Ultrastructural Changes of the Smooth Muscle in Esophageal Atresia.

Esophageal atresia (EA) with or without tracheo-esophageal fistula (TEF) is a relatively rare congenital anomaly. Despite the advances in the management techniques and neonatal intensive care, esophageal dysmotility remains a very common problem following EA/TEF repair. Our current study aimed to describe the most significant ultrastructural changes of the smooth muscle cells (SMCs) trying to highlight some of the underlying mechanisms of esophageal dysmotility following EA/TEF repair. Twenty-three biopsies were obtained from the tip of the lower esophageal pouch (LEP) of 23 patients during primary repair of EA/TEF. Light microscopic examination was performed with hematoxylin and eosin (HE), and Van Gieson's stains. Ultrastructural examination was done using transmission electron microscopy (TEM). Histopathological examination showed distortion of smooth muscle layer and deposition of an abundant amount of fibrous tissue in-between smooth muscles. Using TEM, SMCs exhibited loss of the cell-to-cell adhesion, mitochondrial vacuolation, formation of myelin figures, and apoptotic fragmentation. There were also plasmalemmal projections and formation of ghost bodies. Interestingly, SMCs were found extending pseudopodia-like projections around adjacent collagen fibers. Engulfed collagen fibers by SMCs underwent degradation within autophagic vacuoles. Degeneration of SMCs and deposition of abundant extracellular collagen fibers are prominent pathological changes in LEP of EA/TEF. These changes might contribute to the pathogenesis of esophageal dysmotility in patients who have survived EA/TEF.

Drainage of pancreatic pseudocysts. The importance of cyst wall biopsy in the recent era.

OBJECTIVE: While open internal drainage has been the standard treatment for pancreatic pseudocysts, less invasive techniques which pay little attention to cyst wall biopsy, are becoming popular. The aim of this study is to report on our experience in draining pancreatic pseudocysts and probe the necessity or otherwise of obtaining a wall biopsy at drainage. METHODS: Operation theatre registry, operation log books and medical records at Aseer Central Hospital, Abha, Kingdom of Saudi Arabia, were reviewed to retrieve the clinical details of patients with pancreatic pseudocyst who required a drainage procedure in a 13 years period from August 1989 to November 2002. RESULTS: Sixteen patients were identified. Cyst wall biopsy was obtained in 10 cases, in 8 of them the diagnosis was confirmed, while a true cyst was found in the remaining 2 excluding them from further analysis. In the remaining 14 cases (8 males, 6 females, mean age 38 years, range 4-60), pain was the main presenting feature. Open internal drainage was offered to 12 cases while one patient received external drainage under ultrasound guidance and the other received open external drainage. The type of operation was cystogastrostomy in 9 patients and cystojejunostomy in 3 patients. The recurrence rate after internal drainage was 16.7%, while after external drainage was 100%. There was no mortality in this series. A procedure-related complication occurred in 3 (21.4%) patients. CONCLUSION: The mortality, morbidity and recurrence rates in this series are compared favorably with other reports. The final diagnosis of a presumed pancreatic pseudocyst should rest on the histopathologic examination of the cyst wall.

Esophageal atresia with tracheoesophageal fistula and early postoperative mortality.

BACKGROUND: Several recent reports showed that associated anomalies represent the main cause of postoperative mortality in infants born with esophageal atresia (EA) and/ or tracheoesophageal fistula (TEF) Our observations present additional causes of mortality to the above mentioned. The aim of this study is to identify the major causes of early postoperative mortality in cases of EA and/or TEF in our setup. The ongoing preoperative classifications predicting mortality will be also used for comparison. PATIENTS AND METHODS: We reviewed 101 charts of all cases with EA and/or TEF in a period of 11 years from 1990 to 2000. Morbidity and causes of postoperative mortality during the first admissions were identified. The factors predicting mortality were documented. Patients were classified according to Waterston, Montreal and Spitz classifications. RESULTS: Thirty-one patients (30.7%) died. Two main groups of post operative mortality were identified. The first group included the possibly avoidable causes of mortality which were primary sepsis (n=10, 32.3%), technical problems (n=8, 25.8%) and severe pneumonia (n=5, 16.1%). The unavoidable causes of mortality included major congenital anomalies (n=6, 19.3%) and anomalies incompatible with life (n=2, 6.5%). CONCLUSION: Primary sepsis and sepsis due to technical problems were the main causes of mortality in our series. Factors predicting mortality were pneumonia at presentation, sepsis at presentation or that acquired during hospitalization, major or life threatening anomalies, long gaps and major leaks. The Waterston classification was statistically the best applicable in this study.

Toe tourniquet syndrome.

Toe tourniquet syndrome refers to external, mechanical, circumferential constriction of the toes. We report a series of 4 infants with toe tourniquet syndrome from Saudi Arabia who presented during wintertime with very similar symptoms (approximately 48 hours of inconsolable crying and irritability), similar involved region (toes), and similar constricting agent (hairs). Immediate removal of the hair fibers was carried out in all patients, fortunately followed by fast healing with no signs of tissue necrosis. The prompt diagnosis and treatment of the condition were vital in attaining the good outcome and preventing ischemic complications. 

Medical and surgical management of fecal incontinence after repair of high imperforate anus anomalies.

OBJECTIVE: To report the medical and surgical management of fecal incontinence in children after repair of high imperforate anus. METHODS: Thirty-seven children with fecal incontinence post repair of high imperforate anus were recruited between January 2000 and July 2007 at Aseer General Hospital, Abha, and Alhada Military Hospital, Taif, Kingdom of Saudi Arabia. The decision for surgery (dynamic graciloplasty) was based on the degree of incontinence and failure to respond to medical treatment. Five children were operated from the start according to the input of history, examination, and investigations (incontinence score of 15-20). The remaining 32 patients were treated medically. Those cases that failed to respond to medical treatment had alternatively biofeedback therapy. Cases that failed to respond to biofeedback therapy underwent surgery. RESULTS: Response to medical treatment is favorable (71%). Biofeedback showed discouraging results (22%). Dynamic graciloplasty showed excellent outcome (100% improvement in continence on long term follow up). CONCLUSION: Based on history, examination, and investigations, recruited cases were primarily treated medically; those who had failed the medical treatment approach had dynamic graciloplasty, which demonstrated excellent results during the 1-7 years follow-up period.

Congenital esophageal stenosis associated with esophageal atresia: new concepts.

Congenital esophageal stenosis (CES) is suspected by a fixed intrinsic narrowing of the esophagus that affects the normal swallowing mechanism. The diagnosis is only confirmed by histopathologic picture, which may show fibromuscular disease (FMD) or tracheobronchial remnants (TBR). The latter involves ciliated pseudo stratified columnar epithelium, seromucous glands or cartilage each alone or in combination. The aim of this study is to document the usefulness of histologic picture of surgical specimens obtained from the lower esophageal pouch (LEP) during primary repair in detecting cases of CES associated with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF). Over an 8-year period, 57 consecutive cases operated upon for EA with or without TEF were subjected for histologic examination of surgical specimens obtained from the tip of the LEP. Cases that histologically showed FMD or TBR were included. The usefulness of this histologic picture as a diagnostic and therapeutic aid is assessed. Methods of treatment and outcome were also reviewed. Eight patients out of 57 (14%) had a histologic picture suggestive of CES, two with FMD, four with TBR without cartilage and two with cartilage. Out of 57 patients, 23 developed strictures, six of them had positive biopsies suggestive of CES. One patient with TBR without cartilage did not have stricture. Another case of pure atresia had LEP resection and gastric pull up showed cartilage involving the whole lower esophagus. Excluding the case of pure EA with gastric pull up, all patients suffered from feeding problems and recurrent aspiration. Fluoroscopic barium studies showed late onset minor dysmotility in five patients and late onset major dysmotility in two. All cases studied showed significant gastro-esophageal reflux (GER). Stricture was seen at the anastomotic site extending distally in the two fibromuscular cases and one case with cartilage, at the anastomotic site in three cases with TBR without cartilage. Anti reflux surgical procedures were performed in four patients without benefit in two patients with major dysmotility. Dilatation was successful in the three patients with TBR without cartilage. One patient with cartilage had resection of the anastomotic site and required frequent dilatations and is now doing well. A case of FMD did not improve after frequent dilatations and myotomy together with Nissen's fundoplication and required resection while the other case of FMD responded partially to dilatations. Cartilage in cases of CES requires surgical resection. Those with TBR without cartilage may not develop stricture. If stricture develops, it responds well to dilatation and patients have good clinical outcomes. Unlike isolated CES, GER is a significant feature in CES with EA. Anti reflux procedures should be avoided before definitive surgery for the stricture and if necessary a partial wrap with gastrostomy is recommended. CES should be considered in the etiology of anastomotic stricture. Taking a surgical specimen routinely from the tip of the LEP during primary esophageal repair for histologic studies is highly recommended.