Ipsilateral keratoconus associated with long-standing primary hydatid cyst of the orbit

Hydatid cyst is a cyclozoonotic infection of the larvae form of a platyhelminthes Echinococcus granulosus. The majority of hydatid cysts appear in the liver (65%) and lungs (25%). Kidneys and brain are other less common sites for this disease. Only 1% to 2% cases are seen in the maxillofacial region. These commonly appear as cystic lesions located in the mandible, maxillary sinus, orbit, infratemporal fossa, pterygopalatine fossa, parapharyngeal space, tongue, and parotid and submandibular salivary gland. Hydatid cysts of the orbit are rare and account for 1% of all hydatid cysts. The article presents hydatid cyst of the orbit in a 10-year-old child. Clinical features, investigations, surgical approaches, and adjuvant medical management have been emphasized. We believe that the lateral orbital route allows excellent exposure and safe removal of an intraorbital hydatid cyst located posteriorly, superiorly, and laterally without damaging the surrounding important orbital structures. Upper blepharoplasty incision results in good cosmetic outcome.     

Aggressive pilomatrixoma

We report a case of aggressive pilomatrixoma (PMX) in a 25‐year‐old male who presented with swelling in left supraclavicular region of 2‐month duration. A diagnosis of small round cell tumor was suggested on fine‐needle aspiration cytology. He underwent wide excision of the mass. On histomorphological examination, a diagnosis of aggressive PMX was made. The swelling recurred after 3 months of complete resection and on examination had similar morphological features. The case is presented because of the potential diagnostic difficulties on cytological examination and rare occurrence of aggressive variant of PMX. Diagn. Cytopathol. 2014;42:906–911. © 2014 Wiley Periodicals, Inc.     

Utility of single luteinizing hormone determination 3?h after depot leuprolide in monitoring therapy of gonadotropin-dependent precocious puberty

To determine utility of luteinizing hormone (LH) estimation, post intramuscular (IM) depot leuprolide in comparison with subcutaneous leuprolide stimulation test. Test for monitoring therapy in patients with gonadotropin dependent precocious puberty (GDPP). In seven patients of GDPP, who were treated with 11.25 mg depot leuprolide, the LH peak after subcutaneous (sc) test was compared with LH at hourly interval for 4 h after IM depot leuprolide for 13 tests and 3rd hour value for next ten tests. These two values were compared both before and after therapy. Before therapy, the mean ± SD LH peak after subcutaneous leuprolide stimulation test was 20.6 ± 7.85 IU/l (range 9.64–30.4 IU/l), and it was 27.3 ± 12.21 IU/l 3 h after the first depot leuprolide injection (range 10.5–45.4 IU/l). During therapy, the mean ± SD of LH peak after sc stimulation test was 1.96 ± 0.75 IU/l (range 1.1–3.1 IU/l), and it was 2.58 ± 0.54 IU/l (range 1.4–3.4 IU/l) 3 h after depot leuprolide injection. Three-hour LH value following IM depot leuprolide injection (11.25 mg) can be used for monitoring therapy in patients with GDPP because of its convenience and cost effectiveness.     

Radiotherapy in paediatric Cushing’s disease: efficacy and long term follow up of pituitary function

Pituitary radiotherapy (RT) is an effective second-line treatment for paediatric Cushing’s disease (CD). We report long-term efficacy and anterior pituitary function in a cohort of paediatric CD patients treated with RT. Between 1988 and 2008, from our cohort of 48 paediatric CD patients, eight paediatric CD patients (5 males and 3 females) underwent second-line pituitary RT (45 Gy in 25 fractions), following unsuccessful transsphenoidal surgery. Out of eight whose long term follow up is available, four patients were cured by RT. Minimum follow up of 2 years is available for all patients. Four patients achieved cure after RT. Two patients were not cured even after follow up of 60 and 132 months, respectively. Out of four uncured patients, two of them had suppressible low dose dexamethasone cortisol with altered circadian rhythm suggesting possibility of response in near future with follow up of just 26 months. Five patients were hypogonadal and one patient was hypothyroid. All patients were below their target height at the time of last follow up. None of the patients had posterior pituitary dysfunction. This series of patients illustrates the efficacy and long-term follow up of pituitary function in children with CD treated with RT. This study also emphasizes the need of growth hormone statues assessment and timely intervention.     

Effect of acute exacerbations on skeletal muscle strength and physical activity in cystic fibrosis

BackgroundSkeletal muscle weakness is an important complication of chronic respiratory disease. The effect of acute exacerbations on strength in patients with cystic fibrosis is not known.MethodsQuadriceps (QMVC) and respiratory muscle strength were measured in patients at the time of acute admission, at discharge and one month later. Patients wore an activity monitor during admission and at one month. Convalescent values were compared to the stable clinic population.ResultsData were available for 13 acute admissions and 25 stable CF outpatients. Strength and other parameters including daily step count did not differ significantly between the stable and one month post-admission groups. At admission, QMVC was 16.7 (8.3)% lower than at convalescence, whereas inspiratory muscle strength did not change significantly. Reduction in QMVC did not correlate with activity levels or with markers of systemic inflammation.ConclusionFurther research is needed to identify the mechanisms responsible for the reduction in QMVC.     

Nonvascularized bone grafting for mandibular reconstruction: myth or reality?

This retrospective study analyzes the long-term results and complications of mandibular reconstruction with nonvascularized bone grafts (NVBGs) and suggests refinements. A total of 166 patients underwent mandibular ablation and primary reconstruction between June 1990 and August 2009, of which 101 were NVBGs, 27 were vascularized bone grafts, and 38 were with functional plate reconstruction. Eighty-seven of 101 patients undergoing NVBG, having all records and followed up for a minimum of 12 months, were included in this study. The analysis criteria included site of defect, surgical approach, method of graft fixation, bony continuity and stability, presence or absence of infection, aesthetic and functional prosthetic rehabilitation, donor-site morbidity, and clinical and radiological changes in the reconstructed area.The overall success with NVBGs in our study was 77 (88.5%) of 87. We used ilium in 68 cases (78.16%), fibula in 16 cases (18.39%), and rib in 3 cases (3.44%). Ten cases (11.5%) showed complete failure due to infection (7 cases) and resorption (3 cases).Nonvascularized bone grafts could be used judiciously for reconstruction of selective mandibular resection defects where there is not much of soft tissue loss, or where 2-layer watertight closure can be achieved intraorally and extraorally. Where only a single-layer intraoral closure is anticipated, either additional soft tissue should be brought submucosally or there should not be extraoral-intraoral communication even during surgery. Also precise patient selection, surgical planning, and execution with meticulous nursing care are keys to success.     

Establishment of nomograms and reference ranges for intra-cranial ventricular dimensions and ventriculo-hemispheric ratio in newborns by ultrasonography

AIM: To establish normal ranges for the lateral, third, and fourth ventricular dimensions and the ventriculo-hemispheric ratio (VHR) in neonates using cranial ultrasonography. METHOD: Intra-cranial ventricles were assessed ultrasonographically using anterior fontanelle as acoustic window. Data were analyzed to determine correlation, coefficient of determination (R2), regression equations and plotted against gestational age (GA). RESULTS: Of total 1483 neonates (25-42 w GA), 372(25%) had GA < 34 weeks. GA strongly correlated with anterior horn width (AHW; 0.92), thalamo-occipital distance (TOD; 0.94), and third (0.78) and fourth (0.89) ventricular widths. The R2 values were 0.85, 0.88, 0.66 and 0.80, respectively. VHR had weak correlation (0.12, R(2)= 0.005) with GA and stayed constant (0.27-0.28) across GA. Nomograms of AHW, TOD, and third and fourth ventricular widths were constructed with respect to GA. CONCLUSION: Intra-cranial ventricles increase in size with GA but this increase is limited to only a few mm and is proportional to increment in brain tissue. Nomograms and regression equations of AHW, TOD and widths of third and fourth ventricles can assist in objectively assessing ventricular sizes. The almost uniform VHR can be used to screen ventricle size while suspecting hydrocephalus.     

Association of temperament with dental anxiety and behaviour of the preschool child during the initial dental visit

The aim of this study was to investigate whether there is an association between temperament characteristics of preschool children, dental anxiety, and their dental behaviour. A total of 100 children, aged 3–5 yr, who were attending their initial dental visit accompanied by a parent, were included in this cross‐sectional study. Dental anxiety of children was measured using the Facial Image Scale. The behaviour of children during the initial oral examination and oral prophylaxis was assessed using Frankl's behaviour rating scale. Temperament was assessed using Emotionality, Activity, Shyness Temperament Survey for Children (parental ratings). Statistically significant weak linear positive correlations were seen between the following: the percentage duration of definitely negative behaviour and shyness scores (r_s = 0.28); anxiety level and emotionality scores (r_s = 0.28); and anxiety level and shyness scores (r_s = 0.26). Multinomial logistic regression analysis revealed that children with higher anxiety had higher odds of showing definitely negative behaviour, which decreased with increasing age of the child. Emotionality and shyness temperaments may be weakly associated with dental anxiety, and shyness may be weakly associated with the dental behaviour of the preschool child. Definitely negative dental behaviour is associated with dental anxiety and age of the child.