Bhaskar Saikia

Isolated severe bilateral bronchomalacia.

Airway malacia is uncommon condition having symptoms similar to common respiratory illnesses. Any child having persistent wheeze during infancy should be evaluated for airway malacia. The authors report a case of isolated severe bilateral bronchomalacia managed with tracheostomy and continuous positive pressure ventilation.

Pachymeningitis in a young child responded to antitubercular therapy: a case report.

Pachymeningitis is a rare disease of diverse etiology mainly affecting the adult population. Only 4 pediatric cases have been reported till now. We report the youngest child with pachymeningitis from India. Our case responded very well to antitubercular therapy with near complete recovery. Antitubercular therapy can be considered in children from endemic countries with hypertrophic pachymeningitis before labeling their condition as idiopathic hypertrophic pachymeningitis.

Genetic Analysis in Bartter Syndrome from India

Bartter syndrome is a group of inherited, salt-losing tubulopathies presenting as hypokalemic metabolic alkalosis with normotensive hyperreninemia and hyperaldosteronism. Around 150 cases have been reported in literature till now. Mutations leading to salt losing tubulopathies are not routinely tested in Indian population. The authors have done the genetic analysis for the first time in the Bartter syndrome on two cases from India. First case was antenatal Bartter syndrome presenting with massive polyuria and hyperkalemia. Mutational analysis revealed compound heterozygous mutations in KCNJ1(ROMK) gene [p(Leu220Phe), p(Thr191Pro)]. Second case had a phenotypic presentation of classical Bartter syndrome however, genetic analysis revealed only heterozygous novel mutation in SLC12A gene p(Ala232Thr). Bartter syndrome is a clinical diagnosis and genetic analysis is recommended for prognostication and genetic counseling.

Isolated Cerebellar Tuberculoma

A 10-year-old boy presented with an 8 day history of intermittent headache, progressive loss of vision, and gait ataxia. Therewas no history of fever, vomiting, or seizures. He had normal cognition, bilateral optic disc edema, and cerebellar dysfunction. His elder sister had sputum positive for tuberculosis. Mantoux was 16 mm; erythrocyte sedimentation rate was 64 at 1 hour. Magnetic resonance imaging of the brain revealed multiple conglomerated ring-enhancing lesions in right cerebellum, basal exudates, hydrocephalus, and tonsillar herniation (Fig).

Ventriculo-peritoneal shunt: A rare cause of basal ganglia and thalamic abscess

We report an 18-month-old female child with ventriculo-peritoneal shunt related thalamic abscess treated with stereotactic aspiration. Deep seated abscesses are complex due to difficult access and are associated with an increased risk of intra-ventricular rupture as well as antibiotic resistance, a fact which justifies a more aggressive and immediate neurosurgical management.

Guillain-Barre syndrome masquerading as acute respiratory failure in an infant

Guillain-Barré syndrome (GBS) is a rare entity in infants. We report a case of GBS in a 5-month-old girl. The child presented with cough, loose stools, breathing difficulty, and listlessness. The child was treated as pneumonia with respiratory failure. Due to difficulty in weaning from ventilation with areflexia, marked hypotonia, and reduced power in all four limbs; possibilities of spinal muscular atrophy, poliomyelitis, and myopathies were kept. Nerve conduction velocity study was suggestive of mixed sensory-motor, severe axonal, and demyelinating polyradiculoneuropathy. Cerebrospinal fluid study revealed albuminocytological dissociation. Child was diagnosed as GBS and treated with intravenous immunoglobulin. Child recovered completely on follow-up. GBS should be considered as a differential diagnosis in acute onset respiratory failure with neuromuscular weakness in infants.

Isolated Severe Bilateral Bronchomalacia: Authors' Reply

To the Editor: Severe airway malacia is an uncommon entity and not rare as previously reported by us [1]. With availability of fiberoptic bronchoscopy the documentation of airway malacia is increasing in developing countries. Due to lack of registry, incidence cannot be estimated in our country. As already mentioned in previous report, external compression was ruled out with CT angiogram chest, which was normal. The index case was gradually weaned off from Bipap support and tracheostomy tube was decannulated. Child is currently growing well. Severe airway malacia is life threatening condition and should be managed at specialized centers. A registry should be maintained for estimation of incidence of airway malacia.

Injudicious use of anticonvulsants leading to life-threatening event

Epilepsy is common childhood problem and advent of newer anticonvulsant drugs has led to injudicious uses. Anticonvulsant hypersensitivity syndrome (AHS) has been previously described developing secondary to aromatic anticonvulsants such as phenytoin and carbamazepine. AHS developing secondary to non-aromatic anticonvulsant agents such as lamotrigine (LTG) and valproic acid (VPA) is rare. We present a case of an 11-year-old girl with known epilepsy on VPA, LTG and carbamazepine therapy that developed fever with the clinical pattern of Steven-Johnson syndrome/toxic epidermal necrolysis requiring pediatric intensive care. Judicious use of anticonvulsant should be done in appropriate dose especially in pediatric population and when used in combination to prevent AHS like life-threatening event.