Redo operations for recurrent aneurysmal disease of the ascending aorta and transverse aortic arch

Sixty-seven operations were performed in 59 patients for aneurysmal disease occurring after previous operations involving the ascending aorta and transverse aortic arch. The initial aortic pathological condition included the following: fusiform aneurysm due to medial degenerative disease in 34 patients, 12 of whom had Marfan's syndrome; aortic dissection in a previously undilated aorta in 23; and aneurysm persisting or occurring after brachiocephalic bypass in 2. One of the latter had an aneurysm because of aortitis. Various operations initially performed did not completely treat the disease, and certain complications occurred spontaneously, including infection and dissection. The residual pathological condition led to the development of aortic insufficiency, aortic dissection, coronary artery insufficiency, and progressive aneurysmal dilatation. These complications were treated by composite valve graft replacement of the aortic valve and ascending aorta or the transverse aortic arch or both, simple aortic valve replacement, graft replacement of the ascending aorta or arch or both, and suture of false aneurysm with viable tissue wrap. Twenty patients (34%) had an aneurysm of the distal aorta. The entire aorta was replaced in 3, thoracoabdominal segments in 9, and the abdominal aorta in 1. Of the 59 patients, 49 (83%) were early survivors and 40 (68%) were alive on January 1, 1985. Principles of therapy that may have prevented the complications leading to reoperation include aneurysm replacement at the time of aortic valve replacement and coronary artery bypass; total replacement of the ascending aorta and aortic valve in patients with Marfan's syndrome; the same procedure or aortic valve replacement and separate graft replacement in patients with non-Marfan's medial degenerative disease; ascending aortic replacement in all patients with dissection combined with valve resuspension, aortic valve replacement, or composite valve graft depending on the involvement of the aortic sinuses and the presence of aortic insufficiency.     

Hirschsprung's disease

Hirschsprung's disease (HSCR) is characterized by a lack of enteric nervous system ganglion cells (aganglionosis) in a variable extent of distal bowel. It is the commonest congenital bowel motility disorder and affected neonates usually present with distal intestinal obstruction in the first few days of life. Current treatment involves resection of the aganglionic bowel and a ‘pull through’ procedure to bring the normally innervated bowel down to the anal margin. Despite advances in surgery, outcomes can be poor especially in long-segment HSCR when more bowel than the rectosigmoid is aganglionic. Children with HSCR are more prone to enterocolitis and up to 75% have problems with incontinence or constipation. Some children require a long-term colostomy. This article aims to provide an overview of Hirschsprung's disease, outlining the aetiology of HSCR, and management of children with HSCR.     

Hirschsprung's disease

Hirschsprung's disease (HSCR) is characterized by a lack of enteric nervous system ganglion cells (aganglionosis) in a variable extent of distal bowel. It is the most common congenital bowel motility disorder, and affected neonates usually present with distal intestinal obstruction in the first few days of life. Current treatment involves resection of the aganglionic bowel and a ‘pull-through’ procedure to bring the normally innervated bowel down to the anal margin. Despite advances in surgery, outcomes can be poor especially in long-segment HSCR in which a longer segment of bowel or the entire colon is aganglionic. Children are more prone to enterocolitis and up to 75% have problems with incontinence or constipation. Some children require a long-term colostomy. This article aims to provide an overview of Hirschsprung's disease, outlining the aetiology of HSCR and management of children with HSCR.     

Hirschsprung's disease

This review of Hirschsprung's disease reflects the authors' experience with it and outlines the current recommendations for management of its various manifestations.     

Redo pull-through in Hirschsprung's [corrected] disease for obstructive symptoms due to residual aganglionosis and transition zone bowel

Background

Reoperations in Hirschsprung disease may be required for residual aganglionosis or transition-zone bowel found at the distal pull-through. We aimed to review the management of patients who had this complication and offer suggestions on how to avoid it.

Methods

Ninety-three patients with Hirschsprung disease were referred to our institution with recurrent problems after a pull-through done elsewhere. All required reoperations with a variety of indications, and of these, 25 had residual aganglionosis/transition-zone histology. This was the only indication for redo in 16 children.

Results

Children (range, 2-17 years) presented 6 to 66 months after the initial pull-through. The predominant symptoms were enterocolitis (n = 9 [56%]), constipation (n = 7 [44%]), failure to thrive (n = 5 [31%]), and impaction (n = 4 [25%]). The rectal biopsy performed as part of their post pull-through work up showed hypertrophic nerves (n = 16), absent ganglion cells (n = 6), and normal ganglion cells (n = 10). The original frozen-section biopsy, determining the level of the pull-through, only sampled the seromuscular layer in 3 children, leading to misdiagnosis. Reoperations involved a transanal resection (n = 15) and a posterior sagittal approach (n = 1). In all cases, obstructive symptoms were resolved, and no patient has had recurrent enterocolitis.

Conclusion

Patients' post pull-through with recurrent obstructive symptoms may have residual aganglionosis or transition-zone bowel. Reoperation can result in the resolution of these symptoms. A full-thickness biopsy at the time of the initial pull-through to include the mucosa and submucosa may increase the possibility of identifying hypertrophic nerves.     

Hirschsprung's disease.

Hirschsprung's disease or aganglionic megacolon is an anomaly caused by the absence of ganglion cells in the myenteric plexus of the distal colon. It produces intestinal obstruction or lethal enterocolitis in the neonatal period and constipation of varying degree in the older child. The diagnosis can be made by history alone and confirmed by physical findings, barium enema, motility studies, and rectal biopsy. Colostomy may be a life saving measure in the newborn, to be followed by a definitive pull-through procedure before the age of 1 year. Operative correction consists of various techniques all of which aim for excision of the aganglionic segment with preservation of the internal anal sphincter. The major pitfall of these procedures is a too perfectly preserved sphincter which remains spastic and still produces obstruction. Some damage to this muscle must be accomplished either during the procedure or postoperatively by bouginage in order to obtain a satisfactory result.     

Adult Hirschsprung's disease

This report presents a series of 17 patients with adult Hirschsprung's disease treated at the National Taiwan University Hospital and its affiliated hospitals from 1965 to 1988. Adults with Hirschsprung's disease may present with symptoms and operative findings different from those of children with Hirschsprung's disease. The symptoms, signs, criteria for diagnosis, operative findings, and operative procedures are presented.     

Redo laparoscopic repair of benign esophageal disease

Laparoscopic fundoplication for gastroesophageal reflux disease has been associated with excellent symptom control. Compared with medical treatment, laparoscopic Nissen fundoplication has shown favorable control of typical reflux symptoms. However, in approximately 2% to 17% of patients, surgical treatment fails. The role of reoperative repair for reflux disease and the factors that contribute to it are examined.     

Hirschsprung's disease in adults

Hirschsprung's disease is the malformation of the hindgut characterised by the absence of intramural ganglion cells in the submucosal and myenteric plexuses and manifested by megacolon. Congenital character of this disease stipulates its belonging in general to the scope of activity of the pediatric clinics. Thanks to the progress of the pediatricians problems of diagnosis and treatment of Hirschsprung's disease have been successfully resolved.