ABSTRACT
A case of a female child born at full term after normal vaginal delivery with bilateral secondary complete cleft palate and vomerine hamartoma mimicking intra-oral midline encephalocoele. Radiologically the tumour was confined to the vomer without intra-cranial extension. The lesion was occupying the oral cavity causing feeding problem. Surgical excision of the tumour at the age of six months and two flaps palatoplasty at the age of twelve months were performed. On histopathology the lesion turned out to be a lipomatous hamartoma of a benign nature. The child was followed for 9 years with no evidence of recurrence and a satisfactory speech. To our knowledge this is the first report of cleft palate with vomerine hamartoma in the English literature.
Subject(s)
Proteus Syndrome/surgery , Absorbable Implants , Bone Plates , Bone Screws , Child, Preschool , Diagnosis, Differential , Female , Follow-Up Studies , Frontal Bone/abnormalities , Frontal Bone/surgery , Humans , Parietal Bone/abnormalities , Parietal Bone/surgery , Proteus Syndrome/diagnosis , Temporal Bone/abnormalities , Temporal Bone/surgeryABSTRACT
Infantile systemic hyalinosis (ISH) is a rare familial autosomal recessive disease of unknown etiology. The clinical features are evident either at birth or within 6 months of life. The presentation is painful progressive joint contractures, thickened skin with hyperpigmentation over prominences, small pearly facial papules, gingival hypertrophy, fleshy nodules in the perianal region, diarrhea, increased susceptibility to bone fractures, infections, and failure to thrive. This is a progressive disorder that may lead to death within first 2 years of life, mostly due to recurrent chest infection and diarrhea. Two patients with ISH, one aged 14 years and another aged 10 years, with all the clinical features, though crippled but surviving, were seen at our center. Debulking of hypertrophic gingiva and excision of some symptomatic skin masses in these patients are indicated for comfort and smooth nursing care of the patients and to allow better rehabilitation.
Subject(s)
Collagen Diseases/pathology , Fibromatosis, Gingival/pathology , Hyalin/metabolism , Adolescent , Child , Collagen Diseases/metabolism , Consanguinity , Contracture/pathology , Female , Fibromatosis, Gingival/surgery , Humans , Skin Diseases/metabolism , Skin Diseases/pathology , Skin Diseases/surgeryABSTRACT
Seventy-nine (8.4%) patients during June 1992-May 1996 (Group-1) and 68 (7.2%) patients from June 1996 to May 2000 (Group-2) who developed septicaemia at the burns unit of Al-Babtain Centre for Plastic Surgery and Burns, Kuwait, were retrospectively studied and compared. The mean age of 26 years, male predominance, flame burns as main aetiology and mean burn percentage of >or=40% was observed in both the groups. Both groups revealed extensive flame burn, inhalation injury, intubation and difficult resuscitation as the risk factors. The proportion of satisfactory resuscitation increased significantly (P<0.001) in Group-2. The septicaemia commonly occurred within 2 weeks postburn but the number of episodes during 5 days postburn was less in Group-2. The surface wound was found to be the likely source of entry of the organisms into the blood stream in both the groups. The gram positive organisms were dominant aetiologic factor in both groups but an increase frequency of Acnetobacter was found in Group-2. The proportion of MRSE and Pseudomonas septicaemia was significantly higher (P<0.01) in the Group-1. The rate of survivors, in both the groups was higher among operated patients but it was significantly higher (P<0.001) in the Group-1. A mortality rate 20.6% in Group-2 decreased against Group-1, which can be attributed to better resuscitation, nutritional care, early detection of septicaemia, appropriate antibiotics and early wound excision and skin grafting. MOF was the cause of death of 60.9% in Group-1 and 85.7% in Group-2. There was no role of prophylactic antibiotic in burn patients in the incidence of septicaemia and mortality.
