Atretic Segment Research Articles

Left Aortic Arch and Right Descending Aorta -Interruption or Severe Coarctation of the Aortic Arch in the Newborn

Three consecutive neonates (aged 7, 14 and 30 days, body-weight 2980 g, 3000 g and 3400 g respectively) with interruption of the aorta ( n = 2) and severe coarctation ( n = 1) in the presence of left aortic arch and right descending aorta are reported. Associated lesions were an aortopulmonary window in the first case and an unrestrictive ventricular septal defect in the two others. Intractable heart failure and the complexity of the malformation led to the decision of a staged operation. A prosthetic graft was interposed between the ascending and descending aorta via a right thoracotomy in order to bridge the atretic or hypoplastic segment without using extracorporeal circulation. There was no intraoperative complication. One patient developed a thrombocytopenia within the frame of a sepsis syndrome and died on day 5 after operation, death being caused by a massive bleeding into the left thoracic cavity, although the operation was carried out via a right thoracotomy. Angiography 1 year after operation revealed a good flow through the grafts and no stenosis at the site of the anastomoses. The two surviving infants are clinically well without any medication. The reported operative technique provides an alternative palliative possibility to manage critically ill neonates without any obstacle to later definitive repair.

Left aortic arch and right descending aorta — interruption or severe coarctation of the aortic arch in the newborn

Three consecutive neonates (aged 7, 14 and 30 days, body-weight 2980g, 3000g and 3400g respectively) with interruption of the aorta ( n = 2) and severe coarctation ( n = 1) in the presence of left aortic arch and right descending aorta are reported. Associated lesions were an aortopulmonary window in the first case and an unrestrictive ventricular septal defect in the two others. Intractable heart failure and the complexity of the malformation led to the decision of a staged operation. A prosthetic graft was interposed between the ascending and descending aorta via a right thoracotomy in order to bridge the atretic or hypoplastic segment without using extracorporeal circulation. There was no intraoperative complication. One patient developed a thrombocytopenia within the frame of a sepsis syndrome and died on day 5 after operation, death being caused by a massive bleeding into the left thoracic cavity, although the operation was carried out via a right thoracotomy. Angiography 1 year after operation revealed a good flow through the grafts and no stenosis at the site of the anastomoses. The two surviving infants are clinically well without any medication. The reported operative technique provides an alternative palliative possibility to manage critically ill neonates without any obstacle to later definitive repair.

Pathogenesis of congenital cystic adenomatoid malformation of the lung.

Congenital cystic adenomatoid malformation is a rare developmental abnormality of the lung. In most earlier reported cases, the anatomy of the bronchial tree was poorly documented. We describe four cases studied following autopsy. Post-mortem bronchography or serial microscopical examination showed segmental bronchial absence or atresia in each of them. Our observations provide further evidence pointing to bronchial atresia as being the primary defect leading to the development of congenital cystic adenomatoid malformation. The morphology of the lesion, i.e. the type of malformation, is determined by the extent of dysplastic lung growth beyond the atretic segment. The aetiology of the bronchial atresia is probably heterogeneous and may either represent a primary malformation, or be the result of vascular disruption.

The computed tomographic appearances in pulmonary artery atresia

Isolated pulmonary artery atresia is an uncommon congenital abnormality. Plain radiography and ventilation/perfusion lung scintigraphy may suggest the diagnosis but pulmonary angiography has traditionally been recommended for confirmation. The computed tomographic (CT) appearances of four patients with isolated atresia of a pulmonary artery are described. In each case the atretic segment of the abnormal vessel could be identified. Other CT findings included reduction of volume of the involved hemithorax, increased density of the affected lung, a small hilum, and an abnormal pulmonary vascular pattern thought to be due to the collateral systemic arterial supply to the intrapulmonary branches of the atretic pulmonary artery. The diagnosis of isolated pulmonary artery atresia can be made using dynamic, contrast enhanced CT without recourse to more invasive procedures.

New concepts in preoperative imaging of anorectal malformation. New concepts in imaging of ARM.

In this study of 14 patients with anorectal anomalies CT and MRI were employed for preoperative assessment. The use of a pressure enhanced water soluble enema via the colostomy proved to be an extremely efficient method for showing a fistula. MRI studies were enhanced by the use of vaseline oil and in one case this technique was used prior to surgery to provide important information by injecting through a perineal fistula. CT and axial MRI proved to be more valuable than sagittal MRI which is only useful for the length of the atretic segment. The authors consider that a combined approach using pressure enhanced water soluble enema and MRI will provide the most valuable preoperative information to plan a successful operative approach and enable an accurate prognostic evaluation of continence in these difficult and complex patients.

Meconium per vaginam secondary to ileal atresia and meconium peritonitis

A neonate was referred for an abdominal mass and passing meconium per vaginam. The anorectum was normal. Roentgenograms revealed a small-bowel obstruction and hypogastric peritoneal calcifications. Findings at celiotomy were: ileal atresia, meconium peritonitis, and two meconium-filled pseudocysts draining through the female genital tract. Excision of the inflammatory masses, resection of the atretic segment, and primary ileoileostomy were followed by an uneventful recovery. Only two similar cases, both fatal, are reported in the English literature. Meconium peritonitis should be considered in the neonate passing meconium per vaginam.

Anomalous biliary ducts associated with duodenal atresia

Duodenal atresia is rarely associated with anomalous biliary ducts that permit communication between the proximal and distal duodenal segments. Two such cases are presented herein and the literature is reviewed. Although the typical radiographic pattern of duodenal atresia is the “double bubble” sign with absence of distal bowel gas, air may be present in the distal bowel when anomalous bile ducts provide a conduit around the atretic segment. Contrast studies are generally not performed in the typical clinical and radiographic evaluation of duodenal atresia; however, an upper gastrointestinal series may be useful in defining the more complex anomaly. Clinical presentation may occur relatively late if the biliary communication is large enough to permit passage of some milk or formula. Care should be taken at surgery to avoid obstruction or injury to the anomalous bile ducts, and operative cholangiography may be useful to document continued bile duct patency following repair of the atresia. Theories of the etiology of this anomaly relate to interference with recanalization of the duodenum by the process of hepaticopancreatic duct formation and persistence of the primitive “dual duct” stage.

Ultrasonographic display of complex vascular rings

Ultrasonographic display of vascular ring anatomy has been limited to single-plane views. This does not readily allow for a three-dimensional interpretation of structural relations. A method that included a sweep consisting of multiple contiguous frontal planes was used in 12 patients with a vascular ring before repair for the evaluation of the arch sidedness and number, brachiocephalic vessel pattern, upper descending thoracic aorta sidedness, ductus arteriosus site or sites and proximal pulmonary arteries; 7 patients had Doppler color flow imaging.Complete imaging of the luminal vascular components was possible in all but one patient. Its four other patients, atretic segments of the vascular ring could not be displayed. The addition of Doppler color flow imaging especially aided in the tracing of multiple vascular structures in complex cases and in assessing ductus arteriosus and arch patency. The use of a suprasternal frontal sweep with posterior angulation could display encirclement of the air-filled trachea. Vascular ring segments without (lumens could not be displayed.

Rectal atresia: Transanal, end-to-end, rectorectal anastomosis: A simplified, rational approach to management

Transanal, end-to-end, rectorectal anastomosis (TERA) is a new technique for the surgical correction of rectal atresia. Hitherto performed abdominoperineal or sacroperineal procedures entailed major traumatizing surgery with an inherent risk of complications. The rationale of TERA is based on three factors: (1) the anorectal canal distal to the atresia is normally developed, as are the sphincteric muscles surrounding it; (2) the anorectum can be preoperatively dilated to allow a transanal anastomosis of good size; and (3) the atretic segment can be effectively “intussuscepted” into the anal canal, almost up to the anal verge, by an oversized metal bougie passed through the sigmoid colostomy. A midline sagittal incision over the metal bougie exposes the rectal pouch, which is mobilized from the surrounding muscle fibers, and a direct, end-to-end anastomosis is performed. This technique has been successfully used in two cases of rectal atresia having a gap between the two pouches.

New observations on the pathogenesis of multiple intestinal atresias

It has been suggested that multiple intestinal atresias result from multiple ischemic infarctions of the intestinal tract. We have studied surgical material from 59 neonates with intestinal atresias seen at our hospital between 1975 and 1986. Forty (68%) patients had single intestinal atresias and 19 (32%) had multiple atresias. There were seven cases of hereditary multiple atresias seen in three families and 12 cases of nonhereditary multiple atresias. All hereditary cases had numerous type I or type II gastrointestinal atresias but none had type IIIa atresia. Six of the seven hereditary cases had multiple atresias in the small as well as large bowel. The 12 patients with nonhereditary atresias had various types of atresias but mesenteric or intestinal interruption was observed in only two patients. All patients with hereditary multiple intestinal atresias showed identical microscopic appearances in the small and large intestine, consisting of sieve-like multiple lumina, each surrounded by its own mucosa and muscularis mucosae but sharing a common muscle coat. There was no evidence of lanugo, bile pigments, or squames within the lumen distal to atretic segments in any of these patients. Six nonhereditary cases who had multiple septal atresias affecting only the small bowel demonstrated essentially similar lesions on microscopic examination as seen in hereditary cases. There was no evidence of arterial occlusion in the mesentery and lanugo, bile pigments, and squames could not be found distally in the intestinal contents in any of these cases. These pathologic findings suggest that all cases of hereditary multiple intestinal atresias and some cases of nonhereditary multiple intestinal atresias are a consequence of a malformative process of the gastrointestinal tract rather than an ischemic process.

Hemidesmosome deficiency of gastro-intestinal mucosa, demonstrated in a child with Herlitz syndrome and pyloric atresia

In an 11-day-old premature girl with Epidermolysis bullosa atrophicans generalisata gravis Herlitz and pyloric atresia, hypoplasia of hemidesmosomes in the skin, the gastrointestinal mucosa and the atretic pyloric segment was found by electron microscopy. Pyloric atresia is explained by hemidesmosomal defects causing junctional blistering of the mucosa, subsequent peptic digestion and inflammatory scarring reaction.

ESOPHAGEAL ATRESIA WITH TRACHEO-ESOPHAGEAL FISTULA—A REVIEW OF CRITICAL CARE, ANESTHETIC AND SURGICAL MANAGEMENT

We reviewed the critical and perioperative management of 57 newborns and infants with esophageal atresia (EA) and tracheoesophageal fistula (TEF) during the last 16 years (1970-1985). The common anomaly of proximal EA with distal TEF was present in 54; 3 had the less common H type fisutla. The major preoperative problem was pneumonia ± atelectasis. There was no relationship between intubation technique and subsequent ventilatory problems. Even though serious ventilatory problems after an initial gastrostomy have been reported, no such problem was found in this review. Invasive monitoring (arterial ± central venous lines) was used more often in recent years. When compared to previous reports the overall one-year mortality was significantly better for babies weighing ≤ 2.5 kgs (< 2.0 kgs = 50%; 2-2.5 kgs = 87%). Survival for babies > 2.5 kgs continues to be good (97%). Improved survival maybe related to better monitoring, improved surgical technique, cardiac and nutritional support. Prophylactic esophageal dilation of the repaired atretic segment was done routinely and may have decreased the incidence of chronic aspiration and mortality. However, there was a difference in mortality and morbidity between primary and staged repairs. With the latter, the mortality and morbidity was 8.9% and 89% respectively. With primary repair only 3.6% and 57%. The patients who had staged repair were of younger gestational age, weighed less and had a higher incidence of preoperative pulmonary problems.

Pressure-induced growth (PIG) of atretic esophagus: a contigent management for high-risk esophageal atresia.

A method is described by which a balloon catheter is inserted into the lower pouch, avoiding aspiration pneumonia due to overflow of gastric contents via a lower tracheoesophageal fistula in high-risk cases of esophageal atresia. As a side-effect there is an enlargement of the lower pouch, termed by the authors "pressure-induced growth". The prevention of aspiration pneumonia, induction of growth of the atretic segment, and the application of this method as the first part of a staged management of esophageal atresia in high-risk cases are the advantages.

Bronchial Atresia Associated with a Bronchogenic Cyst: Evidence of Early Appearance of Atretic Segments

We report a case of congenital lobar emphysema in an adult due to bronchial atresia and presenting characteristically with a solitary pulmonary nodule due to a mucous plug. In the same patient the presence of a bronchogenic cyst leads to a hypothesis that the atretic segment is the result of an insult occurring during the 5th or 6th week of intrauterine life rather than one occurring after airway development is complete.

2 cases of pyloric atresia

Two cases of pyloric atresia are presented. In the first case an isoperistaltic gastroenterostomia was performed with an atretic long segment. In the second child, pyloroplasty according to Finney was performed. The different possibilities of treatment are discussed.

An unusual variant of a jejunoileal atresia

This is a report of a patient with an unusual variant of intestinal atresia associated with third trimester bleeding that required an extensive small bowel resection. The patient had a 40 cm segment of atretic intestine removed, and continues to do well at age 11 mo despite a short-bowel syndrome and lack of ileocecal valve. No lumen was present in the atretic segment, but numerous foamy histiocytes were present. She weighs 8.1 kg, and is receiving a combination of parenteral nutrition and oral feedings.

Two-dimensional echocardiographic study of right ventricular outflow and great artery anatomy in pulmonary atresia with ventricular septal defects and in truncus arteriosus

In this study, we reviewed M-mode and two-dimensional (2DE) echocardiographic observations in 13 patients with pulmonary atresia with ventricular septal defect and in six patients with truncus arteriosus in order to attempt to identify echocardiographic features distinguishing these two abnormalities in which no anatomic connection exists between the right ventricle and the pulmonary artery. M-mode features compatible with the diagnosis of pulmonary atresia with a ventricular septal defect (VSD) were a small but identifiable space anterior to the aorta and/or immobile pulmonic valve echoes appearing to open during diastole rather than systole. By 2DE, the proximal and distal segments of the right ventricular outflow tract could be imaged and the length of the atretic segment estimated. In truncus arteriosus, no outflow tract of the right ventricle could be identified by 2DE or M-mode echocardiography, and the origin of the pulmonary artery from the truncus could be imaged directly in four patients with type I and in one patient with type II truncus. Abnormalities of the truncal valve were also present and were imaged by 2DE in three of our five patients. Our study identified specific echocardiographic criteria for diagnosing truncus arteriosus and pulmonary atresia with VSD and for differentiation between them.

Pp. xvi+320. Price SFr. 198J. Piiper and P. Scheid, Progress in Respiration Research, 16. Gas Exchange and Function of Normal and Diseased Lungs, Karger, London (1981).

Congenital bronchial atresia (CBA) is a rare anomaly that results from a congenital focal obliteration of a proximal segmental or subsegmental bronchus with the normal development of distal structures. The short atretic segment leads to accumulation of mucus within the distal bronchi to form a bronchocoele and underventilation of the affected part of the lung. The clinical and radiographic features of three cases of CBA are reported and the literature is reviewed. Radiologically, CBA is characterized by the presence of a branching opacity, the bronchocoele, radiating from the hilum surrounded by an area of hyperlucency. These features can be recognized on the chest radiograph but are more clearly defined by computed tomography. Fibreoptic bronchoscopy (FOB) is often required to exclude acquired proximal bronchial obstruction by tumour, foreign body or inflammatory stricture. In CBA a blind-ending bronchus may be revealed, although FOB is often normal. Bronchographic examination is generally abnormal but the appearances may be confusing and is rarely performed. Pathologically, CBA is characterized by a blind-ending bronchus associated with dilated distal bronchi containing impacted mucus. The surrounding lung parenchyma is hyperinflated due to collateral air drift. CBA can be identified accurately by imaging techniques which may make surgery unnecessary in asymptomatic cases.

Double aortic arch anomalies: diagnosis by countercurrent right brachial arteriography.

The clinical, radiologic, angiographic, and operative findings in 15 patients with double aortic arch are described. In five patients, both arches were functional; in the other 10, a portion of the left arch was atretic. Depending on the location of the atretic segment, the anomalies were classified into types A, B, and C double aortic arch. We considered an additional type D double aortic arch which as yet remains a theoretical possibility. With the help of refined angiographic signs obtained by countercurrent right brachial angiography and on the basis of clinical and radiologic signs of tracheoesophageal compression, the differential diagnosis among various types of double aortic arch and right aortic arch anomalies was improved.

Complicated Intestinal Atresias

In this group of 45 intestinal atresia patients (duodenum, 16; jejunum, 24; ileum five) at the University of Mississippi Medical Center, individual hospitalizations ranged up to 245 days. Twelve patients required multiple operations, and the overall mortality rate was 22% (ten patients). While the patients with duodenal atresia had the greatest incidence of other congenital anomalies, including Down's syndrome, the patients with jejunal atresia presented with the most challenging surgical problems. Of the 24 jejunal atresia patients, only three had a single, simple area of obstruction. The remainder were complicated by other gastrointestinal lesions (five patients), by multiple areas of atresia (seven patients) including those in one surviving patient with 22 separate atretic segments, and by the Christmas tree deformity (nine patients). Intraoperative management of the complicated atresia should include: 1) grouping of multiple atresias during resection, 2) adequate resection of the dilated proximal atonic loop, 3) end-to-end anastomoses, 4) avoidance of intraluminal catheters, 5) additional resection of a segment of the distal loop in the Christmas tree deformity and 6) consideration of the shish kebab technique for multiple atretic webs. Postoperative management should involve early intravenous nutrition and repeated exploration for continued obstruction.