Congenital Esophageal Stenosis: To dilate or To resect

Congenital esophageal stenosis [CES] is a rare condition in children. The definitive preoperative diagnosis often is difficult to make, and a standard therapeutic protocol remains controversial. This study was carried out to determine the proper management strategy in children suffering from CES. The medical records of 17 patients with CES treated during a period of10 years [from 1995 to 2005] were retrospectively reviewed. Each patient was evaluated as regard to the clinical presentation, pathology, management, and outcome. The ages of patients at time of diagnosis ranged from 3 months to 9 years. The sites of stenosis were located more frequently at the lower third of the esophagus [n=10] than the upper third [n-3] and middle third [n=4], The diagnosis was made by esophagogram, esophagoscopy and was confirmed by histopathologic examination. Fifteen patients were diagnosed primarily, while 2 patients were diagnosed after unsuccessful surgical treatment for an initial misdiagnosed achalasia of the cardia. Six patients had confirmed tracheobronchial remnants [TBR], five had fibromuscular stenosis [FMS] and 2 had membranous web stenosis [MS]. The histopathology was unknown in 4 patients due to inadequate biopsies taken during esophagoscopy. All patients were treated initially by repeated esophageal dilatations [2-8 times] over two to thirty month period. The dilatation alone was successful in 11 patients, but was complicated by esophageal perforation in one case. Six patients required surgery; five of them were treated by resection and anastomosis, and one required esophageal replacement The pathology of this later group was TBR in 5 patients and FMS in one. 1. this study emphasizes the diagnostic difficulties in some children with CES; 2. Esophageal dilatation may be tried initially 3. Resection should be reserved for cases not responding to repeated dilatation particularly those with proven TBR

One stage repair of severe hypospadias: Original versus modified koyanagi technique

Although the original Koyanagi technique seemed applicable for the repair of severe hypospadias at or proximal to the penoscrotal junction, its use has resulted in a high complication rate. The technique was modified to ensure better vascularity of the flaps. The purpose of this study is to report the results of original Koyanagi technique and its modifications in patients with severe hypospadias. The original Koyanagi parameatal prepuital flap technique was performed in 16 boys with severe hypospadias [group I]. In a subsequent 11 patients, the modified Koyanagi technique was used to preserve blood supply to the flaps [group II] The meatus was located at or proximal to the penoscrotal junction in all patients. Moderate to severe degrees of chordee was noted in all of them. The median age of patients was 13 and 20 months at time of repair for group I and II respectively. Each patient was evaluated as regard to site of the new meatus, straightness of the phallus, and stream of urine, development of fistula, urethral or meatal stenosis, any other complication, and the need for another operation. Follow up ranged from 36 to 84 months in group I, and 3 to 36 months in group II. A fistula developed in 8 of the 27 patients [29.6%] The frequency of fistula was more common in group I [6116] than in group II [2/11]. Urethral diverticulum occurred in one patient in group II, and meatal stenosis in 2 in group I. Significant infection resulted in a regressed meatal position in 2 [one in each group]. Good cosmetic results were achieved in all except the latter 2 cases. Secondary operations were needed in 8 patients [6 in group I and 2 in group 2] The indication for secondary surgery were closure of persisted urethrocutaneous fistula in 6 patients [5 in group 1 and 1 in group 2] and regressed meatus in 2 [one in each group]. A single staged repair can be safely and effectively performed even in patients with the most severe penoscrotal hypospadias. The modified Koyanagi technique has relatively lower complication rate than original Koyanagi repair. The complication rate is acceptable considering the severity of these cases

Delayed presentation of congenital diaphragmatic hernia

The late-presenting congenital diaphragmatic hernia [CDH] represents a considerable diagnostic challenge. This study was undertaken to define various patterns of delayed presentations, and to analyze the pitfalls in the diagnosis and treatment of these patients. Thirty-three children with CDH were treated between 1993 and 2000; fifteen of these children [45.5%] were diagnosed after age of 2 months form the basis of this report. Each child was evaluated as to age. sex, side of herniation, presenting symptoms, physical and roentgenographic findings, management and outcome. Thirteen patients had Bochdalek hernia, and 2 had Morgagni hernia. The diaphragmatic defect was right- sided in 6 cases, and left sided in 9. The ages ranged from 2 months to 14 years with a median of 2,5 years. Five patients presented acutely, three with respiratory distress, and two with gastrointestinal [GI] obstruction. The remaining 10 patients presented with chronic respiratory or GI complaints. Inappropriate insertion of chest drain occurred in 3 patients misdiagnosed as having pleural effusion [2 cases], and pneumothorax [one patient]. Two patients had previously reported normal chest radiographs. Plain radiographs were sufficient to make a definitive diagnosis in only 6 patients, but GI contrast studies were necessary for confirmation of the diagnosis in the other 9 patients. All patients were treated through abdominal approach with primarily closure of the diaphragmatic defects without patch. A distinct hernial sac was present in 6 cases, and associated malrotation in 6 patients. All patients except one survived the operation with rapid improvement of their GI and respiratory symptoms. Late-presenting CDH should be included in the differential diagnosis of any child with persistent GI or respiratory problems associated with abnormal chest x-ray film. Nasogastric tube placement must be considered as an early diagnostic or therapeutic intervention when the diagnosis is suspected, and GI contrast studies should be part of the diagnostic work-up of these cases

Mesenteric and retroperitoneal cysts in children

Mesenteric and retroperitoneal cysts are rare intraabdominal lesions of childhood. The purpose of this study was to review our experience with these lesions in order to more clearly define their clinical presentation, diagnosis, and treatment. Eight children were diagnosed and treated for mesenteric cysts [5 patients] and retroperitoneal cystic lymphangiomas [3 patients] The medical records of each child was reviewed as regard to age, sex, clinical presentation, duration of symptoms prior to diagnosis, imaging studies, operative findings, histological features, and outcome. There were 5 boys and 3 girls, their ages ranged from 2 months to 16 years [average age, 40.2 months]. Three patients required emergency surgery and five underwent elective intervention. The main presenting symptoms were abdominal pain and mass. Preoperative ultrasounds were diagnostic for a cystic mass in all patients. The cysts were located in the small bowel mesentery in 3 cases, the base of the mesentery with retroperitoneal extension in 3 cases, and the transverse mesocolon in 2 cases. Operative procedures performed included enucleation of the cyst [4 patients], complete excision with intestinal resection [3 patients], and partial excision, colonic resection-anastomosis and marsupialization in one infant with multiple cysts. No recurrence occurred in this series. Accurate preoperative diagnosis is possible with maintaining a high index of suspicion and the use of ultrasonic imaging and computed tomography [CT]. Retroperitoneal cysts should be considered a different entity from mesenteric cysts even though they present clinically in a similar fashion. Complete cyst resection is the procedure of choice and results in an excellent outcome

Study of the role of serum sTNFR-I and sIL-6 in children with acute respiratory failure in intensive care unit

Structural cells as well as immune effector cells of the lung are capable of cytokine production and release. Some of cytokines receiving the most attention to date in relation to pulmonary diseases include [TNF-alpha] and [IL-6], but there are no available reports with respect to the role of soluble tumor necrosis factor receptor-I [sTNFR-I] and soluble interlukin-6 receptor [sIL-6R] in lung diseases especially acute respiratory failure [ARF]. This work was done to assess the role of serum levels of sTNFR I and sIL-6R in critically ill children with acute respiratory failure [ARF] in ICU. The study included 18 patients [11 males and 7 females, aged 3 days-14 years] classified into 8 patients with mild [ARF], 8 patients with moderate [ARF] and 2 patients with severe [ARF]; in addition to, 13 healthy subjects of matched age and sex as controls. They were subjected to thorough history and clinical evaluation, CBC, CRP, ESR, blood gases, pulmonary function test, assessment of Pediatric Index of Mortality [PIM score] and cytokine assay serum [sTNFR-I and sIL-6R] by ELISA technique, Statistical analysis of our results revealed the following: [1] Highly significant increase in serum levels of sTNFR-I [p<0.0001], but insignificant increase in serum levels of sIL-6R [p<0.0001], in acute respiratory failure patients compared to controls; [2] Significant positive correlation between serum levels of sTNFR-I and severity of acute respiratory failure [r = 0.56, p = 0.02] and PIM system [r = 0.49, p = 0.04] While; insignificant correlation between serum levels of sIL-6R and severity of acute respiratory failure [r = 0.04, p = 0.88] and PIM system [r = 0.01, p = 0.96] sTNFR-I may be involved in the pathogenesis of acute respiratory failure. sTNFR may have significant prognostic value in ICU patients with acute respiratory failure since higher serum levels of sTNFR-I may be related to severity and mortality of patients with acute respiratory failure

Bilateral buccinator mucomuscular flaps with double opposiong Z-plasty for repair of wide and/or complete cleft palate

Furlow procedure remains our procedure of choice for narrow and incomplete clefts. In wide clefts of the palate there is no enough tissue present to allow closure of the cleft without side-to-side tightness as to impede drastically palatal junction. In this study we have combined the Furlow technique with bilateral buccinator mucomuscular flaps. We repaired 12 wide and/or complete clefts by this technique. Only one postalveolar fistula was reported. Further follow-up is going to evaluate the long-term effects of this technique on speech and maxillary growth

Bilateral buccinator mucomuscular flaps with double opposing z-plasty for repair of wide and/or complete cleft palate

Furlow procedure remains our procedure of choice for narrow and incomplete clefts. In wide clefts of the palate there is no enough tissue present to allow closure of the cleft without side-to-side tightness as to impede drastically palatal function. In this study we have combined the Furlow technique with bilateral buccinator mucomuscular flaps. We repaired 12 wide and/or complete clefts by this technique. Only one postalveolar fistula was reported. Further follow-up is going on to evaluate the long term effects of this technique on speech and maxillary growth