ABSTRACT
Familial Mediterranean fever is an autosomal recessive disease characterized by recurring inflammatory attacks of synovial membranes. More than 95% of patients show peritoneal involvement which mimics acute abdomen and can sometimes cause unnecessary surgical intervention. The authors present two patients with the diagnosis of familial Mediterranean fever who underwent surgery because of rare abdominal complication of the disease. Two patients with the diagnosis of familial Mediterranean fever underwent laparotomy, and segmental small bowel resection was done because of the necrosis. Adhesive intestinal obstruction with associated bowel strangulation and volvulus is a rare complication of familial Mediterranean fever, and this life-threatening emergency must be kept in mind.
Subject(s)
Familial Mediterranean Fever/complications , Ileum/pathology , Ileum/surgery , Intestinal Volvulus/etiology , Intestinal Volvulus/surgery , Adolescent , Anastomosis, Surgical , Child , Humans , Ileostomy , Male , Necrosis/etiology , Necrosis/surgery , Tissue Adhesions/etiology , Tissue Adhesions/surgeryABSTRACT
Epidermolysis bullosa is a rare disorder which may present various systemic involvement. We present a 15-month-old boy with epidermolysis bullosa simplex who was admitted with symptoms of hematuria and dysuria and discuss the pathology from a urological standpoint.
Subject(s)
Epidermolysis Bullosa Simplex/complications , Epidermolysis Bullosa Simplex/pathology , Hydronephrosis/etiology , Ureteral Diseases/etiology , Urethral Obstruction/etiology , Dilatation, Pathologic/diagnostic imaging , Dilatation, Pathologic/etiology , Dilatation, Pathologic/pathology , Epidermolysis Bullosa Simplex/diagnostic imaging , Humans , Hydronephrosis/diagnostic imaging , Hydronephrosis/pathology , Infant , Male , Radiography , Ureteral Diseases/diagnostic imaging , Ureteral Diseases/pathology , Urethral Obstruction/diagnostic imaging , Urethral Obstruction/pathologyABSTRACT
Embryonal rhabdomyosarcoma of the common bile duct is a very rare malignancy of childhood. The radiological appearance of the lesion is similar to that of congenital choledochal cyst if there is no local invasion to the adjacent tissues. The authors present a case of embryonal rhabdomyosarcoma of the common bile duct which was considered to be a congenital choledochal cyst preoperatively, and they discuss this very rare childhood tumor, with a brief survey of the literature. In conclusion, it is important to know that this rare tumor can simulate congenital choledochal cyst; it should be considered in the differential diagnosis of obstructive jaundice in children.
Subject(s)
Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/therapy , Rhabdomyosarcoma, Embryonal/diagnosis , Rhabdomyosarcoma, Embryonal/therapy , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biliary Tract Surgical Procedures , Child, Preschool , Choledochal Cyst/diagnosis , Combined Modality Therapy , Common Bile Duct Neoplasms/complications , Diagnosis, Differential , Humans , Jaundice, Obstructive/etiology , Jaundice, Obstructive/surgery , Male , Radiotherapy, Adjuvant , Rhabdomyosarcoma, Embryonal/complicationsABSTRACT
PURPOSE: Bronchogenic cysts are rare congenital cystic malformations of the lung. We retrospectively analyzed ten cases of bronchogenic cyst (BC) to reinforce the importance of recognizing this malformation. METHODS: Between 1985 and 2000, ten pediatric patients with BC were treated surgically in our department. Their clinical presentation, radiological, operative, and pathological findings were analyzed retrospectively. RESULTS: There were five boys and five girls, ranging in age from 16 days to 6 years (mean 6.5 months). The clinical signs and symptoms included respiratory distress in seven patients (70%), cyanosis in four (40%), chronic cough and fever in five (50%), and dysphasia in two (20%). Routine chest X-ray revealed a pulmonary air-filled cyst in six patients (60%) and a pulmonary nodular opacity in four (40%). The diagnosis was supported by computed tomography in four patients and by ultrasonography in two. Eight of the patients were treated by cyst excision and two by lobectomy. The pathological diagnosis made from all specimens was bronchogenic cyst, with squamous metaplasia in two. CONCLUSION: In newborns, infants, and even children, the development of dyspnea, cyanosis, chronic cough, and fever should alert us to the suspicion of a cystic malformation in the lung, such as a bronchogenic cyst, especially if an air-filled cyst is seen on a plain chest X-ray.
Subject(s)
Bronchogenic Cyst/surgery , Bronchogenic Cyst/diagnostic imaging , Bronchogenic Cyst/pathology , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Retrospective Studies , Tomography, X-Ray ComputedABSTRACT
The negative effects of ischemia-reperfusion injury (IRI) on intestinal anastomotic healing have been described in previous studies. To determine the effect of pentoxifylline (PTF) on small-bowel anastomoses performed after IRI, 20 male Wistar albino rats were studied. Under ketamine anesthesia, the superior mesenteric artery was occluded for 30 min and ileal resection-anastomosis was performed after 10 min of reperfusion. In group I, no medication was given. In group II, PTF 50 mg/kg was administered intraperitoneally before reperfusion and i.m. on the 1st, 2nd, 3rd and 4th postoperative days (POD). All rats were killed and anastomotic segments were removed on the 7th POD. Anastomotic bursting pressure (ABP) and hydroxyproline (HP) content were measured in the anastomotic segments; parameters were compared statistically with Student's t-test. In group I, the mean ABP was 225.5 +/- 38.18 mmHg the and mean HP level was 16.03 +/- 1.91 micro mol/g tissue. In group II, these levels were 277 +/- 41.1 mmHg and 19.85 +/- 1.81 micro mol/g tissue, respectively. The differences between the groups were significant (P < 0.005). Thus, PTF can improve anastomotic healing in an experimental model after IRI by promotion of collagen synthesis.
