Esophageal ganglioneuromatosis; a rare cause of intractable esophageal stenosis: a case report.

BACKGROUND: Ganglioneuromatosis is a rare type of benign neurogenic tumor that usually affects the sites of the major sympathetic ganglia in the retroperitoneum and the posterior mediastinum. Affection of the gastrointestinal tract is rare, and involvement of the esophagus is exceptional. To the best of our knowledge, only 4 cases of esophageal ganglioneuromatosis in adults were reported in the literature. No cases have been reported in the pediatric age group. CASE PRESENTATION: An 11-year-old boy presented with dysphagia due to severe esophageal stenosis caused by esophageal ganglioneuromatosis. CONCLUSIONS: Despite its rarity, the present case implies that ganglioneuromatosis should be considered in children with idiopathic esophageal stenosis.

Shehata technique versus Fowler-Stephens orchidopexy in intra-abdominal testis: A meta-analysis.

INTRODUCTION: The management of intra-abdominal testis (IAT) represents a significant clinical challenge, necessitating the transposition of the testis from the abdominal cavity to the scrotum. This procedure is rendered complex by the abbreviated length of the testicular vessels. OBJECTIVE: Our purpose in this study was to conduct a systematic review and meta-analysis comparing Shehata technique (ST) versus Fowler Stephens technique (FST) in treating patients with IAT. STUDY DESIGN: We conducted a comprehensive literature search using several databases, including Ovid Medline, Cochrane, PubMed, Google Scholar, Web of Sciences, EMBASE, and SCOPUS until February 2024. This study included research that compared ST and FST for managing intra-abdominal testis. We evaluated the rates of atrophy and retraction, as well as the overall success rates, for both techniques. RESULTS: Six studies were identified as appropriate for meta-analysis, comparing orchidopexy performed using the ST with 169 patients, against the FST involving 162 patients. The comparison showed no statistically significant age difference at the time of surgery between the groups (I2 = 0%) (WMD 0.05, 95% CI - 1.24 to 1.34; p = 0.94). Operative time in first the stage was lower in the FST group than ST group (I2 = 95%) (WMD 10.90, 95% CI 1.94 to 19.87; p = 0.02). Operative time in the second stage was lower in the ST group than FST group (I2 = 83%) (WMD - 6.15, 95% CI - 12.21 to -0.10; p = 0.05). Our analysis showed that ST had a similar atrophy rate (I2 = 0%) (OR: 0.45, 95% CI: 0.20 to 1.01; p = 0.05). No difference was found between techniques in terms of retraction rate (I2 = 0%) (OR: 0.64, 95% CI: 0.17 to 2.47; p = 0.52). The ST demonstrated a notably higher overall success rate compared to FST (I2 = 1%) (RR: 1.14, 95% CI: 1.03 to 1.27; p = 0.009). Overall success rate in ST and FST were 87% and 74%, respectively. Overall atrophy rate in ST and FST were 5% and 12%, respectively. Overall retraction rate in ST and FST were 5% and 10%, respectively. DISCUSSION: The ST, renowned for its pioneering two-stage laparoscopic approach that leverages mechanical traction to lengthen the testicular vessels, is gaining popularity due to its recognized safety and efficacy. Conversely, the Fowler-Stephens technique, a traditional method that relies on collateral blood supply for testicular mobilization, has come under examination for its potential link to an increased risk of testicular atrophy. CONCLUSION: This meta-analysis reveals that the Shehata technique has similar or better outcomes compared to the Fowler-Stephens technique in IAT management. Further prospective multicentric randomized controlled trials are warranted.

Sigmoid volvulus; a rare complicated presentation of Hirschsprung's disease: A case report.

INTRODUCTION: Sigmoid volvulus (SV) is a rare complication of Hirschsprung's disease (HD) with only 31 cases have been reported in the English literature. Although its diagnosis is challenging, unrecognized SV is a life-threatening condition requiring early recognition to decrease morbidity and mortality. PRESENTATION OF CASE: A 14-year-old male presented to our emergency department with massive abdominal distention. Plain erect abdominal X-ray showed massive colonic distention with multiple fluid levels. Colonoscopy failed to pass beyond 15 cm after entering dilated sigmoid loop. Open surgical exploration was done through a lower midline incision and revealed SV with massive distention of the entire colon. After detorsion, we found a markedly dilated sigmoid colon with an evident discrepancy at the lower sigmoid. Due to massive colonic dilatation, the decision was made for terminal ileostomy. Histopathological examination of biopsy from the narrow segment demonstrated aganglionosis and hypertrophic submucosal neural fibers confirming the diagnosis of HD. DISCUSSION: SV is a rare serious complication of HD. Unrecognized SV is a serious life-threatening condition, so it should be considered in children with acute or recurrent abdominal pain, distension, constipation and vomiting as early recognition and management of volvulus is essential to decrease morbidity. CONCLUSION: The presented case highlights the possibility of SV as a rare complication of HD should be considered especially in children with a history of chronic constipation and abdominal distension who present with acute colonic distension and failure to decompress despite rectal washes.

Delayed Presentation of Congenital Jejunal Web beyond the Neonatal Period.

Congenital web of the gastrointestinal tract is a rare anomaly which may present at any site of the gastrointestinal tract. In cases with an intact membrane, the presenting symptoms may take the form of complete intestinal obstruction while in other cases with a fenestrated membrane, it may present with partial intestinal obstruction such as failure to thrive, volume depletion, or poor body-weight gain, representing a chronic condition. It is very rare for a jejunal web to present with retention of accidently ingested foreign body. In this report, we document a case of 14-month boy with retention of accidently ingested button battery who was found to have a jejunal web on surgical exploration. This case report stresses on the importance of prevention of ingestion of inanimate foreign bodies especially in young infants and that the absence of symptoms does not preclude presence of foreign body in children. Also, surgeons should be prepared to deal with other unsuspected findings intraoperatively. After a review of the literature, jejunal web should be considered in the differential diagnosis of chronic partial intestinal obstruction even in adults.

Huge ovarian cyst in a neonate with classical 21-hydroxylase deficiency.

Congenital adrenal hyperplasia is the most common cause of ambiguous genitalia worldwide, with an incidence of 1 in 15,000 live births. The most frequently-occurring subtype, 21-hydroxylase deficiency, results in diminished production of aldosterone and cortisol as well as increased androgen secretion. Previous studies have reported a relationship between ovarian cyst formation and adrenal androgen excess; nevertheless, neonatal large ovarian cysts have rarely been reported in newborns with congenital adrenal hyperplasia. Herein, we present the unique case of a neonate with classical 21-hydroxylase deficiency who underwent surgery for a huge unilateral solitary ovarian follicular cyst on the seventh postnatal day. Possible mechanisms by which androgen excess may cause ovarian cyst formation are also discussed.

Role of Wrap-Crural Fixation and Minimal Dissection in Prevention of Transmigration After Laparoscopic Nissen Fundoplication in Children.

Background: Laparoscopic Nissen fundoplication is the gold standard antireflux procedure in pediatric age group. Intrathoracic migration of the fundic wrap is a common cause failure, leading to recurrence of gastroesophageal reflux disease (GERD) symptoms. Objectives: To investigate the impact of wrap-crural fixation and minimal esophageal dissection in prevention of wrap transmigration after laparoscopic Nissen fundoplication in children. Methods: Prospective randomized study of 46 pediatric patients with refractory GERD who underwent laparoscopic Nissen fundoplication divided into two equal groups. In Group A, wrap crural fixation was done, whereas in group B no fixation was done. Minimal esophageal dissection with preservation of the phrenoesophageal ligament was done in both groups. Approval of the Ethics Committee of our Faculty was obtained. Results: There was no difference between both groups regarding operative time, intraoperative complications, or length of hospital stay. Two patients in group B without wrap fixation suffered recurrence of GERD symptoms. On contrast study, they both showed intrathoracic wrap migration. One of them was reoperated. Whereas in group A, no recurrence of symptoms and no wrap transmigration were noticed in follow-up. Conclusion: In laparoscopic Nissen fundoplication, with minimal esophageal dissection and preservation of the phrenoesophageal ligament, there is no additional benefit from wrap-crural fixation in prevention of wrap transmigration.

Congenital short bowel syndrome: a rare cause of neonatal intestinal obstruction.

Congenital short bowel syndrome (CSBS) is an uncommon gastrointestinal disorder in which an unclear aetiology causes considerable intrauterine reduction in small bowel length. As a result of reduced absorptive intestinal length, chronic diarrhoea, vomiting, and consequently, failure to thrive are likely. We report a case of CSBS in a 26-day-old girl who had malrotation and a short bowel with a length of bowel from the pylorus to the ileocecal junction of approximately 40 cm. The patient underwent Ladd's procedure, but she is still dependent on parenteral nutrition.

Acute intrathoracic gastric volvulus: A rare delayed presentation of congenital diaphragmatic hernia: A case report.

INTRODUCTION: Acute intrathoracic gastric volvulus occurs when the stomach undergoes organoaxial rotation in the chest due presence of a diaphragmatic defect. Gastric volvulus associated with congenital diaphragmatic hernia is extremely rare and can be explained as 2 of the 4 ligaments supporting the stomach (gastrophrenic and gastrosplenic) which are connected to the left diaphragm may become elongated or absent. According to the current literature, only 27 pediatric cases have been reported so far. PRESENTATION OF CASE: We describe an 8 years old boy who presented to our emergency department with acute epigastric pain and vomiting. The chest radiograph obtained in the emergency department demonstrated an elevated gastric air-fluid level in the left hemithorax. A computed tomography scan demonstrated a sizable left diaphragmatic defect admitting stomach, small bowel loops and transverse colon with organoaxial gastric volvulus. Emergent laparotomy was done for reduction of the viscera to the abdominal cavity and repair of the diaphragmatic defect. The patient showed an uneventful recovery without experience any pain or difficulty with eating. DISCUSSION: Acute gastric volvulus is a rare pathology defined as an abnormal rotation of the stomach for more than 180° leading to a closed-loop obstruction which may progress to ischemia and strangulation. A plain standing abdominal X-ray and an upper gastrointestinal contrast study are useful for diagnosis, but some authors recommend performing CT or MRI to confirm the diagnosis. The standard treatment for volvulus is open laparotomy with detorsion and anterior gastropexy. CONCLUSION: The presented case highlights congenital diaphragmatic hernia associated with gastric volvulus is a serious condition with very high morbidity and mortality. It should be considered in the differential diagnosis of children with epigastric pain and uncontrolled non bilious vomiting. An upper gastrointestinal contrast study is useful for early diagnosis and surgical treatment should not be delayed awaiting further complementary imaging tests.

Synthesis and spectral properties of new fluorescent hydrazone disperse dyes and their dyeing application on polyester fabrics.

A new series of solid state fluorescent disperse dyes based on hybrid structure of pyrrolinone ester (PES)/coumarin moiety was prepared and fully characterized using spectroscopic analysis such as 1HNMR, MS and IR spectra. The coumarin derivative (7- amino-4- methyl coumarin) was initially prepared from amino phenol via multi steps condensation reactions and its diazonium salt coupled with the pyrrolinone ester to form the hydrazone dyes. 1HNMR data of the prepared dyes exhibited a pure hydrazone form as a mixture of E, Z isomers. These dyes showing a significant emission in solid state as well as on the dyed fabric. The dyeing application on polyester fabrics was investigated which showing that these dyes show high affinity to polyester fabric, and minor change in the dye affinity by changing pH even under alkaline conditions was observed.