[Fiberoptic laryngoscopic assisted diagnosis and operation of congenital and recurrent tracheoesophageal fistula]

Fiberoptic laryngoscopy [FOL] is usually used in the management of difficult airway intubations. Since its introduction, it has been used for few other indications. In this paper we present a new indication of fiberoptic bronchoscopy [FB] to detect the exact location of tracheoesphageal fistula [TEF] intraoperatively in fourteen cases of pediatric group patients to prevent an unnecessary traumatic manipulation during operation. In all of these cases [12 esophageal atresia and TEF and 1 recurrent TEF and 1 pure TEF] we were able to detect the exact location of TEF with using a LF type Fiberoptic laryngoscope which was been passed through the endotracheal tube. The TEF could then be found and repaired perfectly with minimal dissection and trauma to the trachea and esophagus through thoracotomy [Except 1 case that did not need thoracotomy] Postoperative course was uneventful and all of the patients were discharged in good condition. We believe that intraoperative fiberoptic bronchoscopy in these types of operations not only leads to shortening the operation time, but it can also prevent unnecessary trauma to vital thoracic organs

[Sinus histiocytosis with massive lymphadenopathy [Rosai-Dorfman disease]: a case report]

Sinus histiocytosis with massive lymphadenopathy [SHML] or Rosai-Dorfman disease [RDD] is a rare benign histiocytic disease of unknown etiology, usually associated with lymph node enlargement in various superficial or deep sites. It usually shows a prolonged clinical course with occasional exacerbation and remission phases. In this article case of RDD in a 5-year-old male with large right inguinal and bilateral cervical lymphadenopathies were, occasional fever and anemia was described. The clinical presentation, histopathologic characteristics, and treatments of RDD were discussed

[Subpubic block in superficial operations of peins]

Circumcision is the most common surgical procedure. As same as other superficial operations, they cause not only severe postoperative pain but also are accounted a severe intraoperative stimulation. There are many anesthetic techniques for circumcision. It seems supplementary penile block to general anesthesia is the best approach. Our aim in this study is to evaluate the efficacy of sub pubic approach to dorsal penile nerve block in relieving postoperative pain. This descriptive study was done in Dr Sheikh Pediatric Hospital during summer of 2004. Sub pubic approach to dorsal penile nerve block was evaluated in 60 boys. Two injections were performed with plain Bupivacaine 0.5%, 0.1 ml/kg in each side [total 0.2 ml/kg] at puncture sites, which had been marked before, just 0.5 to 1 cm blow pubic symphisis and lateral to midline in the supine position. Postoperative pain was evaluated with two methods, FLACC and Wong and Baker, and parental report up to 24 hours after general anesthesia. Collected data was analyzed by using descriptive and analytical statistics. Two cases [3.3%] were excluded. Two patients [3.4%] experienced moderate pain postoperatively. Others [96.5%] were completely pain free up to 24 hours after procedure. No complications occurred. There was no relationship between needle length and patients' age. According to this and previous studies, although there is little experience, this technique is the most successful and safe approach for intra and postoperative long [up to 24h] pain relief that with some experience is also very easy

Congenital pouch colon with imperforate anus; a report of 3 cases

Congenital pouch colon [CPC] is an unusal condition in imperforate anus [IA] usually of high variety this malfotmation is mostly reported from India, where it comprises about 7% of all anorectal malformations. In this anomaly the normal colon is very short or absent and is replaced by a pouch like dilatation that usually communicates through a fistula with genitourinary tract or persistent cloaca. We are reporting on three cases of newborns [2 females, and 1 male] with this anomaly diagnosed during September 2001 to September 2004 These three cases comprise less than 3% of all cases of IA during this time period. The two girls had anorectal agenesia, short segment of colon and a pouch with vaginal fistula and persistent cloaca [Type II]. Colon in the third case was replaced by a dilated pouch that communicated with a wide fistula to the bladder [Type I]. This is very important for pediatric surgeons to be aware of the features of this condition to allow for proper diagnosis and surgical management

Wolfram Syndrome: report of five cases and review of literature

To report five cases of Wolfram syndrome, an autosomal recessive neurodegenerative disease with Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness[DIDMOAD syndrome]. All of the five patients had diabetes mellitus and optic atrophy. Four patients had hearing loss. In spite of persistence of polyuria and polydipsia, diabetes insipidus had been previously detected in only one patient and its presence was discovered after the diagnosis of the syndrome in the other three. Two brothers had open angle glaucoma. Although Wolfram syndrome is rare, it should be considered in all insulin-dependent diabetics in whom polyuria and polydipsia persists despite adequate control of blood sugar. It should also be considered in familial cases of diabetes mellitus type I. Presence of optic atrophy is a helpful sign for diagnosis. Fundoscopy is crucial for early diagnosis. With early management of diabetes insipidus, late urologic complications may be prevented. Quality of life may be improved with visual and hearing aids

[ comparison of tubeless repair of esophageal atresia with conventional techniques]

Introduction: Previously, management following the repair of esophageal atresia with tracheoesophageal fistula [TEF] included the routine use of an intercostal chest draining tube [CT], a gastrostomy or a transanastomotic tube [TAT] for enteral nutrition and a routine contrast swallow [CS] before oral feeds. There has been a trend toward simplification of the management, but not universally

Methods and Materials: The aim of this prospective study was to evaluate the safety of a simplified management regimen in neonates undergoing primary repair of esophageal atresia over a 3.5-years period from January 2000 till July 2003 in Pediatric Surgery Unit of Sheikh Pediatric Hospital. Recorded parameters included demographic details, time of first enteral feeding and early complications. Thirty-six patients were studied in three groups. Group 1 included seven patients without TAT or CT. Fourteen patients of group 2 had TAT, but no CT. Group 3 patients had both TAT and CT. Sex distribution, gestational age and birth weight were comparable in all groups

Result: There was no significant difference in survival rate between groups with and without CT

Conclusion: The majority of infants with esophageal atresia and TEF can be safety managed without routine chest drainage or contrast study. Early introduction of oral feeding in the Non-TAT group is not associated with an increased complication rate