ABSTRACT
BACKGROUND: Extra-pituitary birth defect (EPBD) in children with congenital hypopituitarism is largely unknown. OBJECTIVE: The study aims to evaluate the incidence and pattern of EPBD in children with congenital hypopituitarism and to evaluate whether it can serve as a clue to diagnose this condition. PATIENTS AND METHODS: Retrospective analysis of hospital record of patients of short stature due to various etiology from which patients with congenital hypopituitarism with age ≥18 years were recruited for the analysis. Clinical, hormonal, radiological and ocular electrophysiological studies were done in all patients and all EPBD were noted. RESULTS: Twenty seven patients (79%) had multiple pituitary hormone deficiency (MPHD) of which growth hormone was universal followed by gonadotropin (62%), TSH (59%), ACTH (44%) and prolactin (12%). Nineteen patients (56%) had multiple EPBD in various combinations. Twenty three ocular abnormalities were present in 12 patients (35%). Nine patients (26%) had other associated EPBD along with ocular abnormalities while 3 had ocular abnormalities without any other associated birth defect. Skeletal defects were present in 10 patients (29.5%). On the contrary, 5 patients in the EPBD group had total 15 visual defects. The most common abnormality of the visual system were abnormal visual evoke response (VER, 18%), followed by strabismus (15%), visual acuity (VA, 12%), electroretinogram (ERG) and electrooculogram (EOG) 8% each and visual field defect 6%. There was a trend towards early age at presentation with EPBD. CONCLUSIONS: Presence of EPBD in a short child is a sensitive marker to diagnose congenital hypopituitarism. Subtle abnormalities of visual pathway without absent septum pellucidum or midline brain defects were common.
Subject(s)
Birth Injuries , Dwarfism , Hypopituitarism , Pituitary Gland/diagnostic imaging , Pituitary Hormones , Sella Turcica/diagnostic imaging , Adult , Birth Injuries/complications , Birth Injuries/epidemiology , Dwarfism/diagnosis , Dwarfism/epidemiology , Eye Abnormalities/diagnosis , Female , Growth Hormone/analysis , Humans , Hypopituitarism/congenital , Hypopituitarism/diagnosis , Hypopituitarism/epidemiology , Hypopituitarism/etiology , India/epidemiology , Magnetic Resonance Imaging/methods , Male , Pituitary Hormones/analysis , Pituitary Hormones/deficiency , Retrospective Studies , Septo-Optic Dysplasia/diagnosis , Septo-Optic Dysplasia/epidemiology , Statistics as Topic , Tomography, X-Ray Computed/methodsABSTRACT
AIM: To report the results of paediatric cataract surgery with primary intraocular lens (IOL) implantation. METHODS: Children with congenital or developmental cataract who underwent phacoaspiration with posterior chamber (PC) IOL implantation were retrospectively studied. Group A included children with polymethyl methacrylate, group B hydrophobic acrylic and group C silicone IOLs. Outcome measures were visual axis clarity, visual outcome and refractive changes. RESULTS: There were 230 children (381 eyes) with the age ranging from 1 months to 15 years. Group A comprised 208, group B 144 and group C 29 eyes. Posterior capsule opacification was seen in 38/208 in group A and 21/144 in group B and 4/29 eyes in group C. The mean refractive error at 2 weeks, 1 year and 3 years after cataract surgery in the age group ≤2 years was +3.38±3.07 D (median +3.75 D), +1.72 D±3.19 (median +1 D) and -0.51 D±3.59 (median -0.5 D); in the age group >2-8 years +0.84±3.18 D (median+1.5 D), +0.27 D±3.14 (median +0.5 D) and -0.62 D±2.81(median -0.75D); and in the age group >8 years -0.44±1.73 D (median -0.5 D), -0.70±1.77 D (median -0.75 D) and -0.89 D±1.60 (median -0.75D) respectively. Children ≤2 years had a significant myopic shift (p<0.001). LogMAR visual acuity was ≥0.3 in 62.2% of eyes in bilateral and 30.90% in the unilateral group. CONCLUSIONS: Paediatric cataract surgery with primary PCIOL implantation is safe. Refractive changes and PCO are the main hurdles for achieving optimal visual outcome.
