Undifferentiated Mesenchymal Neoplasm of the Esophagus in a Child: Case Report and Comparison with Gastrointestinal Stromal Tumor

Mesenchymal neoplasms of the esophagus are relatively uncommon in adults and exceedingly rare in children. Childhood tumors consist almost exclusively of smooth muscle tumors (leiomyomas). We report a case of an undifferentiated mesenchymal neoplasm occurring in the distal esophagus of a 15-year-old boy which is not a gastrointestinal stromal tumor. To our knowledge, this is the first reported case of such a neoplasm occurring in the esophagus of either an adult or child.     

Esophageal reconstruction in very long atresias by elongation of the lesser curvature

The treatment of long esophageal atresias is associated with numerous risks and complications. Bouginage and circular (Livaditis) or spiral myotomy permits the proximal blind pouch to be lengthened by a maximum of 2–3 cm. For longer atresias interposition of a loop of colon or stomach or intrathoracic displacement of the stomach becomes necessary. These measures make actual reconstruction of the esophagus impossible; new means for total preservation of the available esophagus and cardia must be sought. Experimental studies on cadavers have shown that mobilization of the distal esophagus is possible only to a limited extent due to the fixation of the lesser curvature and left gastric artery and that tension on the esophagus affects primarily this curvature. Ligation of the left gastric artery and transverse/diagonal division of the lesser curvature permits mobilization of 6–8 cm of distal esophagus with preservation of the cardia, ensuring that primary anastomosis of the esophageal ends is always possible. We have performed mobilization and transverse division of the lesser curvature using a stapler in five children with an esophageal gap of 4–8 cm. The esophageal anastomoses were tension-free and either intrathoracic or cervical. In one case of atresia without a tracheoesophageal fistula the esophagus was drawn retrosternally to the neck, making a thoracotomy unnecessary. In all patients a semifundoplication was performed as an anti-reflux measure and a pyloroplasty was done to improve gastric emptying. Complications were few after these procedures: one leaking anastomosis in the neck closed spontaneously, two stenoses required bouginage of the anastomosis. In most cases of esophageal atresia, even those with very long gaps, primary anastomosis of the esophagus is possible by elongating the lesser curvature. Substitution plasty can always be avoided. Our results in five children with this procedure have been encouraging.     

Bilateral epstein-barr virus-associated adrenal leiomyomas in a child without an established immunodeficiency

ABSTRACT Adrenal leiomyomas are rare, bilateral ones being rarer. Literature available on these rare tumors documents only 4 cases in children less than 12?years of age. Each case has been associated with acquired immune deficiency syndrome or some other immunodeficiency state. Here we present a rare case of large, bilateral, adrenal leiomyomas in a child with no known immunodeficiency. An 11-year-old girl with a past history of herpes zoster (1?year before the present complaints) was admitted with abdominal pain of 2?months' duration. Radiology revealed bilateral adrenal neoplasms, probably bilateral pheochromocytoma. Histology showed bilateral adrenal leiomyomas that were Epstein-Barr virus associated. We report this case to draw attention to the occurrence of a common pathologic entity at an uncommon site in a setting of no definite known immunodeficiency.     

Results of the operative treatment of gastroesophageal reflux in childhood with particular focus on patients with esophageal atresia.

AIM: Although the literature on fundoplications in childhood is relatively extensive, only few reports exist which correlate the rate of complications with the primary disease. Other important questions such as the incidence of postoperative dumping syndrome or Barrett's esophagus in childhood are barely treated. Nor have operative techniques such as single or double-row cuff sutures or the benefit of performing pylorotomy or pyloroplasty in addition to fundoplication been investigated with respect to their recurrence rates. In particular studies on laparoscopic fundoplications tend to be generally confined to a discussion of the feasibility of the endoscopic procedure, the duration of the intervention, the length of the hospital stay and the costs. The study presented here aims to analyze such still unanswered questions listed above for open fundoplication procedures, including an analysis of the authors' own patient population, and to discuss the questions together with the most important reports in the literature. The aim is to create a basis for later studies which will compare conventional and laparoscopic fundoplications. METHODS: In the period between 1993 and 2005, 160 children underwent a fundoplication procedure. From 2003 onwards, fundoplications were carried out laparoscopically. The data of 148 patients were analyzed, some of them on the basis of clinical follow-up and some on the basis of an extensive questionnaire and among others with the help of the parents' support group KEKS. RESULTS: The underlying disease in 87 patients was reflux disease stemming from esophageal atresia, previous diaphragmatic operation in 8 children and mental retardation with swallowing difficulties in 30 patients. Only 23 patients suffered from isolated gastroesophageal reflux disease without an underlying primary disease. Intraoperative complications occurred in 4.6 % of patients with esophageal atresia (EA), while the rate for the remaining collective of patients was 1.6 %. Postoperative complications were observed in 10.3 % of the children with esophageal atresia and in 8.2 % of the other cases. The recurrence rate was 16.1 % in the children with EA and 6.5 % in the other cases. Dysphagia and/or stenosis occurred in 17.2 % and 6.5 % of children, respectively, and dumping syndrome was observed in 18.3 % of the EA group and only in 1.6 % of the comparison group. An evaluation of 79 esophageal biopsies showed no difference between patients with EA and the comparison group concerning the degree of histological changes. When evaluating the suturing technique, it was found that a double-row fundus suture was more effective in preventing reflux recurrence than a single-row suture but also resulted in an increased rate of dysphagia. When considering the not infrequent occurrence of dumping syndrome, it was found that drainage operations such as pyloroplasty or pyloromyotomy are only indicated for reduced gastric motility. The data of the children without EA, who initially served as a comparison group, was analyzed further. Four summaries of the current literature in table form complete this discussion. CONCLUSION: Particularly in children with EA, Nissen fundoplication cannot be considered a procedure with few complications. The problems resulting from disturbed gastric and esophageal motility should not be underestimated nor should the postoperative occurrence of dumping syndrome. Postoperative long-term follow-up until the patients reach adulthood is absolutely necessary to ensure that development of a Barrett's esophagus is not overlooked.     

小儿食管良性狭窄的诊断与外科治疗

小儿食管良性狭窄２６例，经手术、病理证实属气管软骨迷入型１６例（６１．５％），胃粘膜异位型２例（７．７％），肌层增生肥厚型３例（１１．５％），Ａｌｌｉｓｏｎ－Ｊｏｈｎｓｔｏｎ狭窄合并食管裂孔疝及返流者４例（１５．４％），Ｓｃｈａｔｚｋｉ纤维环性狭窄１例（３．９％）。由于病因和狭窄程度不同，诊治方法各异。结合本组病例特点，就诊断与外科治疗问题进行讨论。     

Esophageal replacement using the colon: a 15-year review

Colonic interposition is a treatment option in childhood when esophageal replacement (ER) is necessary. We reviewed 18 children who underwent ER by colon between 1984 and 1999. There were 5 with esophageal atresia and 13 with corrosive esophagitis; 15 had long-term follow-up (mean 38 months). Three procedures were performed by the Waterston technique and 12 by the retrosternal technique. ER was completed in a single stage in all but 1 patient. Pyloroplasty or antireflux surgery were not done routinely during colonic interposition. As early complications, we observed 11 cervical leaks and 2 pulmonary problems. As late complications, there were 4 redundancies, 3 gastrocolic refluxes, 2 cervical anastomotic stenoses, and 1 each intestinal obstruction due to adhesions, cologastric stricture, cosmetic deformity of the thorax, and bulging of the neck. Six patients with complications required secondary surgery. There were 4 deaths, 2 of them unrelated to the surgery. Cervical leakage, which was the most commonly observed problem, healed well. We believe the colon is still one of the best substitutes for the esophagus and that there is no need to perform a routine pyloroplasty or antireflux procedure as an adjunct to the primary surgery. Accepted: 12 January 2000     

A long-term survival case of tracheal agenesis: management for tracheoesophageal fistula and esophageal reconstruction

Tracheal agenesis is a very rare disorder which leads to severe respiratory disorders immediately after birth. Reports are very limited on long-term survival cases. We report here a long-term survival case with Floyd’s type I tracheal agenesis. During the neonatal stage, the patient underwent abdominal esophageal banding to substitute esophagus for trachea and transection at the cervical esophagus with esophagostomy. Subsequently, airway management was difficult due to a fragile tracheoesophageal fistula, but the fistula was conservatively treated and stabilized with the patient’s growth. This patient is a very rare case in whom oral feeding was achieved after esophageal reconstruction using a gastric tube. For this case, we describe mainly (1) the management method of the tracheoesophageal fistula and (2) esophageal reconstruction without thoracotomy.     

小儿食管异物71例临床分析

目的：探讨小儿食管异物及其并发症的特点及治疗方法以指导临床诊治。方法对2009年1月至2014年12月我院耳鼻喉科收治的71例食管异物患儿的临床资料进行回顾性分析。结果食管异物患儿71例,其中男42例,女29例;年龄2个月~14岁,1~3岁发病率最高。在食管内异物存留时间短者2 h,长者15 d。吞入金属性异物43例,植物性异物12例,动物性异物9例,化学性异物7例;异物位于食管上段51例,中段9例,下段7例,食管入口上3例,入胃1例。71例患儿中发生并发症9例。71例患儿治愈69例,好转2例,治愈率为97.2%,平均治愈时间4 d。结论及早诊治、避免食管穿孔是预防小儿食管异物并发症的关键,及时准确处理食管穿孔等并发症是提高治愈率的有力举措。     

Leiomyoma of the esophagus and bronchus in a child

Leiomyomas of the esophagus and/or the bronchus have rarely been reported in children. To our knowledge, the simultaneous presence of this tumor in both the esophagus and a bronchus in a child has not been previously reported. A 7-year old boy presented with respiratory and esophageal symptoms and was found to have a leiomyoma of the esophagus and right main bronchus. The esophageal leiomyoma was treated with limited myotomy, but bronchoscopic resection was possible for the bronchial lesion. The postoperative result was excellent, with normal swallowing and no residual respiratory problems at 1-year follow-up. Accepted: 24 September 1996     

Management of esophagogastric corrosive injuries in children.

There has been an increase in the number of patients admitted to our hospital with caustic esophageal injuries during the last five years. The aim of this study was to analyze the complications and results of the treatment of corrosive esophagogastric injury. Between 1990 and 2000, 120 caustic ingestion accidents were admitted to our unit. The mean age was 4 years, with a 2 : 1 male to female ratio. The average time between the caustic ingestion and admission to hospital was 14.9 days. The ingested substances were alkali in 80.9 % and acid in 19.1 % of the cases. Stenosis of the esophagus developed in 31 (25.8 %) and gastric outlet obstruction (GOO) in 6 (5 %) patients. Management of the esophageal stricture consisted of dilatation in 28 patients. Three children underwent colonic interposition without a dilatation attempt. Six children were lost to follow-up; 4 patients were successfully treated; 13 patients were still in the dilatation program at the time of writing with 6 improving and 2 patients waiting for interposition surgery; 4 patients underwent colonic interposition and 1 patient underwent resection of the stenotic part of the esophagus. Among the patients in the dilation program, we observed 4 esophageal perforations. Three of them were treated medically and further dilatations were carried out, while one was managed by colonic interposition. The treatment modalities for GOO cases consisted of pyloroplasty in 3, Billroth I in 2 and balloon dilation of the pylorus in 1 child. Although balloon dilatation of the esophagus carries the risk of perforation, it should be the first line of treatment in suitable cases. GOO cases may require surgical therapy following a detailed endoscopic evaluation.     

Contribution of ultrasonography in the diagnosis of achalasia.

We present a case of achalasia diagnosed with the aid of ultrasonography (US). After having examined the clinical-radiological characteristics of the disease, and after having noted the diagnostic possibilities of manometry and endoscopy, we emphasize the use of US to clearly show the thickening of the muscular wall and the dilatation of the distal esophagus. In those of pediatric age, particularly, US permits differential diagnosis with the esophageal leiomyoma, which is difficult to obtain with other techniques. We suggest, therefore, including US in the study of patients with stenosis of the third distal section of the esophagus.     

Esophageal duplication in children: a report of three cases evaluated by computed tomography

Primary tumors of the esophagus are extremely rare in children. A common periesophageal mass in children is duplication. It constitutes only 0.5 to 2.5% of all esophageal lesions usually diagnosed in infancy or early childhood. Duplication of the esophagus may be diagnosed on examination by the emergency department (ED) physician when it produces symptoms or when it is inadvertently found on a chest radiograph. The duplication may produce acute gastrointestinal symptoms such as vomiting and dysphagia or respiratory symptoms such as cough, wheezing, or dyspnea. Currently the examination of choice for evaluation of esophageal duplication is a barium swallow. However, computed tomography (CT) has the advantage over conventional diagnostic procedures, since it demonstrates the cystic nature of the mass and its relationship to adjacent structures in a noninvasive manner. An esophageal duplication has a well-marginated spherical mass contiguous with the esophagus, with preserved surrounding fat planes, and numbers of 15 to 30 Hausenfield units (HU) on CT. We are reporting three cases of this anomaly as demonstrated by CT.     

Respiratory distress due to a posterior mediastinal mass following failed repair of esophageal atresia

Respiratory distress due to masses in the posterior mediastinum have been extensively described, including duplication cysts of the esophagus. Retained esophagus following failed repair of esophageal atresia (EA) and esophageal replacement was described only once. Double atresia and midportion esophageal cyst is also a rare entity. We describe a baby with respiratory distress following esophageal replacement for failed repair of EA with tracheoesophageal fistula who was cured by the removal of an overlooked mediastinal cystic mass of esophageal origin at the age of 16 months.     

Cervical esophageal perforation diagnosed by endoscopy in a premature infant: review of recent literature

A case of cervical esophageal perforation in a premature infant by an orogastric polyvinyl chloride (PVC) tube is reported. Perforation appeared as an esophageal atresia, suspected because of the inability to aspirate gastric secretions. Initially, atresia was confirmed by an esophagogram, but endoscopy revealed a "double esophagus" with a normally located and developed esophagus and a blind mediastinal fistula starting at the opposite side of the glottis. The "double esophagus" was confirmed by an esophagogram, followed by a contrast study in the pharynx. Esophageal perforation in the neonate is an iatrogenic disease that may mimic esophageal atresia. We recommend endoscopy instead of contrast studies for suspected esophageal atresia.     

Beta-sarcoglycanopathy

Sarcoglycanopathies are relatively rare progressive muscular dystrophies with autosomal recessive inheritance; which belong to the group of limb girdle muscular dystrophies. The phenotype resembles dystrophinopathies due to proximal muscle weakness and calf hypertrophy. Reports from the Indian subcontinent are scarce. The authors report a case of primary beta-sarcoglycanopathy and describe literature pertaining to this rare entity.     

Unusual osteoblastoma of the skull

Benign osteoblastoma is a rare primary bone tumor, the location in the frontal bone being particularly rare (only a few cases described). The youngest age of occurrence reported is 3 years. We present a case of osteoblastoma occurred in a 4 1/2-month-old boy, located in the frontal bone.     

Two hyperplastic esophagogastric polyps in a child with neurofibromatosis type 1 (NF-1).

Hyperplastic esophagogastric polyps usually occur in the distal esophagus or gastroesophageal junction and have been associated with damage to the esophageal mucosa. Histologically these polyps show hyperplastic gastric foveolar and/or squamous epithelium with inflamed stroma. Reports of esophagogastric polyps in the pediatric population are rare. Most of these reports only describe chronic inflammation within the lamina propria of the polyp, with only rare reports specifying the presence of epithelial hyperplasia. There have been 2 previous cases of hyperplastic esophagogastric polyps occurring in the context of neurofibromatosis type 1 (NF-1) in a single article. Here we report a 3rd case of hyperplastic esophagogastric polyps occurring in an 11-year-old male with NF-1. This case is unique in that there were 2 polyps in the same patient and in that the polyps showed hyperplastic gastric peptic glands in addition to foveolar-type and focal squamous epithelium. The case is discussed and literature reviewed.     

Syringocystadenoma papilliferum: a rare breast tumor in a young boy.

Syringocystadenoma papilliferum of the breast is extremely rare in children. However, some occasional cases have been diagnosed in females. This report documents a case of syringocystadenoma papilliferum occurring in a healthy 4-year-old male. Histopathologically, this tumor, located deep in the dermis, was characterized by the presence of papillary projections extending into the lumen of epithelial cystic invaginations in a background of dense fibrous tissue. The papillary projections and the duct-like structures were lined by an inner columnar epithelium, which demonstrated luminal apical decapitation secretion, and had an outer layer of smaller cuboidal cells. These findings support apocrine differentiation in this case. A fairly dense mononuclear infiltrate in the stroma of the tumor, especially in the papillary projections, was also present. Although syringocystadenoma papilliferum is rare in children, it should not be summarily ruled out during examinations of masses found in the pediatric male breast. It is important to recognize this unusual location of the tumor so that a prompt diagnosis, based on histopathology and surgical excision, can be proposed.     

High doses of steroids in the management of caustic esophageal burns in children]

Esophageal burns are frequent in some countries. Esophageal stricture is a severe complication after caustic ingestion. Its frequency is estimated to 5% and reaches 47% in severe esophagitis. AIM OF THE STUDY: To study the influence of management of severe esophageal burns with high doses of steroids in the occurrence of esophageal stricture through the experience of an endoscopic unit. PATIENTS AND METHODS: Twenty-six children with a mean age of 3.5 years (15 months-8 years) with a second b- (N =22) or a third-degree (N =4) esophageal burns due to accidental ingestion of a caustic substance were included between 1993 and 1999. Corrosive substances ingested were sodium hydroxide (N =17), bleach (N =4), alkali (N =3), others (N =2). Upper GI endoscopy was performed in 17 children within the 24 hours of caustic ingestion and within the first 48 hours in all cases. All patients received methylprednisolone (1 g/1.73 m2/day) plus cimetidine and ampicillin plus early oral feeding resumption. RESULTS: Three children (2 grade II et one grade I) were not controlled on day 30 of the protocol and have then been excluded. The remaining 23 children were divided in two groups depending on the upper GI endoscopic results: group I of 12 patients completely or partially healed and group II of 11 patients who developed an esophageal stricture. No difference was observed between the two groups with regard to the delay between caustic ingestion and the start of treatment and the number of shots of methylprednisolone. Second b- degree esophagitis complicated with an esophageal stricture underwent a median of five esophageal dilations (1-12). After a median follow-up of three years, four of them have a normal esophagus. All children with a third-degree esophagitis developed an esophageal stricture. One had a surgical replacement of the esophagus with a segment of colon with good outcome. The three others underwent a median of seven esophageal dilations (5-10). One of them has a normal esophagus after a follow-up of five years while the two others were lost to follow-up. CONCLUSION: High dose of corticosteroids seems to improve second b-degree esophagitis prognosis and may prevent from esophageal stricture.     

The use of collagen-coated vicryl mesh for reconstruction of the canine cervical esophagus

Collagen-coated vicryl mesh (CCVM) was used experimentally to patch partial and total segmental defects in the canine cervical esophagus. The esophageal healing process over a 6-month period with successful incorporation of the CCVM and esophageal wall regeneration are described. Twenty-two (91.7%) of 24 dogs survived the procedure without any evidence of esophageal stricture or dysphagia. CCVM has proved to be a suitable material for substitution of esophageal tissue in the canine model. Its clinical use in congenital and acquired esophageal lesions should be considered only pending further experimental laboratory work. Accepted: 28 February 1997