Management of Late-Presenting Congenital Combined Heart Defect - Bicuspid Aortic Valve and Ventricular Septal Aneurysm.

BACKGROUND Bicuspid aortic valve is the most common congenital heart malformation, encountered in 1-2% of the population, while interventricular septal defect and patent ductus arteriosus are the most common congenital malformations associated with bicuspid aortic valve. Although bicuspid valve can have no clinical manifestation, patients with bicuspid valve are prone to develop vascular abnormalities. Aortic dilatation is the most common of these abnormalities, which in turn can lead to serious complications and often requires surgical treatment. Coexistence of bicuspid aortic valve and interventricular septal aneurysm is very extremely rare. CASE REPORT We present a very rare case of a female patient with combined congenital cardiac pathology. The patient was asymptomatic until age 68 years, and presented with nonspecific persistent cough. The diagnostic work-up for the unexplained cough showed normal function of the bicuspid valve and an ascending aorta aneurysm accompanied with interventricular septal aneurysm. The patient was referred for surgery. The diagnostic work-up and the decision for surgical treatment were thoroughly discussed to determine whether it was a true or a false aneurysm. CONCLUSIONS The presented case is an example of late diagnosis of a congenital cardiac defect. The silent evolution and the scarce clinical presentation led to incidental discovery of the pathology, which was fully assessed only by computed tomography. Although echocardiography is essential for discovering heart defects, non-invasive imaging techniques are required for detailed morphological assessment and for planning optimal surgical treatment.

Long-term Follow-up of Partial Bleb Excision in Late-onset Bleb-related Complications by a Single Surgeon Using Conjunctival Advancement.

PURPOSE: The purpose of this study was to describe the long-term results of partial bleb excision in late-onset bleb-related complications by a single experienced surgeon using the same surgical technique. PATIENTS AND METHODS: This was a retrospective study of 21 eyes of 11 women and 10 men aged 34 to 87 years (mean 64±12.8 y) who underwent first repair of late-onset bleb leaks with or without numerical hypotony (NH) and dysesthesia. The surgical technique consists of removing nonviable conjunctiva until the functional tissue becomes visible, thus adapting to individual conditions, and later conjunctival advancement. Complete success was defined as maintenance of intraocular pressure control without additional bleb revision, surgery, or glaucoma medications. Qualified success met these criteria, but with glaucoma medications. RESULTS: The mean follow-up was 5.6±4.4 years (1 to 17 years). Sixty-two percent of the cases were considered complete success, and a moderate number of cases (19%) needed glaucoma medication for achieving qualified success at the end of the follow-up period. Interestingly, bleb leak with NH seems to have long-term outcomes, like the other bleb-related complications (in terms of success and failures), with a significant intraocular pressure increase at 1 month after revision that tended to remain within normal values and lead to visual acuity recovery without recurrent NH. CONCLUSIONS: Partial bleb excision seems to be a good technique for different late-onset bleb-related complications. Bleb leak with NH showed a good long-term response, like the other bleb revision indications.

Diagnostic challenges in late-presenting congenital diaphragmatic hernia: a 16-year experience from tertiary care centre in North India.

With increasing use of ultrasound screening, the prenatal diagnosis of congenital diaphragmatic hernia (CDH) in better resourced areas has become the norm. However, early diagnosis is still not universal in resource-poor settings and late presentations of CDH continue. We retrospectively analysed the medical records of children operated for late-presenting CDH from 2001 to 2016 at our tertiary care centre in North India. A total of 32 patients were operated during the period with a male-to-female ratio of 3:1. Of these, 78% presented with respiratory symptoms, 37% with recurrent vomiting and 18% with an acute abdomen. Nine (28%) had been treated erroneously for gastroenteritis and another six (18%) had received anti-tubercular therapy for variable periods. A plain chest radiograph with a Ryle's tube in situ was confirmatory in 75% (24/32). In conclusion, initial misdiagnosis and subsequent unnecessary therapeutic interventions were the leading cause of morbidity .

Late and sustained intraocular pressure elevation related to intravitreal anti-VEGF injections: Cases requiring filtering surgery.

We report cases of delayed, sustained elevated intraocular pressure (IOP) associated with repeated intravitreal anti-VEGF injections (IVI), which ultimately resulted in the need for filtering surgery. Two of the three cases demonstrated severe IOP elevation despite maximal medical treatment following unilateral IVI and required urgent filtering surgery. Optic nerve involvement was severe in all three cases. These intravitreal injections were performed for exudative age-related macular degeneration (AMD), and the patients did not show any sign of glaucoma or ocular hypertension prior to the initiation of treatment. Elevated IOP secondary to intravitreal steroids is a well-known side effect, as is immediate transient IOP elevation associated with anti-VEGF injection. Late, sustained IOP elevation after repeated injections of anti-VEGF, described approximately ten years ago, is often underestimated. Its incidence is estimated between 2.1 % and 13 % according to studies and increases with the number of IVI (cumulative effect). The pathophysiologic process is becoming increasingly understood, and several risk factors for this chronic IOP elevation have been identified. Most often, it is a moderate IOP elevation for which topical monotherapy is sufficient, or sometimes two, three or four medications or even selective laser trabeculoplasty (SLT). However, filtering surgery may rarely be required. Our findings illustrate a little-described phenomenon: a sudden, severe, late IOP elevation in response to anti-VEGF by an "overflow" effect, requiring urgent filtering surgery.

Sialocutaneous fistula to the external auditory canal repaired with superficial parotidectomy and temporoparietal flap: A case report.

RATIONALE: Gustatory otorrhea can lead to cutaneous changes, recurrent infection, and social disruption. We present a case of a late, evolving sialocutaneous fistula to the external auditory canal, managed surgically after failing conservative therapies. This case is unique by late evolution, whereby the symptoms presented with significance 27 years after her operation and 19 years after mild symptoms initially arose. PATIENT CONCERNS: Gustatory, left-sided clear otorrhea with acutely increased volume over 8 months causing social disruption. DIAGNOSES: Sialocutaneous fistula to the external auditory canal. INTERVENTIONS: Superficial parotidectomy and temporoparietal flap for closure of fistula. OUTCOMES: No postoperative complications and resolution of gustatory otorrhea at one-year follow-up. LESSONS: This rare, but important, postoperative complication can present late with evolving symptoms, causing significant social disruption. It can be treated with conservative medical management and several surgical approaches.

Hernia diafragmática congénita de aparición tardía: la importancia de la clínica / Tardive appearing congenital diaphragmatic hernia: the importance of the symptoms

El diagnóstico de hernia diafragmática es raro después del período neonatal. Presentamos a un lactante de 8 meses con decaimiento y diagnóstico final de hernia diafragmática, ya que se trata de una presentación atípica, en una edad poco común y que presentó una evolución tórpida, a fin de difundir el conocimiento de esta entidad y sus posibles presentaciones a la comunidad científica (AU)

Diaphragmatic hernia diagnosis after neonatal period is a rare issue. We show a 8 month-baby with decay and final diagnosis of late-onset diaphragmatic hernia, because it is an atypical presentation, an uncommon age, and a torpid evolution, thus, it would be interesting to spread this subject to scientific community (AU)

Clinical and pathological challenges in the diagnosis of late-onset biliary atresia: four case studies

Biliary atresia (BA) is classically described at the neonatal age. However, rare cases of BA in older infants have also been reported. We report four cases of late-onset BA in infants older than 4 weeks (3 males, 1 female), and describe the diagnostic and management difficulties. One of the cases had a late-onset (29 weeks) presentation with a successful surgical procedure. We highlight the importance of this unusual differential diagnosis in infants with cholestatic syndrome, who may benefit from Kasai surgery, regardless of age.

Clinical and pathological challenges in the diagnosis of late-onset biliary atresia: four case studies.

Biliary atresia (BA) is classically described at the neonatal age. However, rare cases of BA in older infants have also been reported. We report four cases of late-onset BA in infants older than 4 weeks (3 males, 1 female), and describe the diagnostic and management difficulties. One of the cases had a late-onset (29 weeks) presentation with a successful surgical procedure. We highlight the importance of this unusual differential diagnosis in infants with cholestatic syndrome, who may benefit from Kasai surgery, regardless of age.