Congenital microgastria with esophageal stenosis and diaphragmatic hernia

A rare case of congenital microgastria in association with distal esophageal stenosis and left-sided congenital diaphragmatic hernia is reported. Other features included megaesophagus and asplenia. The probable causative factors are discussed.     

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.     

Congenital esophageal stenosis associated with tracheoesophageal fistula

Two patients with congenital esophageal stenosis associated with an H-type tracheoesophageal fistula in one and esophageal atresia with distal fistula in the other are presented. In both patients the correct preoperative diagnosis was made by esophagram. Satisfactory results were obtained with surgical excision. Histologic studies revealed fibromuscular stenosis and membranous esophageal mucosa, respectively.     

Esophageal duplication and congenital esophageal stenosis

Esophageal duplication and congenital esophageal stenosis (CES) may represent diseases with common embryologic etiologies, namely, faulty tracheoesophageal separation and differentiation. Here, we will re-enforce definitions for these diseases as well as review their embryology, diagnosis, and treatment.     

Congenital pyriform aperture stenosis

Nasal airway obstruction is a potentially life-threatening condition in the newborn. Neonates are obligatory nasal breathers. The pyriform aperture is the narrowest, most anterior bony portion of the nasal airway, and a decrease in its cross-sectional area will significantly increase nasal airway resistance. Congenital nasal pyriform aperture stenosis (CNPAS) is a rare, unusual form of nasal obstruction. It should be considered in the differential diagnosis of any neonate or infant with signs and symptoms of upper airway compromise. It is important to differentiate this level of obstruction from the more common posterior choanal stenosis or atresia. CNPAS presents with symptoms of nasal airway obstruction, which are often characterized by episodic apnea and cyclical cyanosis.     

Congenital hypertrophic pyloric stenosis

Pyloric stenosis (PS) has been reported in multiple births. We report the presentation of PS in identical twins, whose symptoms led to simultaneous referral for surgical care. The theories of inheritance of PS are reviewed. Correspondence to: J. S. Young     

Congenital tracheobronchial stenosis in monozygotic twins

Conclusion This case report shows that tracheobronchial stenosis may present in monozygotic twins. The pattern of malformation in twins differs from cases described previously. Received: 7 April 1998 / Accepted in revised form: 1 July 1998     

婴幼儿食管瘢痕狭窄的外科治疗

目的：回顾性分析婴幼儿食管良性狭窄30例外科治疗结果。探讨外科治疗各种方法的优劣。方法：30例中男20例，女10例。年龄1岁5个月-5岁，平均29个月。体重5-20kg。除1例为农药烧伤外，余均为误服强酸、碱烧伤。颈段食管完全闭塞3例，基本闭塞5例，余食管造影均见钡剂在食管全长呈不规则线样通过、其中5例伤后曾有时间不等声嘶及吸气性呼吸困难，6例在外院行胃造瘘，全部病例采用保留结肠左动脉升支供血，经胸骨后径路顺蠕动吻合横结肠代食管结肠颈部吻合或结肠咽吻合。结果：手术后颈部吻合口瘘1例，吻合口狭窄1例。术后气管切开1例，经治疗后顺利进食，无手术死亡。经4-20余年随访，患儿发育正常。结论：主张对的食管瘢痕狭窄应积极采取食管重建术。食管替代物中以横结肠为最佳。同期食管瘢痕切除食管的危险性大，手术应以食管旷置为佳。重建平面需在颈部或咽部，不主张任何形式的胸内吻合。     

Congenital pyloric stenosis: a modified umbilical incision for pyloromyotomy

Thirty-two patients underwent a pyloromyotomy via an umbilical incision; in 11 a modified umbilical approach was used to facilitate delivery of the pyloric mass. Incisions are made in the skin fold of the upper half of the umbilicus and at the midline, joining the two at the top. The skin incision is closed by upper umbilical translocation with a very good cosmetic result. This incision allows easy access to the pylorus and provides more convenient exposure. The absence of traction on the retractors avoids tissue ischemia, which leads to wound abscess development.     

Intramural tracheobronchial remnants associated with esophageal atresia: diagnostic aids

A patient with isolated esophageal atresia and congenital esophageal stenosis resulting from intramural tracheobronchial remnants (TBR) in the distal esophagus is presented. The esophagographic appearance and operative findings suggesting the presence of TBR are discussed.     

Congenital ureteric stenosis: a study of 17 children

Aim  To review cases of congenital ureteric stenosis treated in the period between 1999 and 2007. We propose to analyze the type of presentation, management and results. Material and methods  We report 17 children aged 20 days to 8 years with obstructive uropathy due to congenital stenosis of the ureter at one or more levels. This condition could be mistaken for the more common pelviureteric junction obstruction (PUJO) or primary megaureter, but it is a distinct and more serious anomaly. 13 of the 17 children had one or more associated anomalies, the most significant of which was a contralateral multicystic dysplastic kidney. Other associated anomalies included PUJO, megacalyx, vesicoureteric reflux, urogenital sinus, duplicate vagina, anorectal malformation and agenesis of the bladder. 16 children were symptomatic at presentation, with uremia (serum creatinine >1 mg/dl) in 5, while 1 was diagnosed antenatally. The correct preoperative diagnosis was made in only three children. Reconstruction included ureteroureteral anastomosis, ureteric reimplantation or ureteral substitution. Results  There is follow up for 15 of the 17 patients. Length of follow up ranges from 1 to 7 years (average 2.7 years). There was satisfactory urinary drainage established in all 17 cases and uremia has resolved 3 of the 5 children. The children with solitary functioning kidney are at risk of uremia in later life. Conclusion  Congenital ureteric stenosis is a rare condition, but distinct anomaly with possible grave consequence and has been distinguished from other causes of congenital ureteric obstruction.     

Lower esophageal sphincter function in infantile hypertrophic pyloric stenosis

Esophageal manometry was performed in 18 infants between 29 and 105 days of age with hypertrophic pyloric stenosis. Continuous 24-h esophageal pH monitoring was also carried out in 9 cases before and after pyloromyotomy in order to study lower esophageal sphincter (LES) function in infantile hypertrophic pyloric stenosis (IHPS). The mean preoperative pressure and length of the LES in IHPS were 9.30 mmHg and 1.30 cm respectively, which were significantly lower and shorter than those in controls (P <0.01), and gastroesophageal reflux was highly significant. The esophageal manometric studies showed that LES pressure increased markedly on the 7th postoperative day to 12.58 ± 2.56 mmHg and returned to normal values within 2 to 11 months after operation. The pH monitoring indicated that in most cases reflux had disappeared on the 7th postoperative day and the reflux parameter decreased from 8.34% to 3.34%. Our data suggest that most cases of IHPS (12/18) were accompanied by LES incompetence, but LES competence as a pressure barrier at the gastroesophageal junction became normal after operation. Offprint requests to: Y. X. She     

Congenital nasal piriform aperture stenosis with vestibular abnormality

We present a neonate with congenital nasal piriform aperture stenosis associated with an abnormal vestibular aperture. Radiological evaluation with CT is essential to confirm the diagnosis and delineate the anatomy for surgical planning. Extension of the scan field of view to include the petrous temporal bone is essential to identify associated abnormalities of the vestibule.     

Management of congenital distal esophageal stenosis with combined Collis gastroplasty-Nissen fundoplication

Surgical resection for the treatment of congenital distal esophageal stenosis due to tracheobronchial remnants is sometimes complicated by esophageal shortening and gastroesophageal reflux. We propose the combined operation of Collis gastroplasty and Nissen fundoplication to prevent these complications. It can be safely performed through an abdominal approach.     

Communicating bronchopulmonary foregut malformation: particular emphasis on concomitant congenital tracheobronchial stenosis

A total of four patients with communicating bronchopulmonary foregut malformation were treated surgically at Kobe Children’s Hospital between 1993 and 2004. Of these, three patients displayed congenital tracheobronchial stenosis and developed life-threatening respiratory distress soon after birth. In each case, anomalous bronchi arose from the lower portion of the esophagus and connected to the lower part of the ipsilateral lung. This anomaly involved the right lung in two patients, and the left lung in one patient. Tracheobronchial stenosis extended from the inlet of the thorax to the carina in one patient, and to the contralateral main stem bronchus in two patients. Surgical treatment included division of the esophageal bronchus and anastomosis of bronchus to the trachea in one patient. In the other patient, the ipsilateral lung was resected and the stenotic tracheobronchus was stented. The remaining patient underwent pneumonectomy of the ipsilateral lung. Details of this fatal anomaly and a discussion of appropriate surgical management are described herein.     

腹腔镜胃造口术联合Forley尿管球囊扩张治疗小儿瘢痕性食管狭窄

目的 探讨腹腔镜胃造口术联合Forley尿管球囊扩张治疗小儿瘢痕性食管狭窄的可行性和疗效.方法 自2006年7月至2014年2月间河北医科大学第二医院小儿外科收治瘢痕性食管狭窄患儿11例,男8例,女3例,年龄(39.27±29.32)个月.均于全身麻醉下行腹腔镜胃造口术,术后第7天利用简易Forley尿管球囊进行狭窄食管扩张.于术后1个月、3个月、6个月、1年、2年回院复查,食管钡餐检查,测量对比术后食管扩张不同时间段狭窄段食管直径变化.结果 所有11例患儿均顺利完成手术,手术时间(24.叭±5.02)min,出血量(15.91±4.37)ml,术后住院(8.18±0.98)d.术后第7天开始扩张食管,操作顺利.扩张1个月后,4例可进少量固体食物;3个月时,9例可进普通饮食;6个月时,患儿几乎全部恢复正常饮食.术后1～3个月期间食管扩张效果最明显,与其他时间段比较均有显著型差异(F=13.407,P=0.000＜0.01).术后造瘘口周围皮炎或湿疹8例,造瘘口脱垂1例,扩张后食管黏膜出血2例,无造瘘口回缩、食管破裂穿孔、导丝或尿管断裂、腹膜炎等发生.结论 腹腔镜胃造口术联合Forley尿管球囊扩张治疗小儿瘢痕性食管狭窄,损伤轻、出血少、恢复快,采用器械简单,操作方法简易,疗效明显.     

小儿先天性肥厚性幽门狭窄合并肠旋转不良的临床特点

目的 探讨小儿先天性肥厚性幽门狭窄合并肠旋转不良的临床特点.方法 回顾性分析2003年1月至2009年12月于我科治疗的6例先天性肥厚性幽门狭窄合并肠旋转不良患儿的临床资料.结果 通过胃肠透视及超声检查,4例患儿术中确诊该病并经1次手术解决,2例术前仅诊断为先天性肥厚性幽门狭窄,行幽门肌切开术,再次出现呕吐后行2次手术发现肠旋转不良.所有病例预后良好.结论 先天性肥厚性幽门狭窄合并肠旋转不良较为少见,病因不清,先天性肥厚性幽门]狭窄的症状易掩盖肠旋转不良的临床表现而贻误其诊断.胃肠透视对于诊断该病有重要意义,彩超检查有良好的应用价值,及时的诊断治疗可获得良好的治疗效果.     

Intramural esophageal pseudodiverticulosis

A case of intramural esophageal pseudodiverticulosis in a five-year-old with a 16-year follow up is presented.     

先天性食管狭窄15例诊疗体会

目的 总结先天性食管狭窄诊断和治疗体会,提出最佳的诊断、治疗方法.方法 回顾我院1990至2009收治的15例先天性食管狭窄临床资料,对临床表现(症状、体征、发病年龄等),诊断方法(UGI、胃镜等)进行分析.将患儿分为手术组与非手术组,比较两组治疗过程和预后情况(饮食状况、并发症等).结果 15例先天性食管狭窄患儿,男9例,女6例.发病年龄(10±5.5)个月(0～3岁9个月).主要临床表现为反复进食后呕吐.GI检查发现狭窄段位于食管下段者9例,位于中下段者6例.7例仅行胃镜辅助下食管扩张术,手术治疗8例,其中6例行狭窄段食管切除+端端吻合术,1例行狭窄段肌层切开+胃底折叠术,1例行狭窄段全层切开、纵切横缝+胃底折叠术.术后病理诊断2例为气管软骨异位型,1例为狭窄局部炎性假瘤形成,5例为肌层增厚型.术后4例出现吻合口狭窄,2例出现胃食管反流,1例伴发术侧膈膨升.结论 上消化道钡剂造影(UGI)是诊断首选方法,电子胃镜可作为诊断及辅助治疗的手段.治疗方法建议首选食管扩张术,对扩张2～3次以上无效者进行手术探查.下段狭窄者应行胃底折叠术避免术后并发胃食管反流.

Abstract：

Objective The aim of this study was to find an optimal procedure of diagnosis and treatment for congenital esophageal stenosis (CES). Methods From 1990 to 2009, 15 CES patients were treated in our hospital. Demographical, clinical, pathological and radiological data were collected retrospectively. Patients were further divided into surgery group and non-surgery group. Outcomes and complications were studied to identify the indication and timing for operation. Results The age of presentationof of these 15 patients (9 boys and 6 girls) were 10± 5. 5 months (1 day ～ 41months).Vomiting was the most significant and common symptomign. UGI were performed in all the 15 patients. 9 patients had stenosis in lower esophagus and 6 in mid-lower esophagus. In surgery group, 6patients underwent surgical resection; the remaining 2 patients underwent Heller myotomy esophagomyotomy and stricturoplasty, respectively. Tracheobronchial remnants were found in 2 patients and submucosal thickening were found in 5 patients. Inflammatory pseudotumor was found in the rest 1patient. The frequency of esophageal anastomotic stricture, gastroesophageal reflux and diaphragmatic erentration were 44.4 % (4/9), 22. 2 % (2/9) and 11.1% (1/9), repectively. Conclusions UGI is the first choice for the diagnosis of CES, while electronic gastroscope can play an alternative role. Esophageal dilatation could be the first therapy for CES, and operation is indicated when outcome dilatation fails.