Nasal Pyriform Aperture Stenosis in a Newborn: When to Operate.

Congenital nasal pyriform aperture stenosis (CNPAS) is a very rare cause of neonatal respiratory distress and is often missed because of its rarity. It arises from the overgrowth of the nasal process of the maxilla. Maxillofacial CT scan findings of pyriform aperture width <11 mm in a full-term baby, median central incisor, triangular-shaped palate, and median palatal ridge confirm the diagnosis. We describe here a case of CNPAS admitted with respiratory distress that increased further on feeding. An infant feeding tube of size 6 was not negotiable through the nostrils. Resistance was appreciated at the inlet of the nostril. Maxillofacial CT showed pyriform aperture stenosis of 3.4 mm, suggesting CNPAS. The child could not be weaned off a high-flow nasal cannula despite conservative management with decongestants, steroids spray, dilatation, and stenting for 20 days. Subsequently, surgical widening of the nasal aperture by a sublabial approach was done. The child was discharged on the 10th postoperative day on full oral feeds. It is important to suspect CNPAS in neonates with respiratory distress where other common causes have been ruled out, as it can be treated by surgery in cases refractory to conservative management.

Laparoscopic choledochal cyst excision and biliary reconstruction in patients with previous surgery/ intervention: Feasibility and outcome.

INTRODUCTION: The aim of the study was to evaluate the feasibility and outcome of laparoscopic surgery in complicated choledochal cyst (CDC) with previous interventions (laparotomy or biliary drainage). PATIENTS AND METHODS: Patients with CDC who underwent surgery from July 2014 to July 2019 were evaluated. CDC without previous interventions (Group A) was compared with CDC that had previous interventions (Group B) to assess the feasibility and outcome of laparoscopic surgery. RESULTS: In 5 years' period, 38 patients were operated for CDC. The mean age was similar in both groups (3.78 ± 2.27 in Group A and 4.08 ± 2.73 in Group B). Out of six CDC with previous intervention (Group B), five patients were previously managed at other institutions as follows: (1) Laparoscopic cholecystectomy and endoscopic retrograde cholangiopancreatography (ERCP) stenting. (2) Laparotomy for biliary peritonitis and ERCP. (3) Percutaneous drainage of the large cyst. (4) Laparoscopic cholecystectomy. (5) ERCP stenting. (6) Percutaneous drainage for biliary ascites. All patients underwent laparoscopic CDC excision and hepatico-duodenostomy. The mean duration of surgery was 160.3 ± 17.22 in Group A and 169.2 ± 17.5 in Group B ( P = 0.258). None required intraoperative blood transfusion. None had a bile leak. Drain was removed at 4.47 ± 0.98 in Group A, while at 4.17 ± 0.75 days in Group B ( P = 0.481). There was statistically no significant difference in feed starting time or length of stay. In follow-up of 6 months-3 years, all patients are asymptomatic. CONCLUSIONS: Laparoscopy in complicated CDC with previous intervention is technically tedious but is feasible. The procedure is safe and delivers a good outcome.

Retrospective Observational Study Amidst Myriad Conundrums and Myths of Pediatric Headaches: A Critique on Diagnostics and Effectiveness of Interventions.

Objective To study the etiological profile of pediatric headaches (PH) in a tertiary child neurology clinic and to determine the utility of diagnostics, interventions, and long-term prognosis. Methods Children (ages 4-15) observed over four years were recruited retrospectively. In primary headaches, the headache frequency and impact on quality of life (QOL) parameters at pre-treatment (T1) were compared post-treatment at follow-up (T2). Results Of the 311 eligible patients, 285 had primary headaches (Tension-Type Headache {TTH}: 156; Migraine: 129), and 26 had secondary headaches. The mean (±SD) onset age was 10 (±3) years with a male-to-female ratio of 2.3:1. Migraine was more common in children aged less than seven years (17/28) and TTH in older patients (146/283). The most common causes of secondary headache were intracranial hypertension (ICH) in 11/26 patients (four idiopathic intracranial hypertension (IIH), four following aseptic meningitis, three with cortical vein thrombosis), and ophthalmologic causes in 7/26 (of these five had convergence insufficiency). Hypertension was a rare cause of secondary headaches (2/26 patients). Neuroimaging was performed in 173/311 (56%), primarily for parental anxiety (160/173; 92%), and was abnormal in only four. At T2 (Median time to follow-up: 29 months; Interquartile range: 22-37 months), data were collected in 207/285 patients with primary headaches (TTH: 109; Migraine: 98). In both migraine and TTH groups, there were statistically significant reductions (p-value <0.0001) in headache frequency and QOL parameters. Conclusion In our study, TTH was the most common cause of PH. Neuroimaging was normal in most cases. Psychological interventions were effective but underutilized. The symptoms of primary headaches improved significantly over time, despite poor adherence to prophylactic medications.

Improving cardiovascular magnetic resonance access in low- and middle-income countries for cardiomyopathy assessment: rapid cardiovascular magnetic resonance.

AIMS: To evaluate the impact of a simplified, rapid cardiovascular magnetic resonance (CMR) protocol embedded in care and supported by a partner education programme on the management of cardiomyopathy (CMP) in low- and middle-income countries (LMICs). METHODS AND RESULTS: Rapid CMR focused particularly on CMP was implemented in 11 centres, 7 cities, 5 countries, and 3 continents linked to training courses for local professionals. Patients were followed up for 24 months to assess impact. The rate of subsequent adoption was tracked. Five CMR conferences were delivered (920 attendees-potential referrers, radiographers, reporting cardiologists, or radiologists) and five new centres starting CMR. Six hundred and one patients were scanned. Cardiovascular magnetic resonance indications were 24% non-contrast T2* scans [myocardial iron overload (MIO)] and 72% suspected/known cardiomyopathies (including ischaemic and viability). Ninety-eighty per cent of studies were of diagnostic quality. The average scan time was 22 ± 6 min (contrast) and 12 ± 4 min (non-contrast), a potential cost/throughput reduction of between 30 and 60%. Cardiovascular magnetic resonance findings impacted management in 62%, including a new diagnosis in 22% and MIO detected in 30% of non-contrast scans. Nine centres continued using rapid CMR 2 years later (typically 1-2 days per week, 30 min slots). CONCLUSIONS: Rapid CMR of diagnostic quality can be delivered using available technology in LMICs. When embedded in care and a training programme, costs are lower, care is improved, and services can be sustained over time.

Use of rapid cardiac magnetic resonance imaging to guide chelation therapy in patients with transfusion-dependent thalassaemia in India: UMIMI study.

AIMS: To explore the impact of incorporating a faster cardiac magnetic resonance (CMR) imaging protocol in a low-middle-income country (LMIC) and using the result to guide chelation in transfusion-dependent patients. METHODS AND RESULTS: A prospective UK-India collaborative cohort study was conducted in two cities in India. Two visits 13 months apart included clinical assessment and chelation therapy recommendations based on rapid CMR results. Participants were recruited by the local patient advocate charity, who organized the patient medical camps. The average scanning time was 11.3 ± 2.5 min at the baseline and 9.8 ± 2.4 min (P < 0.001) at follow-up. The baseline visit was attended by 103 patients (mean age 25 years) and 83% attended the second assessment. At baseline, 29% had a cardiac T2* < 20 ms, which represents significant iron loading, and 12% had left ventricular ejection fraction <60%, the accepted lower limit in this population. Only 3% were free of liver iron (T2* ≥ 17 ms). At 13 months, more patients were taking intensified dual chelation therapy (43% vs. 55%, P = 0.002). In those with cardiac siderosis (baseline T2* < 20 ms), there was an improvement in T2*-10.9 ± 5.9 to 13.5 ± 8.7 ms, P = 0.005-and fewer were classified as having clinically important cardiac iron loading (T2* < 20 ms, 24% vs. 16%, P < 0.001). This is the first illustration in an LMIC that incorporating CMR results into patient management plans can improve cardiac iron loading. CONCLUSION: For thalassaemia patients in an LMIC, a simplified CMR protocol linked to therapeutic recommendation via the patient camp model led to enhanced chelation therapy and a reduction in cardiac iron in 1 year.

Thoracoscopic excision of double-pulmonary sequestration in left haemithorax, without anomalous blood supply (supply from pulmonary artery) in a neonate: First case.

Pulmonary sequestration (PS) is a rare anomaly. PS is a mass of abnormal pulmonary tissue that does not communicate with the tracheobronchial tree and is supplied by an anomalous systemic artery. Although aberrant systemic arterial supply is considered the key element to diagnose PS, rarely it can have arterial supply from the pulmonary artery as a spectrum of sequestration. Here, we present an unusual case of double (upper and lower) extralobar sequestration, present unilaterally (left haemithorax) in a neonate, without anomalous blood supply (supply from the left pulmonary artery and drainage into the superior and inferior pulmonary veins), not reported in literature earlier, to the best of our knowledge. In the present case, a child presented with respiratory discomfort at birth, requiring surgery due to inability to wean off respiratory support. It is important to be aware of this variant of sequestration spectrum. In a 18 days old child, both pulmonary sequestrations were resected thoracoscopically, making it a rare case, not described earlier in literature.

Giant Omphalocele Complicated by Postoperative Duodenal Obstruction.

Omphalocele is a congenital defect in the abdominal wall, usually treated at birth or within 1-2 years of life depending on condition of patient and size and contents of the defect. We repaired a giant omphalocele without mesh in a 9-year-old girl. She developed duodenal obstruction in the postoperative period requiring another laparotomy and duodeno-jejunostomy to bypass obstruction.

Lysinuric Protein Intolerance Presenting with Recurrent Hyperammonemic Encephalopathy.

BACKGROUND: Lysinuric protein intolerance is an inherited disorder of transport of cationic amino acids, causing amino aciduria. CASE CHARACTERISTICS: A 3-year-old boy with 12 month history of episodic change in behavior (decreased sleep, poor interaction), stunted growth and hyperammonemia. OUTCOME: Genetic analysis revealed a homozygous mutation, c.158C>T (p.Ser53Leu) in exon 1 of SLC7A7 gene. With appropriate management of hyperammonemia episodes, his neurodevelopmental outcome is normal. MESSAGE: Lysinusic protein intolerance is a potentially treatable disorder and should not to be missed.

Ectopic pancreas: a diagnostic dilemma.

An ectopic pancreas is defined as pancreatic tissue lacking vascular or anatomic communication with the normal body of the pancreas. It is rarely symptomatic as it is found incidentally at laparotomy most of the time. Despite advances in diagnostic modalities, it still remains a challenge to the clinician to differentiate it from a neoplasm. It is prudent to differentiate it from neoplastic etiologies, as simple surgical excision can potentially be curative. We discuss the presentation, diagnosis, and treatment of an interesting case of ectopic pancreas presenting as a gastric antral tumor.

Intrasphincteric botulinum toxin versus pneumatic balloon dilation for treatment of primary achalasia.

GOALS: Despite a high success rate, pneumatic dilatation for achalasia is accompanied by a significant risk of esophageal perforation. Injection of botulinum toxin (botox) into the lower esophageal sphincter (LES) can lead to improvement in symptoms with reduced risk of complications. Direct comparisons of the two techniques are needed to define their role in clinical management. STUDY: We compared pneumatic dilatation to botox for patients with achalasia using a double blind, randomized study design. Patients underwent clinical, manometric, radiographic and endoscopic evaluation to confirm primary achalasia. They were randomized to receive either 80 units of botox into the LES or Witzel balloon dilatation. Patients also received sham dilatation or injection, respectively. The patients and investigators assessing symptom response were blinded to therapy. Symptoms and esophageal function were assessed at 3 weeks, 3 months and 1 year after therapy. Treatment failure was defined as the lack of decrease in symptom grade more than 1 or recurrence of symptoms. Patients with treatment failure crossed over to the alternative treatment. RESULTS: Thirty four patients were studied, and 31 completed the trial. Of the 18 patients randomized to Witzel dilatation, 16 (89%) of 18 remained in clinical remission. Of the two patients with treatment failure, one responded to botox injection. Of the 16 patients randomized to botox, (38%) 6 of 16 remained in clinical remission. Four patients had initial failure, and 6 relapsed at a mean of 4 months after therapy. Of the nine patients who crossed over to dilatation, seven responded well, but two required surgical management of perforation. Although both treatments had excellent initial clinical improvement, patients randomized to Witzel dilatation had superior long-term success ( < 0.01). CONCLUSION: Initial therapy with Witzel dilatation is associated with better long-term outcome than a single injection of botox. Because of the risk of endoscopic perforation, botox remains a viable alternative to dilatation.