred in one patient. Successful decannulation was achieved in eight of the nine patients.
Conclusion
Revision cricotracheal resection is the definitive curative treatment for recurrent stenosis after previous unsuccessful resection. It has high success rates, provided that careful pre-operative assessment and meticulous operative technique are performed.
We aim to assess the safety and efficacy of the transcatheter balloon dilation of superior cavopulmonary anastomosis (SCPA).
Background:
SCPA stenosis can lead to impaired pulmonary blood flow, hypoxemia and development of veno-venous collaterals with right-to-left shunt. Balloon dilation of SCPA has been rarely reported and follow-up information is lacking.
Methods:
We performed a retrospective review of patients who underwent cardiac catheterisation and angioplasty of SCPA and reviewed patient’s demographics, diagnosis, SCPA surgery and post-operative course, catheterisation haemodynamics, procedural technique, angiography, and the findings of follow-up catheterisation.
Results:
Between 2008 and 2017, seven patients showed significant narrowing of SCPA and underwent balloon angioplasty, all of whom had undergone bidirectional Glenn (BDG). Indications for cardiac catheterisation included persistent pleural effusion, hypoxemia, and echocardiographic evidence of BDG stenosis or routine pre-Fontan assessment. Five patients had bilateral SCPA. The procedure was successful in all cases with increase in the stenosis diameter from a median of 3.3 mm (range 1.2–4.7 mm) to a median of 4.7 mm (range 2.6–7.8 mm). All patients had at least one follow-up cardiac catheterisation. Only one patient required repeat angioplasty at the 2.3-month follow-up with no further recurrence. Sustained results and interval growth were noted in all other cases during up to 29 months of follow-up. No adverse events were encountered.
Conclusion:
Based on our small series, balloon angioplasty of BDG stenosis is feasible and safe and appears to provide sustained improvement with interval growth and only the rare recurrence of stenosis.
This study aimed to evaluate the association between cochlear nerve canal dimensions and semicircular canal abnormalities and to determine the distribution of bony labyrinth anomalies in patients with cochlear nerve canal stenosis.
Method
This was a retrospective study in which high-resolution computed tomography images of paediatric patients with severe-to-profound sensorineural hearing loss were reviewed. A cochlear nerve canal diameter of 1.5 mm or less in the axial plane was classified as stenotic. Semicircular canals and other bony labyrinth morphology and abnormality were evaluated.
Results
Cochlear nerve canal stenosis was detected in 65 out of 265 ears (24 per cent). Of the 65 ears, 17 ears had abnormal semicircular canals (26 per cent). Significant correlation was demonstrated between cochlear nerve canal stenosis and semicircular canal abnormalities (p < 0.01). Incomplete partition type II was the most common accompanying abnormality of cochlear nerve canal stenosis (15 out of 65, 23 per cent).
Conclusion
Cochlear nerve canal stenosis is statistically associated with semicircular canal abnormalities. Whenever a cochlear nerve canal stenosis is present in a patient with sensorineural hearing loss, the semicircular canal should be scrutinised for presence of abnormalities.
Kawasaki disease is an acute febrile medium-vessel vasculitis affecting children. Though coronary artery aneurysm is a common complication, stenosis and occlusion are rare. Here, we report a case of Kawasaki disease with concomitant aneurysm and complete occlusion of different coronary arteries in the same patient.
Canalplasty for auditory exostoses is reserved for symptomatic patients. This study reviewed the outcomes of our technique regarding cicatricial stenosis.
Method
A chart review was conducted on patients undergoing canalplasty for auditory exostoses between 2002 and 2017. The surgical technique is described.
Results
The study comprised 43 adults (50 operated ears). Exostoses were bilateral in 40 cases (94 per cent) and occlusive in 33 (66 per cent). After drilling, the external auditory meatus was covered with a graft in 34 cases (68 per cent) and a silicone sheet was used in 32 (64 per cent). Cicatricial stenosis appeared in eight cases (16 per cent). Skin grafts were not used in six of these eight cases (p < 0.04), and silicone sheets were used only in one of these eight (p < 0.01).
Conclusion
Canalplasty is challenging because of its potential complications. Our data showed that the use of skin grafts and silicone sheets to cover the bared external auditory meatus was associated with a lower rate of cicatricial stenosis.
An ancient yoga technique called sutra neti, which is extensively used in India to keep the sinuses healthy, led to complete velopharyngeal stenosis in a 67-year-old male patient who presented with bilateral nasal obstruction, mouth breathing, anosmia and a change in voice.
Method
The patient was diagnosed by nasal endoscopy using a zero-degree Hopkins rod endoscope, and adhesions were released using coblation.
Results
The patient had post-operative alleviation of symptoms and a patent velopharyngeal inlet on examination.
Conclusion
Vigorous sutra neti can lead to velopharyngeal stenosis. Release of the stenosis is then required to cure the nasal blockade.
The use of three-dimensional printing has been rapidly expanding over the last several decades. Virtual surgical three-dimensional simulation and planning has been shown to increase efficiency and accuracy in various clinical scenarios.
Objectives
To report the feasibility of three-dimensional printing in paediatric laryngotracheal stenosis and discuss potential applications of three-dimensional printed models in airway surgery.
Method
Retrospective case series in a tertiary care aerodigestive centre.
Results
Three-dimensional printing was undertaken in two cases of paediatric laryngotracheal stenosis. One patient with grade 4 subglottic stenosis with posterior glottic involvement underwent an extended partial cricotracheal reconstruction. Another patient with grade 4 tracheal stenosis underwent tracheal resection and end-to-end anastomosis. Models of both tracheas were printed using PolyJet technology from a Stratasys Connex2 printer.
Conclusion
It is feasible to demonstrate stenosis in three-dimensional printed models, allowing for patient-specific pre-operative surgical simulation. The models serve as an educational tool for patients’ understanding of the surgery, and for teaching residents and fellows.
Takayasu’s arteritis is a rare idiopathic arteritis causing stenosis or aneurysms of the aorta, pulmonary arteries, and their branches. It usually occurs in women, but has been described in children.
Objective
The objective of this study was to determine the clinical presentation, demographic profile, vascular involvement, origins, management, and outcome of children diagnosed with Takayasu’s arteritis at a Southern African tertiary care centre between 1993 and 2015.
Methods
This is a retrospective analysis of all children with Takayasu’s arteritis captured on a computerised electronic database during the study period.
Results
A total of 55 children were identified. The female:male ratio was 3.2:1, and the mean age was 9.7±3.04 years. Most originated outside the provincial borders of the study centre. The majority presented with hypertension and heart failure. In all, 37 (67%) patients had a cardiomyopathy with a mean fractional shortening of 15±5%. A positive purified protein derivative test was documented in 73%. Abdominal aorta and renal artery stenosis were the predominant angiographic lesions. A total of 23 patients underwent 30 percutaneous interventions of the aorta, pulmonary, and renal arteries: eight stents, 22 balloon angioplasties, and seven had nephrectomies. All patients received empiric tuberculosis treatment, immunosuppressive therapy, and anti-hypertensive agents as required. Overall, there was a significant reduction in systolic blood pressure and improvement in fractional shortening (p<0.05) with all treatments.
Conclusion
Takayasu’s arteritis is more common in girls and frequently manifests with hypertension and heart failure. The abdominal aorta and renal arteries are mostly affected. Immunosuppressive, anti-hypertensive, and vascular intervention therapies improve blood pressure control and cardiac function.
The pyriform aperture comprises the central area of facial bone structure. It is formed by the free corners of the nasal bone and the frontal processes of the maxillae, which articulate with each other at the nasomaxillary suture lines. Congenital nasal pyriform aperture stenosis might be linked to various craniofacial problems. This review presents all aspects of pyriform aperture stenosis and enlargement.
Methods:
A literature search was conducted. Pyriform aperture definition, nasal development, congenital nasal pyriform aperture stenosis and pyriform aperture enlargement were reviewed.
Results:
One of the most common abnormalities is holoprosencephaly, which is a midline developmental deficiency that may also be present in combination with facial clefting. The aetiology of nasal pyriform aperture stenosis remains unclear. When diagnosed, the choice of treatment is between non-surgical and operative methods, depending on the seriousness of the problem. Provided the sufferer can maintain a secure air passage with the help of specialised medical procedures and respiratory tract adjuvants, operative therapy may be delayed.
Conclusion:
The operative outcomes are extremely good, and the prognosis relies mainly on coexisting neural and endocrine problems. This paper evaluates the nasal pyriform aperture in detail.
Nasal stenosis is a rare but significant complication of chemoradiation treatment for nasopharyngeal carcinoma. It can cause distressing obstructive symptoms for the patient and potentially interfere with monitoring for recurrence. Quality-of-life indicators are known to be very poor in this group of patients; however, there is very little evidence in the literature as to management of this complication.
Methods:
This paper presents an endoscopic day-case surgical procedure to address total posterior nasal stenosis, as conducted in three patients, which involves division of adhesions and removal of the posterior septum and posterior inferior turbinates, without the need for packing or stenting.
Results:
In this series, there was resolution of obstructive symptoms and no recurrence of stenosis during follow up (up to 20 months).
Conclusion:
This endoscopic procedure performed to manage total nasal stenosis differs from those previously described in the literature, as post-operative stenting or packing is not required, and removal of the posterior aspect of the septum is performed routinely.
A theoretical study of an unsteady two-layered blood flow through a stenosed artery is presented in this article. The geometry of a rigid stenosed artery is assumed to be $w$-shaped. The flow regime is assumed to be laminar, unsteady and uni-directional. The characteristics of blood are modelled by the generalized Oldroyd-B non-Newtonian fluid model in the core region and a Newtonian fluid model in the periphery region. The governing partial differential equations are derived for each region by using mass and momentum conservation equations. In order to facilitate numerical solutions, the derived differential equations are nondimensionalized. A well-tested explicit finite-difference method (FDM) which is forward in time and central in space is employed for the solution of a nonlinear initial boundary value problem corresponding to each region. Validation of the FDM computations is achieved with a variational finite element method algorithm. The influences of the emerging geometric and rheological parameters on axial velocity, resistance impedance and wall shear stress are displayed graphically. The instantaneous patterns of streamlines are also presented to illustrate the global behaviour of the blood flow. The simulations are relevant to haemodynamics of small blood vessels and capillary transport, wherein rheological effects are dominant.
Anomalous aortic origins of the coronary arteries comprise approximately one-third of all coronary artery anomalies and are characterised by coronary arteries with anomalies of aortic origin involving abnormal courses, stenoses, and compression that can lead to myocardial ischaemia and sudden death. Operative techniques to treat these anomalies have not been standardised yet. Moreover, the management of potential complications has not been addressed. Common and rare forms of anomalous aortic origins of the coronary arteries are reviewed and understood standard techniques for an uncomplicated unroofing procedure are illustrated. Also noted are techniques that can be applied to unexpected anatomical findings and unwanted complications that could prove to be life-threatening. Several technical recommendations are offered.