- Choanal Atresia: Birth Without Breath. [Journal Article]
- IJIndian J Otolaryngol Head Neck Surg 2018; 70(1):53-58
- Bilateral Choanal atresia is a medical emergency. Corrective surgery is the mainstay of the treatment. Hegar's dilator was used in all cases to break the bony/membranous atretic plate. The 22 cases o...
Bilateral Choanal atresia is a medical emergency. Corrective surgery is the mainstay of the treatment. Hegar's dilator was used in all cases to break the bony/membranous atretic plate. The 22 cases of choanal atresia all operated by the first author were included in this study. Eight cases were 1-5 year old with bilateral choanal atresia and all required immediate surgery as they had repeated attacks of respiratory distress and cycle of cyanosis. Eight cases were of CHARGE Syndrome. All the cases were operated under general anaesthesia. Hegar's dilators were used and nasal stents were placed in all cases. Although complete nasal patency was achieved by surgery, in 8 cases, neonates could not survive due to the CHARGE Syndrome. The mortality was unrelated to the surgery. The 14 cases which were not related to CHARGE Syndroma had a good postoperative recovery. 3 cases were above 15 years old with unilateral complete bony/membranous choanal atresia and they presented with continuos nasal discharge. Hegar's dilators are a safe and simple method of surgery for choanal atresia. Nasal stenting is mandatory to prevent restenosis.
- Criss-cross heart: Transthoracic echocardiographic features. [Journal Article]
- IHIndian Heart J 2018 Jan - Feb; 70(1):71-74
- CONCLUSIONS: CCH is an extremely rare congenital cardiac anomaly. Superior-inferior ventricular relationship often co-exists with CCH, but is not necessarily present in all cases. CCH requires early diagnosis because of its common association with diverse cardiac anomalies.
- Arterial duct and pulmonary arteriovenous malformations: A shunt masking a shunt. [Journal Article]
- APAnn Pediatr Cardiol 2018 Jan-Apr; 11(1):89-91
- A 5-month-old infant, referred to our institution for percutaneous arterial duct (AD) embolization, showed multiple huge pulmonary arteriovenous malformations (PAVMs) associated with a small AD and s...
A 5-month-old infant, referred to our institution for percutaneous arterial duct (AD) embolization, showed multiple huge pulmonary arteriovenous malformations (PAVMs) associated with a small AD and several tiny systemic-to-pulmonary collaterals. This anatomic arrangement was a possible cause of lack of cyanosis and disproportionately higher hemodynamic relevance of the ductal shunt. The PAVMs became pathophysiologically evident immediately after the closure of AD and systemic to pulmonary artery collaterals and presented clinically with a life-threatening cyanosis. To improve the patient's clinical and hemodynamic condition, the PAVMs were closed in multiple sittings using a large number of Amplatzer Vascular Plugs (St. Jude Medical Inc., St. Paul, MN, USA). The hemodynamic burden of cardiac malformations resulting in left-to-right shunt may be magnified by the presence of PAVMs as a result of low pulmonary vascular resistance which in turn may completely mask the clinical impact of the latter. Transcatheter approach is life-saving in these complex arrangements.
- Metahemoglobinemia in infants over one year. [Journal Article]
- MCMed Clin (Barc) 2018 Feb 10
- CONCLUSIONS: Although the most frequent clinical manifestation is cyanosis associated with respiratory distress, methaemoglobinaemia can present with more severe and confusing symptoms. The main treatment is oxygen therapy at high concentrations and methylene blue as an intravenous antidote only in selected cases. The intake of vegetables is the main cause of this in infants, so it is important to give adequate information to parents about the consumption and preservation of these foods during the first year of life.
- Bascule syndrome associated with syncopal episodes. [Journal Article]
- PDPediatr Dermatol 2018 Feb 13
- Bascule syndrome is a recently described benign vasomotor dermatosis characterized by Bier anemic spots, cyanosis, and urticaria-like eruption. We report a case of a 13-year-old girl with cutaneous l...
Bascule syndrome is a recently described benign vasomotor dermatosis characterized by Bier anemic spots, cyanosis, and urticaria-like eruption. We report a case of a 13-year-old girl with cutaneous lesions consistent with Bascule syndrome who had had three exercise-related syncopal episodes. It would be recommended to exclude orthostatic intolerance or postural orthostatic tachycardia syndrome when evaluating patients with Bascule syndrome.
- [Digital ischemia revealing multiple myeloma]. [Journal Article]
- JMJ Med Vasc 2018; 43(1):61-64
- Digital ulcers generally arise in a context of microangiopathy-related focal ischemia. In women, connective tissue diseases are the main etiology, while in men the cause is often diffuse arterial dis...
Digital ulcers generally arise in a context of microangiopathy-related focal ischemia. In women, connective tissue diseases are the main etiology, while in men the cause is often diffuse arterial disease, e.g. Leo-Buerger disease, or emboligenic heart disease. A paraneoplastic origin of digital necrosis due to ischemia is rarely reported. A 75-year-old man presented with cyanosis of the fingertips and toes that had begun one month earlier. The physical examination found pulp ulcers on the fingers and toes of both hands and feet. Two weeks later, necrotic damage developed distally, with no other associated symptoms. Blood tests were suggestive of Kahler disease; immunodeficiency disorders tests were negative; the cyroglobulin test was positive. Multiple-drug chemotherapy was followed by clinical improvement. Distal necrotic damage is a frequent inaugural symptom in vascular disease. If the common causal mechanisms (iatrogenic, occupational, toxic, atheromatous, emboligenic heart disease, or systemic disease) have been ruled out, it is important to search for a blood disorder or cancer as the cause of distal necrotic damage.
- Congenital diaphragmatic eventration complicated with neonatal gastric perforation. [Journal Article]
- TMTunis Med 2017; 95(2):136-138
- Congenital diaphragm eventration is a rare and usually asymptomatic developmental defect. Neonatal gastric perforation is also a rare but lifethreatening condition. In our knowledge, the association ...
Congenital diaphragm eventration is a rare and usually asymptomatic developmental defect. Neonatal gastric perforation is also a rare but lifethreatening condition. In our knowledge, the association of these two pathologies has been, exceptionally reported. We report a case who illustrates clinical and radiological features of this possible co-morbidity. A full-term male neonate was born from uneventful pregnancy and delivery. The antenatal scan was reported as normal. At birth, clinical exam was normal, no special resuscitation was necessary. The newborn was examined and admitted the 4th day of life for fever, tachypnea, cyanosis, hemodynamic shock and refusing feeds. Clinical examination suggested peritonitis. Chest radiography and ultrasonography suggested congenital hernia. A laparotomy was performed after a brief resuscitation and confirmed the presence of diaphragm eventration with gastric perforation. Suturing of gastric perforation with a diaphragmatic plication was performed with favorable evolution.
- Phlegmasia Cerulea Dolens Following Heparin-Induced Thrombocytopenia. [Journal Article]
- IJIndian J Crit Care Med 2018; 22(1):51-52
- The authors present a case of a 49-year-old woman who underwent coronary artery bypass grafting after suffering from an acute coronary syndrome and later developed phlegmasia cerulea dolens with hepa...
The authors present a case of a 49-year-old woman who underwent coronary artery bypass grafting after suffering from an acute coronary syndrome and later developed phlegmasia cerulea dolens with heparin-induced thrombocytopenia (HIT) and HIT thrombosis (HITT).
- Challenges in the diagnosis of acute cyanide poisoning. [Journal Article]
- CTClin Toxicol (Phila) 2018 Feb 08; :1-9
- CONCLUSIONS: Contrary to general reviews published on cyanide toxicity, reports of cherry red skin and bitter almond odor were rare among published cyanide cases. Consistent with other studies, metabolic acidosis with significant lactic acidosis were the laboratory values consistently associated with cyanide toxicity. Healthcare providers may overlook cyanide toxicity in the differential diagnosis, if certain expected characteristics, such as the odor of almonds or a cherry red color of the skin are absent on physical examination.
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- Undiagnosed intraoperative methaemoglobinaemia. [Journal Article]
- IJIndian J Anaesth 2018; 62(1):72-74
- Methaemoglobinaemia is a rare but potentially dangerous haemoglobinopathy that is often underdiagnosed. It is one of the causes for unexplained cyanosis with dark-coloured blood, especially in the ab...
Methaemoglobinaemia is a rare but potentially dangerous haemoglobinopathy that is often underdiagnosed. It is one of the causes for unexplained cyanosis with dark-coloured blood, especially in the absence of cardiac or pulmonary pathology. Not uncommonly so, it is an incidental perioperative finding in cases of dark-coloured blood not improving with oxygen in apparently acyanotic patients. The present case report is of a child with deaf-mutism posted for cochlear implant surgery who presented with 'chocolate-coloured blood' in the surgical field, despite blood gas analysis showing a normal partial pressure of oxygen.