Crouzons syndrome: a case report.

Human skull is made up of many bone joints connected by sutures. The sutures fuse in later life after the complete growth of the brain. If any of these sutures closes early, it may interfere with the normal growth of the brain. The developing brain may exert pressure on the skull and may grow in the direction of the other open sutures. Premature sutural fusion may occur alone or together with other anomalies, making up various syndromes. Crouzons syndrome is an example of such a syndrome that is associated with premature synostosis of the sutures of the skull. Presented in this article is a case of Crouzons syndrome seen in a boy aged 9 years.

Crossed hemifacial hyperplasia: a diagnostic dilemma.

Crossed hemifacial hyperplasia is a rare condition producing facial asymmetry and overgrowth of the extremities of the opposite side. Very few cases are reported in literature. A case of an 11-year-old female is presented here to supplement existing clinical knowledge with many of the reported clinical and orofacial findings. Emphasis is placed on diagnosis of the condition by thoughtful elimination and the necessity of a multi-disciplinary approach to its management.

Versatility of orthognathic surgery in the management of maxillofacial deformities.

Orthognathic surgery is a surgical procedure which is widely practiced throughout world for the correction of various maxillofacial deformities. The procedure for correction of a particular deformity will be done after proper evaluation, which includes cephalometric and dental model analysis and photographs. The patient undergoes pre surgical orthodontic correction for the dental compensation after which the surgery is planned.

Total oral rehabilitation in a patient with portwine stains.

Sturge-Weber syndrome is an uncommon condition characterized by presence of Portwine stains on the face along with ocular disorders, mental retardation, oral involvement and leptomeningeal angiomas. A report of a case with atypical manifestations of this syndrome along with a step-by-step protocol oral rehabilitation of such patients is described.

Evaluation of intraperitoneal vincristine in malignant peritoneal effusion.

The efficacy and safety of intraperitoneal administration of vincristine sulphate was determined in mice bearing Ehrlich ascitic carcinoma. The tumor bearing animals were administered with 0.5 mg/kg body weight (b.wt) of freshly prepared vincristine sulphate intraperitoneally on day 6 after tumor transplantation followed by drug administration once daily 5 days a week consecutively. The observations regarding the survival, alteration in the volume of peritoneal fluid, increase in life span and pathological changes in the liver, kidney, gastrointestinal tract and bone tissues were made. The vincristine sulphate treatment reduced the malignant cell population significantly and there were no significant changes in the histological picture of liver, kidney, bone, except the intestine, where atropy of villi demonstrating nests and cords of uniform small round cells were observed. Our experimental data suggests that intraperitoneal administration of vincristine is beneficial in malignant peritoneal effusion.

A review of odontogenic keratocyst with a report of unusual occurrence in the maxilla.

Odontogenic keratocysts (OKC) are keratinizing epithelium-lined cycts of the jaws with well-defined histologic criteria and possessing one clinical feature warranting their recognition and separation as a distinctive entity, due to their exceedingly high recurrence rate. This report describes a case of OKC which occurred in the anterior maxilla in a 12 year old female child, with its treatment. These lesions are normally seen in the mandible, and are rare in the maxilla.

Systemic lupus erythematosus and sickle cell disease.

A case of 10-year-old girl with an unusual association of sickle cell disease (SCD) with systemic lupus erythematosus (SLE) is presented. The report discusses the clues to the diagnosis of this rare combination with review of relevant literature and highlights the diagnostic dilemma that may arise because of the similarity of symptoms.

Arteriovenous malformation of the mandible--report of a case.

An eight-year-old girl was referred for the management of recurrent bleeding from the lower, left gingival region and a diffused swelling on the left side of her face. Doppler study and carotid angiogram were suggestive of an arteriovenous malformation. Segmental mandibular resection was performed after ligation of the left external carotid artery. Post-operative period was uneventful and there has been no recurrence of the lesion till date.

Spindle cell carcinoma of right lower alveolus. A case report.

A case report of spindle cell carcinoma of the right mandibular alveolus is reported, which clinically mimicked a squamous cell carcinoma. Careful histological interpretation is necessary for successful treatment of such tumors. The use of radiation therapy after radical neck dissection is also stressed upon.

Capillary hemangioma--report of a case.

A ten-year-old girl was referred for the management of a recurrent lobulated mass present in the upper anterior region. Histopathological investigations were suggestive of capillary hemangioma. Surgical excision of the lesion was performed and postoperative recovery was uneventful.

Quantitation of proteinuria using protein-creatinine ratio in random urine samples.

Estimation of proteinuria in children is cumbersome when a 24 h urine collection is needed. In the presence of a stable glomerular filtration rate, the ratio of urinary protein and creatinine should reflect the protein excretion. One hundred samples of urine (24 h and random samples) were collected from 50 children with nephrotic syndrome, 25 with nephrotic syndrome in remission and 25 normal children. The 24 h urine total protein and random urine protein-creatinine ratio were assessed on these samples. Linear regression analysis of the results showed excellent correlation between the values (r = 0.81, p less than 0.001). A random urine protein-creatinine ratio of greater than 3.5 correlated with massive proteinuria, while a ratio less than 0.2 was suggestive of physiological values. The sensitivity, specificity, positive and negative predictive values of the protein-creatinine ratio in massive proteinuria were very high. We conclude that the random urine protein-creatinine ratio can be used reliably to assess the degree of proteinuria in children.

Neonatal citrobacter sepsis: clinical and epidemiological aspects.

Fourteen neonates were diagnosed to have Citrobacter sepsis during 1986-89, representing 4.6% of all cases with bacteriologically proven sepsis. Most of these infants were low birth weight (mean 2046 gm, +/- 750) and preterm (mean 34.8 weeks, +/- 3.8). Mean age at onset of sepsis was five days. In 10 cases the hematological profile was suggestive of sepsis. Infants had clinical evidence of multisystem infection; 2 with septic arthritis and 3 meningitis. The case fatality rate was 61%. Resistance to antibiotics was frequent. Citrobacter species were also cultured from other sites: umbilical stumps, eye swabs, urine, skin pustules and umbilical catheter tips. The epidemiological features and virulence of this organism call for vigilance and strict control measures.