Ineffective esophageal motility: Characterization and outcomes across pediatric neurogastroenterology and motility centers in the United States.

OBJECTIVES: Ineffective esophageal motility (IEM) on high-resolution manometry (HRM) is not consistently associated with specific clinical syndromes or outcomes. We evaluated the prevalence, clinical features, management, and outcomes of pediatric IEM patients across the United States. METHODS: Clinical and manometric characteristics of children undergoing esophageal HRM during 2021-2022 were collected from 12 pediatric motility centers. Clinical presentation, test results, management strategies, and outcomes were compared between children with IEM and normal HRM. RESULTS: Of 236 children (median age 15 years, 63.6% female, 79.2% Caucasian), 62 (23.6%) patients had IEM, and 174 (73.7%) patients had normal HRM, with similar demographics, medical history, clinical presentation, and median symptom duration. Reflux monitoring was performed more often for IEM patients (25.8% vs. 8.6%, p = 0.002), but other adjunctive testing was similar. Among 101 patients with follow-up, symptomatic cohorts declined in both groups in relation to the initial presentation (p > 0.107 for each comparison) with management targeting symptoms, particularly acid suppression. Though prokinetics were used more often and behavioral therapy less often in IEM (p ≤ 0.015 for each comparison), symptom outcomes were similar between IEM and normal HRM. Despite a higher proportion with residual dysphagia on follow-up in IEM (64.0% vs. 39.1%, p = 0.043), an alternate mechanism for dysphagia was identified more often in IEM (68.8%) compared to normal HRM (27.8%, p = 0.017). CONCLUSIONS: IEM is a descriptive manometric pattern rather than a clinical diagnosis requiring specific intervention in children. Management based on clinical presentation provides consistent symptom outcomes.

Time is Gut. Approaching Intestinal Leiomyositis: Case Presentation and Literature Review.

T-lymphocytic intestinal leiomyositis is a rare cause of "pediatric intestinal pseudo-obstructions." Diagnosis may be difficult and requires full-thickness bowel biopsies during laparotomy or laparoscopy with possible enterostomy. Currently, immunosuppressive therapy is the only available treatment. A delay in diagnosis and therapy may negatively affect the prognosis because of ongoing fibrotic alterations; therefore, early diagnosis and consequent treatment are crucial. This review summarizes the available information on the nosology, diagnostic steps, and treatment modalities. Here, we report the youngest case of enteric leiomyositis reported in the last two decades and analyze its management by reviewing previous cases.

[Click phenomenon in acquired Jaensch-Brown syndrome and trigger finger/thumb: the Notta syndrome]. / "Klick"-Phänomen beim erworbenen Jaensch-Brown-Syndrom und schnellenden Finger/Daumen: dem Notta-Syndrom.

CLINICAL FEATURES: The click phenomenon occurs when an acquired mechanical restriction of the elevation in adduction of the eye or of the extension of the finger/thumb, is forcefully overcome. The common cause is a nodule either of the superior oblique tendon posterior to the trochlea in the case of a Jaensch-Brown syndrome or of the digital flexor tendon anterior to the A1 annular pulley in the case of a trigger finger. Both locations share similar anatomical conditions for the development of the nodule and the pathomechanism of the click. RESULTS: From these identical findings in the eye and the hand in small children it can be assumed that the results from the studies of the hand in newborns and infants with a trigger thumb/finger are also applicable to the situation of the eye. 1. This motility disorder is not congenital. This is most likely due to an incomplete development at the time of birth of the sliding factors needed for a free passage of the tendon through the trochlea and the A1 annular pulley. 2. A distinction must be made between stages 0-3: stage 0 = no more restriction of the motility and no click phenomenon; stage 1 = forced active extension/elevation possible; stage 2 = only passive extension/elevation, each with a click phenomenon; stage 3 = no extension/elevation possible and no click phenomenon. 3. In most cases in early childhood there is a spontaneous complete recovery (75% after 6-7 years). In the eye this spontaneous course can only limitedly be shortened with motility exercises in combination with segmental occlusion. CONCLUSION: The click phenomenon is a symptom of stages 1 and 2 of an acquired mechanical restriction of the elevation in adduction of the eye or the extension of the finger/thumb. It should not be called a syndrome.

The origin of intraluminal pressure waves in gastrointestinal tract.

The gastrointestinal (GI) peristalsis is an involuntary wave-like contraction of the GI wall that helps to propagate food along the tract. Many GI diseases, e.g., gastroparesis, are known to cause motility disorders in which the physiological contractile patterns of the wall get disrupted. Therefore, to understand the pathophysiology of these diseases, it is necessary to understand the mechanism of GI motility. We present a coupled electromechanical model to describe the mechanism of GI motility and the transduction pathway of cellular electrical activities into mechanical deformation and the generation of intraluminal pressure (IP) waves in the GI tract. The proposed model consolidates a smooth muscle cell (SMC) model, an actin-myosin interaction model, a hyperelastic constitutive model, and a Windkessel model to construct a coupled model that can describe the origin of peristaltic contractions in the intestine. The key input to the model is external electrical stimuli, which are converted into mechanical contractile waves in the wall. The model recreated experimental observations efficiently and was able to establish a relationship between change in luminal volume and pressure with the compliance of the GI wall and the peripheral resistance to bolus flow. The proposed model will help us understand the GI tract's function in physiological and pathophysiological conditions.

Resolution of severe gastroparesis induced by parasympathetic surge following facial trauma: a case report.

BACKGROUND: Gastroparesis is a condition that affects the motility of the gastrointestinal (GI) tract, causing a delay in the emptying process and leading to nausea, vomiting, bloating, and upper abdominal pain. Motility treatment along with symptom management can be done using antiemetics or prokinetics. This study highlights the diagnostic and therapeutic challenges of gastroparesis and suggests a potential link between facial trauma and symptom remission, indicating the need for further investigation. CASE PRESENTATION: A 46-year-old Hispanic man with hypertension, type 2 diabetes (T2D), and hyperlipidemia on amlodipine 10 mg, lisinopril 5 mg, empagliflozin 25 mg, and insulin glargine presented with a diabetic foot ulcer with probable osteomyelitis. During hospitalization, the patient developed severe nausea and vomiting. The gastroenterology team advised continuing antiemetic medicine and trying very small sips of clear liquids. However, the patient didn't improve. Therefore, the gastroenterology team was contacted again. They advised having stomach emptying tests to rule out gastroparesis as the source of emesis. In addition, they recommended continuing metoclopramide, and starting erythromycin due to inadequate improvement. Studies found a 748-min stomach emptying time. Normal is 45-90 min. An uneventful upper GI scope was done. Severe gastroparesis was verified, and the gastroenterology team advised a percutaneous jejunostomy or gastric pacemaker for gastroparesis. Unfortunately, the patient suffered a mechanical fall resulting in facial trauma. After the fall, the patient's nausea eased, and emesis stopped. He passed an oral liquids trial after discontinuation of erythromycin and metoclopramide. CONCLUSION: This case exemplifies the difficulties in diagnosing and treating gastroparesis. An interesting correlation between parasympathetic surges and recovery in gastroparesis may be suggested by the surprising remission of symptoms following face injuries.

Sudden-Onset Back Pain and Intermittent Dyspnea While Eating: A Case of Pediatric Achalasia.

A 14-year-old girl with a history of asthma was hospitalized because of sudden-onset back pain around her thoracic region that spread to her chest and abdomen. She had been experiencing dysphagia and breathing difficulties for two years, especially after overeating, which often resulted in vomiting undigested food. CT imaging revealed a severely dilated esophagus narrowing at the gastroesophageal junction, suggestive of type 1 achalasia. Further testing confirmed the diagnosis, with an esophageal manometry showing a lack of esophageal contractions and sphincter relaxation. She then underwent a laparoscopic Heller myotomy with relief to her symptoms. This case underscores the rarity of pediatric-onset achalasia with significant esophageal dilation and secondary airway compression, presenting with unusual musculoskeletal and respiratory symptoms. Timely diagnosis and treatment are crucial to prevent worsening and complications.

Oesophageal fluoroscopy in adults-when and why?

Oesophageal fluoroscopy is a radiological procedure that uses dynamic recording of the swallowing process to evaluate morphology and function simultaneously, a characteristic not found in other clinical tests. It enables a comprehensive evaluation of the entire upper gastrointestinal tract, from the oropharynx to oesophagogastric bolus transport. The number of fluoroscopies of the oesophagus and the oropharynx has increased in recent decades, while the overall use of gastrointestinal fluoroscopic examinations has declined. Radiologists performing fluoroscopies need a good understanding of the appropriate clinical questions and the methodological advantages and limitations to adjust the examination to the patient's symptoms and clinical situation. This review provides an overview of the indications for oesophageal fluoroscopy and the various pathologies it can identify, ranging from motility disorders to structural abnormalities and assessment in the pre- and postoperative care. The strengths and weaknesses of this modality and its future role within different clinical scenarios in the adult population are discussed. We conclude that oesophageal fluoroscopy remains a valuable tool in diagnostic radiology for the evaluation of oesophageal disorders.

Delayed Presentation of Malignancy-Associated Pseudoachalasia of the Gastric Cardia.

Pseudoachalasia is a condition in which symptoms, manometry, and imaging findings highly resemble primary achalasia but has a secondary etiology. The majority of patients with pseudoachalasia have the condition as the result of a malignancy, most often at the gastroesophageal junction. There may be issues with timely identification of this malignancy as symptoms are often obscure with diagnostic testing yielding nonspecific results. We describe a case of a 65-year-old diabetic female smoker with a four-month history of intractable vomiting, abdominal pain, and weight loss who was belatedly found to have an adenocarcinoma at the gastric cardia necessitating a total gastrectomy and chemotherapy administration. The case educates clinicians on the clinical alarm symptoms related to malignant pseudoachalasia and stresses the paramount importance of performing a timely esophagogastroduodenoscopy in all cases of achalasia, even with seemingly normal imaging, to rule out pseudoachalasia related to malignancy.

The Functional Implications of Transanal Irrigation: Insights from Pathophysiology and Clinical Studies of Neurogenic Bowel Dysfunction.

Defecation function is negatively impacted in patients with neurogenic bowel dysfunction (NBD), who require effective bowel care for stool evacuation. NBD comprises fecal incontinence and/or constipation, which can reduce the quality of life and dignity. Transanal irrigation (TAI) is recommended by several clinical guidelines as the second-line treatment after conservative treatment and before surgical options are considered. As the only class in the second-line treatment with an established safety and efficacy profile, the mechanism of action of TAI has not fully been elucidated when administered through a rectal catheter with a balloon. This review examines the current understanding regarding the mechanism of action of TAI, with a focus on the pathophysiology of neurogenic bowel and irregular defecation. By understanding the functional implications of TAI, clinicians may be better able to integrate this modality into bowel care programs, especially for patients with NBD who have significant constipation due to delayed colonic motility and impaired stool emptying with loss of voluntary control of defecation, and those who are unresponsive to conservative treatment, including enemas.

Comprehensive Assessment of Esophageal Disorders Associated with Hiatus Hernia: Insights from Big Data Analysis.

Hiatus hernia (HH) is a prevalent endoscopic finding in clinical practice, frequently co-occurring with esophageal disorders, yet the prevalence and degree of association remain uncertain. We aim to investigate HH's frequency and its suspected association with esophageal disorders. We reviewed endoscopic reports of over 75,000 consecutive patients who underwent gastroscopy over 12 years in two referral centers. HH was endoscopically diagnosed. We derived data on clinical presentation and a comprehensive assessment of benign and malignant esophageal pathologies. We performed multiple regression models to identify esophageal sequela associated with HH. The overall frequency of HH was (16.8%); the majority (89.5%) had small HHs (<3 cm). Female predominance was documented in HH patients, who were significantly older than controls (61.1±16.5 vs. 52.7±20.0; P < 0.001). The outcome analysis of esophageal pathology revealed an independent association between HH, regardless of its size, and erosive reflux esophagitis (25.7% vs. 6.2%; OR = 3.8; P < 0.001) and Barrett's esophagus (3.8% vs. 0.7%; OR = 4.7, P < 0.001). Furthermore, following rigorous age and sex matching, in conjunction with additional multivariable analyses, large HHs were associated with higher rates of benign esophageal strictures (3.6% vs. 0.3%; P < 0.001), Mallory Weiss syndrome (3.6% vs. 2.1%; P = 0.01), and incidents of food impactions (0.9% vs. 0.2%; P = 0.014). In contrast, a lower rate of achalasia was noted among this cohort (0.55% vs. 0%; P = 0.046). Besides reflux-related esophageal disorders, we outlined an association with multiple benign esophageal disorders, particularly in patients with large HHs.

Brief guidelines for beginners on how to perform and analyze esophageal high-resolution manometry.

High-resolution manometry (HRM) is a diagnostic tool for surgeons, gastroenterologists and other healthcare professionals to evaluate esophageal physiology. The Chicago Classification (CC) system is based on a consensus of worldwide experts to minimize ambiguity in HRM data acquisition and diagnosis of esophageal motility disorders. The most updated version, CCv4.0, was published in 2021; however, it does not provide step-by-step guidelines (i.e., for beginners) on how to assess the most important HRM metrics. This paper aims to summarize the basic guidelines for conducting a high-quality HRM study including data acquisition and interpretation, based on CCv4.0, using Manoview ESO analysis software, version 3.3 (Medtronic, Minneapolis, MN).

Dysphagia associated with esophageal wall thickening in patients with nonspecific high-resolution manometry findings: Understanding motility beyond the Chicago classification version 4.0.

BACKGROUND: Previous studies have demonstrated that 50% of patients with normal high-resolution manometry (HRM) findings or ineffective esophageal motility (IEM) may have abnormal functional luminal imaging probe (FLIP) results. However, the specific HRM findings associated with abnormal FLIP results are unknown. Herein, we investigated the relationship between nonspecific manometry findings and abnormal FLIP results. METHODS: We retrospectively analyzed 684 patients who underwent HRM at a tertiary care center in Seoul, Korea, based on the Chicago Classification version 4.0 protocol. KEY RESULTS: Among the 684 patients, 398 had normal HRM findings or IEM. Of these 398 patients, eight showed esophageal wall thickening on endoscopic ultrasonography or computed tomography; however, no abnormalities were seen during esophagogastroduodenoscopy. Among these eight patients, seven showed repetitive simultaneous contractions (RSCs) in at least one of the two positions: 61% (±29%) in 10 swallows in the supine position and 51% (±30%) in five swallows in the upright position. Four patients who underwent FLIP had a significantly decreased esophagogastric junction distensibility index (1.0 ± 0.5 m m 2 mmHg - 1 at 60 mL). Two of these patients underwent per-oral endoscopic myotomy (POEM) due to a lack of response to medication. Esophageal muscle biopsy revealed hypertrophic muscle with marginal eosinophil infiltration. CONCLUSIONS & INFERENCES: A subset of patients (2%) with normal HRM findings or IEM and RSCs experienced dysphagia associated with poor distensibility of the thickened esophageal wall. FLIP assessment or combined HRM and impedance protocols may help better define these patients who may respond well to POEM.

Natural history of symptoms and prognostic information of the rapid drink challenge and solid bolus swallows in esophagogastric junction outflow obstruction defined by manometry.

BACKGROUND/INTRODUCTION: Esophagogastric junction outflow obstruction (EGJOO) is a condition characterized by poor relaxation of the lower esophageal sphincter (LES), which can manifest as dysphagia and chest pain. The best treatment of EGJOO is unknown as some patients improve without any specific therapy, whereas some patients undergo invasive therapy. Currently, prognostic factors are lacking. We aimed to assess the long-term prognosis and predictors of dysphagia and chest pain by the rapid drink challenge and solid bolus swallows in EGJOO. METHODS: We retrospectively assessed high-resolution esophageal manometries (HRM) performed at our center between 2015 and 2018. The patients completed a dysphagia and chest pain questionnaire a median of 34 months after the HRM/baseline assessment, including the Impaction dysphagia questionnaire-10 (IDQ-10) complemented with questions regarding chest pain and esophageal treatments. Symptoms were compared with HRM findings. RESULTS: In all, 980 HRMs were analyzed and 66 (6.5%) were identified as having HRM findings compatible with EGJOO. Of these, 27 patients with EGJOO (41%) completed the follow-up questionnaires and had no exclusion criteria, and 70% of these patients had dysphagia and 44% chest pain at least once a week. Dysphagia at follow-up was more common in patients with elevated integrated relaxation pressure (IRP) on all three HRM metrics (water swallows, solid bolus swallows, and rapid drink challenge) (p = 0.03, odds ratio: 8.4 (95% CI: 1.2-56.0)), but this was not seen for chest pain (p = 0.45). Abnormal motility patterns on rapid drink challenge or solid bolus swallows were not associated with dysphagia or chest pain at follow-up. CONCLUSIONS: Having a high IRP on three HRM metrics-water swallows, solid bolus swallows, and rapid drink challenge-is associated with a worse prognosis in patients with EGJOO and could potentially be used to select candidates suitable for invasive procedures.

Characteristics of Esophageal Motility and Associated Symptom Profiles in Patients with Esophageal Diverticulum: A Study Based on High-Resolution Impedance Manometry.

BACKGROUND: Esophageal diverticulum (ED) is an uncommon structural disorder with heterogenous manifestations and elusive pathophysiology. Our aim was to investigate esophageal motility and associated symptom profiles in patients with ED based on high-resolution impedance manometry (HRIM). METHODS: Consecutive patients with ED referred to our motility laboratory between 2015 to 2022 were identified in our electronic database. All patients were evaluated based on an upper endoscopy, HRIM, and standardized symptom questionnaires. Patients with ED were further stratified into upper, middle, and lower (epiphrenic) cases. Esophageal motility was evaluated with HRIM and the updated Chicago Classification v4.0. RESULTS: Twenty-four patients with ED (9 upper, 4 middle, and 11 epiphrenic) were analyzed. Patients with ED were generally older (mean: 65 ± 13.3 years) and predominantly women (58.3%). Most ED cases were unilaterally located (95.8%) and left-side predominant (62.5%). Mean symptom duration was 20 months (range: 1-120) and the most common symptoms were dysphagia (70.8%) and regurgitation (37.5%). Erosive esophagitis was noted in 16 patients (69.6%), while barium stasis was noted in 5 patients (20.8%). Fourteen patients (58.3%) were diagnosed with esophageal motility disorders using HRIM, with achalasia being the most common diagnosis (n = 5, 20.8%). Patients with epiphrenic diverticulum had significantly higher symptom scores and achalasia prevalence. CONCLUSION: Patients with ED tended to be older and was associated with a high prevalence of EMD. A multi-disciplinary evaluation, including complete anatomical and motility surveys, may help clarify the underlying pathophysiology and tailor further treatment strategies.

The spectrum of gastrointestinal functional bowel disorders in joint hypermobility syndrome and in an academic referral center.

Joint hypermobility syndrome (JHS) is a non-inflammatory hereditary disorder of connective tissue with varied clinical presentations, including frequent joint dislocations, hyperextensible skin, easy bruising, and abnormal paper-thin scar formation. Many of these patients have unexplained gastrointestinal (GI) symptoms. Our aim was to evaluate the prevalence of JHS in a tertiary gastroenterology motility clinic and the spectrum of functional bowel disorders in JHS patients. In this retrospective case series, we screened the medical records of 277 patients seen over 4 years at an academic GI Motility Center. The patients who met the criteria for JHS by Beighton hypermobility score were evaluated for the presence of functional GI disorders by Rome IV criteria. They also underwent gastric emptying study and glucose breath testing for small intestinal bacterial overgrowth. The prevalence of JHS in the study population was 9.7%. The mean age was 27 years, and 92.5% were female. The symptoms experienced by these patients include nausea/vomiting (89%), abdominal pain (70%), constipation (48%), and bloating (18.5%). The disorders associated with JHS include gastroparesis (52%), irritable bowel syndrome (55.5%), and gastroesophageal reflux disease (30%). Also, 10 patients (37%) were diagnosed with postural hypotension tachycardia syndrome secondary to autonomic dysfunction. Approximately 10% of patients with suspected functional bowel disorders have hypermobility syndrome. Hence, it is crucial to familiarize gastrointestinal practitioners with the criteria utilized to diagnose JHS and the methods to identify physical examination findings related to this condition.

Hood attached vs conventional triangular tip-jet knife in peroral endoscopic myotomy: a propensity score matching cohort study.

BACKGROUND & AIM: Peroral endoscopic myotomy (POEM) is a safe and effective endoscopic treatment for achalasia and other esophageal motility disorders, and TTJ (Triangle Tip Knife J; Olympus, Tokyo, Japan) is currently widely used in POEM. Recently, we reported a novel modification of TTJ, which was adjusted to knife length 2 mm by attaching a disposable clip (QuickClip Pro; Olympus) sheath to the tip as a hood attachment. In this study, we compared the safety and effectiveness of TTJ and TTJ with hood attachment (TTJ-H) in POEM. METHODS: In this 1:1 propensity score matched retrospective cohort study, we compared the procedure time, myotomy efficiency, number of coagulation forceps usage, adverse events, length of hospital stay after POEM, procedural success and clinical success between TTJ and TTJ-H groups. RESULTS: We examined 682 consecutive patients who underwent POEM between January 2021 and June 2023. We excluded 134 patients who had already undergone POEM or laparoscopic Heller myotomy as prior myotomy. Finally, we identified 98 propensity score-matched pairs (n = 196). The mean procedure time was shortened from 93.5 to 80.2 min (14% reduction, P = 0.012) when comparing TTJ-H group to TTJ group. The mean myotomy efficiency was improved from 2.76 to 2.32 min/cm (16% improvement, P = <0.001), and usage of coagulation forceps for hemostasis was decreased from 3.87 to 0.55 (86% reduction, P = <0.001). CONCLUSIONS: This study showed that use of TTJ-H could reduce total procedure time, improve myotomy efficiency, and reduce costs compared to TTJ.

What to expect from primary inferior oblique overaction after esotropia surgery : Primary inferior oblique overaction in esotropia.

BACKGROUND: Overelevation in adduction is common in patients with primary esotropia. This study evaluates the variation in ocular motility pattern in patients with primary inferior oblique (IO) muscle overaction after esotropia surgery. METHODS: The medical records of consecutive patients who underwent surgery for infantile, partially accommodative, and basic esotropia over eleven years and had at least one year of follow-up were reviewed. Patients with primary inferior oblique muscle overaction (IOOA) presented at baseline or during follow-up were selected and divided according to the first surgery performed concurrently with horizontal rectus surgery: without IO recession (NO-recess), with unilateral IO recession (UNIL-recess), and with bilateral IO recession (BIL-recess). The success (version normalisation or at least 2 points upgrade in severity scale [0-4] in the operated eye), recurrence rates, and the evolution of the non-operated IO muscles were evaluated. RESULTS: One hundred and ten patients were included - 53 NO-recess, 26 UNIL-recess, and 31 BIL-recess. Medial rectus muscle posterior fixation sutures surgery (PFS) was performed in 88.2% of patients for esotropia. A recession with graded anterior transposition was the weakening IO procedure. In the NO-recess group, 28 (52.8%) patients normalised their mild IOOA after PFS surgery alone. In the UNI-recess group, the success rate was 88.5%, with 16 (61.5%) patients showing worsened IO muscle of the fellow eye, which prompted additional surgery in 10 patients. In the BIL-recess group, all 31 patients improved the adduction pattern of the operated eye for an 80.6% success rate (6 improved marginally). CONCLUSION: Graded anterior transposition of the inferior oblique muscle effectively normalises versions. However, it's frequent for a contralateral overaction to become manifest after unilateral IO surgery.

Noninvasive electrical neuromodulation for gastrointestinal motility disorders.

INTRODUCTION: Gastrointestinal motility disorders are highly prevalent without satisfactory treatment. noninvasive electrical neuromodulation is an emerging therapy for treating various gastrointestinal motility disorders. AREAS COVERED: In this review, several emerging noninvasive neuromodulation methods are introduced, including transcutaneous auricular vagal nerve stimulation, percutaneous auricular vagal nerve stimulation, transcutaneous cervical vagal nerve stimulation, transcutaneous electrical acustimulation, transabdominal interference stimulation, tibial nerve stimulation, and translumbosacral neuromodulation therapy. Their clinical applications in the most common gastrointestinal motility are discussed, including gastroesophageal reflux disease, functional dyspepsia, gastroparesis, functional constipation, irritable bowel syndrome, and fecal incontinence. PubMed database was searched from 1995 to June 2023 for relevant articles in English. EXPERT OPINION: Noninvasive neuromodulation is effective and safe in improving both gastrointestinal symptoms and dysmotility; it can be used when pharmacotherapy is ineffective. Future directions include refining the methodology, improving device development and understanding mechanisms of action.

Comparison of the efficacy of diltiazem versus fluoxetine in the treatment of distal esophageal spasm: A randomized-controlled-trial.

BACKGROUND AND STUDY AIM: Distal esophageal spasm is an uncommon esophageal motility disorder presenting with non-cardiac chest pain and dysphagia. The main goal of therapy is symptom relief with pharmacologic, endoscopic, and surgical therapies. Pharmacologic treatment is less invasive and is the preferred method of choice. The purpose of this study was to compare the effectiveness of diltiazem versus fluoxetine in the treatment of distal esophageal spasm. PATIENTS AND METHODS: A total of 125 patients with distal esophageal spasm diagnosed using endoscopy, barium esophagogram, and manometry were evaluated. Patients were divided into diltiazem and fluoxetine groups and received a 2-month trial of diltiazem + omeprazole or fluoxetine + omeprazole, respectively. Of 125 patients, 55 were lost to follow up and 70 were eligible for final analysis. Clinical signs and symptoms were assessed before and after therapy using four validated questionnaires: Eckardt score, short form-36, heartburn score, and the hospital anxiety and depression scale. RESULTS: Both regimens significantly relieved symptoms (a decrease in mean Eckardt score of 2.57 and 3.18 for diltiazem and fluoxetine groups, respectively; and a decrease in mean heartburn score by 0.89 and 1.03 for diltiazem and fluoxetine groups, respectively). Patients' quality of life improved based on short form-36 (an increase in mean score of 2.37 and 3.95 for fluoxetine and diltiazem groups, respectively). There was no relationship between patients' improvement and severity of symptoms. Psychological findings based on the hospital anxiety and depression scale were inconsistent (a decrease in mean of 0.143 and 0.57 for fluoxetine and diltiazem groups, respectively; p > 0.05). CONCLUSION: Fluoxetine and diltiazem were effective for clinical symptom relief in patients with distal esophageal spasm, but were not promising for improving psychological symptoms. Neither regimen was superior in terms of efficacy. Consequently, it is key to consider side effects and comorbidities when choosing a therapy.

Present state of intestinal transplantation in Japan.

INTRODUCTION: Intestinal transplantation (ITx) is the ultimate treatment for intestinal failure (IF). In Japan, most cases of IF are a result of pediatric disease, including secondary or congenital intestinal disease or allied disorders of Hirschsprung's disease. Here, we report the results of the Japanese ITx registry. METHODS: A web-based survey form was completed. We investigated the number, age, sex, indication, surgical procedure, immunosuppressants, postoperative course, and the effects of transplantation in patients who underwent cadaveric or living-donor ITx. RESULTS: By the end of 2022, 42 cases of ITx have been performed in 38 patients in Japan. The donor sources included cadavers (29 cases) and living donors (13 cases). The surgical method was isolated ITx (N = 40) and combined liver and ITx (n = 2). Survival rates were 92%, 73%, and 59% at 1 year, 5 years, and 10 years, respectively. Ninety percent of patients completely discontinued parenteral nutrition. Approximately 80% of the patients had a performance status of 1 or less, indicating that the QOL of patients after ITx was extremely good. CONCLUSION: The results of ITx are acceptable to treat IF patients and the QOL after transplantation is also good.