Laser therapy and light sources for labial lentigines in patients with Peutz-Jeghers syndrome.

Labial lentigines associated with Peutz-Jeghers syndrome are challenging and represent a cosmetic problem. Laser and intense-pulsed light sources (IPLS) can be used to treat these macules. However, there are few guidelines regarding the different types of protocols found in the literature. Thus, the purpose of this study was to review the pertinent literature on lasers and IPLS as therapy for labial lentigines in patients with Peutz-Jeghers syndrome. A detailed literature search was conducted in seven databases (Cochrane Library, Embase, Lilacs, PubMed, SciELO, Scopus and Web of Science) to November 2020. Data were extracted and analyzed from selected studies including study design, sample size, participants' skin color, age, and gender, parameters of the laser or IPLS used, interval and total sessions of laser application, results obtained, follow-up time and side effects. Thirteen out of 124 published studies met our eligibility criteria, covering 81 patients. Ten types of lasers and IPLS were used, and most of the sample (n = 57) was treated with Q-switch Alexandrite Laser (QSAL). The total number of sessions ranged from 1 to 12 and the interval between sessions ranged from 2 to 16 weeks. All studies have shown satisfactory results, with no further complications. The follow-up ranged from 2 to 97 months. Laser therapy and IPLS are effective in the treatment of lentigines associated with PJS. Although QSAL was used in 70.3% of the sample, different types of lasers showed comparable satisfactory results.

Giant rectal polyp prolapse in an adult patient with the Peutz-Jeghers syndrome.

Peutz-Jeghers Syndrome (PJS) is an autosomal dominant intestinal polyposis syndrome characterised by the presence of hamartomatous polyps and mucocutaneous pigmentation. Prolapse of the polyps through the anus is an infrequent manifestation in children with PJS, and this complication is extremely rare in adult patients. We report the case of a 30-year-old man recently diagnosed with PJS who was seen at the emergency department because of the abrupt onset of severe anal pain with a foreign body sensation in the anal canal and rectal bleeding.Physical examination revealed a giant prolapsed polyp.

Risk factors for surgery in pediatric intussusception in the era of pneumatic reduction.

INTRODUCTION: Surgical treatment is still necessary for intussusception management in a subgroup of patients, despite advances in enema reduction techniques. Early identification of these patients should improve outcomes. METHODS: The medical records of patients treated for intussusception at our institution from 2006 to 2011 were reviewed. Univariate and multivariate analyses, including stepwise logistic regression, were performed. RESULTS: Overall, 379 patients were treated for intussusception, and 101 (26%) patients required operative management, with 34 undergoing intestinal resection. The post-operative complication rate was 8%. On multivariate analysis, failure of initial reduction (OR 9.9,p=0.001 95% CI, 4.6-21.2), a lead point (OR 18.5,p=0.001 95% CI, 6.6-51.8) or free/interloop fluid (OR 3.3,p=0.001 95% CI, 1.6-6.7) or bowel wall thickening on ultrasound (OR 3.3,p=0.001 95% CI, 1.1-10.1), age <1 year at reduction (OR 2.7,p=0.004, 95% CI, 1.4-5.9), and abdominal symptoms>2 days (OR 2.9,p=0.003, 95% CI, 1.4-5.9) were significantly associated with a requirement for surgery. Similarly, a lead point (OR 14.5, p=0.005 95% CI, 2.3-90.9) or free/interloop fluid on ultrasound (OR 19.8, p=0.001 95% CI, 3.4-117) and fever (OR 7.2, p=0.023 95% CI, 1.1-46) were significantly associated with the need for intestinal resection. CONCLUSION: Abdominal symptoms>2 days, age<1 year, multiple ultrasound findings, and failure of initial enema reduction are significant predictors of operative treatment for intussusception. Patients with these findings should be considered for early surgical consultation or transfer to a hospital with pediatric surgical capabilities.

Peutz-Jeghers syndrome: data from the Singapore Polyposis Registry and a shifting paradigm in management.

INTRODUCTION: Peutz-Jeghers Syndrome (PJS) is an uncommon autosomal dominant hamartomatous polyposis syndrome. Morbidity arises from polyp-related complications and increased risks of malignancy. We report on PJS patients registered in the Singapore Polyposis Registry, identified principal causes of morbidity and appraised current management strategies. A followup protocol based on recent literature has been proposed. MATERIALS AND METHODS: A search of a prospectively collected database in the Singapore Polyposis Registry was made. Only patients who fulfilled the diagnostic criteria of PJS were included. The clinical records were retrieved for review. Information on affected family members was obtained from the Registry's pedigree records. RESULTS: Seven unrelated patients fulfilled the criteria of having PJS. Principal causes of morbidity include recurrent bouts of abdominal colic, episodes of intestinal obstruction, gastrointestinal bleeding and the need for repeated laparotomies. Six out of 7 patients had initial presentation with acute intestinal obstruction requiring emergency laparotomy. Management was mostly problem-oriented and marked inter-surgeon variation with regard to cancer screening and genetic counselling was observed. CONCLUSION: Patients with PJS suffer gastrointestinal complications from polyposis and are at increased risks for developing cancers. A move towards surveillance and planned comprehensive care may reduce the morbidity of the condition. A protocol driven approach conducted in the setting of a Polyposis Registry is ideally suited to facilitate such care.

Q-switched alexandrite laser treatment of oral labial lentigines in Chinese subjects with Peutz-Jeghers syndrome.

BACKGROUND: Peutz-Jeghers syndrome (PJS) is characterized by gastrointestinal hamartomatous polyps and visible mucocutaneous lentigines distributed in the oral and anal mucosa, hands, face, and nails. Although there are many reports regarding successful treatment of intestinal polyps of PJS, there is little information regarding treatment of lentigines. Q-switched lasers are the preferred method of treatment for benign melanocytic lesions. OBJECTIVE: To evaluate the efficacy of the Q-switched alexandrite laser (QSAL) in treating oral labial lentigines of PJS. METHODS AND MATERIALS: Fourteen cases of laser treatment of oral labial lentigines in patients diagnosed with PJS were studied between 2005 and 2008. All were treated using a single treatment with a QSAL with a 3-mm handpiece and a fluence of 4.0 to 9.0 J/cm(2). Retrospective analysis of the effect of QSAL was conducted. RESULTS: All 14 cases exhibited successful elimination of treated lentigines after one laser treatment. There were significant acute side effects. After a median 2-year follow-up, there were no recurrences or scars in treated sites. CONCLUSIONS: The QSAL provides effective treatment for removal of labial lentigines associated with PJS.

Clinical spectrum and surgical approach of adult intussusceptions: a multicentric study.

BACKGROUND: The preoperative diagnosis of adult intussusceptions (AIs) remains difficult, and the assessment of the radiological methods has been evaluated very little in the literature. The aim of this study was to evaluate the interest of the different imaging modalities for the preoperative diagnosis of AI and describe causes of AI. PATIENTS AND METHODS: Consecutive patients of 15 years and older with the postoperative diagnosis of intussusception from 1979 to 2004 were reviewed retrospectively for this multicentric study. Data concerning clinical considerations, morphological examinations, surgical procedure, histological conclusions, mortality rate and recurrence were analysed. RESULTS: Forty-four patients with documented intussusception were included. The mean age was 51 years (15-93 years). The preoperative diagnosis of intussusception was made in 52% of the cases. The sensitivities of the different radiological methods were abdominal ultrasounds (35%), upper gastrointestinal barium study (33%), abdominal computed tomography (CT) (58%) and barium enema (73%). An organic lesion was identified in 95% of the cases. There was 29 enteric and 15 colonic (including appendicular) intussusceptions. Thirty-seven percent of the enteric lesions were malignant, and a bit less than 50% of them were metastatic melanomas. The benign enteric lesions were Meckel's diverticulum and Peutz-Jeghers syndrome in half of the cases. Fifty-eight percent of the pure colonic lesions (excluding appendix) were malignant, and 85% of them were primary adenocarcinomas. The benign colonic lesions were lipomas in 80% of the cases. All patients, except one, had a surgical treatment, and 13 of them had a complete reduction of the intussusception before resection. The mortality rate was 16% and recurrence occurred in three patients; two of them had a Peutz-Jeghers syndrome. CONCLUSION: Intussusception rarely occurs in adults, but nearly half of their causes are malignant. The CT scan is a helpful examination for enteric intussusceptions whether barium enema seems to be the most performing method for colonic lesions. Surgery is the recommended treatment, with or without a primary reduction of the intussusception. During the surgical procedure, this reduction can lead to a more limited bowel resection.

Colo-colonic intussusception caused by a solitary Peutz-Jeghers polyp.

A case of acute large bowel obstruction by colo-colonic intussusception in a healthy 19-year-old man is presented. The lead point of the intussusception is a rare solitary colonic Peutz-Jeghers polyp in the descending colon of a man without the full Peutz-Jeghers syndrome. The clinical presentation, imaging findings on plain radiographs, single contrast enema and CT, and findings at colonoscopy and surgery are correlated with pathology results and a brief review of the literature.

Intussusception of the small bowel due to Peutz-Jeghers syndrome: a case report.

The Peutz-Jeghers syndrome is an autosomal dominant disorder characterized by hamartomatous polyposis of the gastrointestinal tract, melanin pigmentation of the skin and mucous membranes, and an increased risk for cancer. The incidence of surgical complications in these patients is relatively rare, and correlates with the size and location of the polyps. Herein we report the case of a 27-year-old woman presented with episodes of abdominal pain, abdominal distention and intermittent vomiting. Moreover, multiple pigmentation of the mouth was also noted. A preoperative diagnosis of a double jejunal intussusception and jejunal occlusion was based on the findings of small bowel enema and computed tomography. The diagnosis was confirmed at laparotomy.

[Small intestinal polyposis in Peutz-Jeghers syndrome: combined treatment with surgery and intra-operative endoscopy]. / La poliposi del tenue nella sindrome di Peutz-Jeghers: trattamento combinato chirurgia-endoscopia intraoperatoria.

Polyps occur throughout the gastrointestinal tract in Peutz-Jeghers syndrome, but the most serious problems are encountered in the management of small bowel polyposis. We report here on a case of Peutz-Jeghers syndrome admitted to hospital for intestinal obstruction and anaemia. The patient was submitted to colonoscopy, oesophagogastro-duodenoscopy and small bowel enema. At laparotomy, multiple intussusceptions were found and we conducted a combined surgical-endoscopic approach. Most of the polyps were identified and removed endoscopically (snare polypectomy). Five enterotomies were performed to remove 18 very large polyps (> 3 cm). Finally, a limited portion of the jejunal tract (20 cm) was resected owing to the presence of multiple, large, obstructive polyps. None of the polyps showed cancerous transformation. The shortcomings of the traditional surgical approach include repeated small bowel resections and often early reoperation to manage complications caused by polyps missed at the time of previous surgery. If surgical intervention is required, intraoperative endoscopy is always indicated. Conservative surgical management, the role of intraoperative endoscopy, planned medical follow-up and the need for a national registration system are stressed.

The impact of imaging in the management of intussusception owing to pathologic lead points in children. A review of 43 cases.

OBJECTIVE: To review the imaging appearances, management and outcome of a large number of children with intussusception owing to pathologic lead points (PLP) in an attempt to define the role of various imaging modalities in this clinical setting. MATERIALS AND METHODS: Review of the records and imaging studies of 43 children with intussusception due to PLP diagnosed between 1986 and 1999. RESULTS: The commonest PLP found were Meckel diverticulum, polyps, Henoch-Schonlein purpura and cystic fibrosis. PLP were depicted on sonography in 23 (66%) of 35 patients, on computed tomography in 5 (71%) of 7, on air enema in 3 (11%) of 28, and on barium enema in 6 (40%) of 15. Air enema successfully reduced 60% of the intussusceptions. Nine children had recurrent intussusceptions. CONCLUSION: Sonography depicted two-thirds of PLP and provided a specific diagnosis in nearly one-third of our series. Our review does not provide sufficient data on how to continue the investigation of those patients in whom sonography does not depict a PLP but in whom there is a high index of suspicion for its presence. It remains a diagnostic challenge as to how to search for PLP in these patients, and other imaging modalities have to be requested according to each particular case.

Intussusception on small bowel examinations in children.

Six children in whom an intussusception was diagnosed on a small bowel follow-through examination are presented. The radiographic signs of intussusception on such a study include: (1) a narrow channel of barium representing the compressed lumen of the intussusceptum, (2) a soft-tissue mass on either side of this channel due to hypertrophy and edema of the walls of the intussuceptum and intussuscipiens, (3) a coil spring appearance around the narrow channel, and (4) a mass lesion at the distal end of the narrow channel. Not all of these signs are present in each case. Intussusception is a dynamic process and the value of delayed films and frequent fluoroscopy during small bowel follow-through studies in these cases is stressed. All six cases had a demonstrable lesion as a lead point. Jejunal intussusceptions are usually caused by bening lesions--malignancy being found almost exclusively in distal intussusceptions.

[Angiographic diagnosis and therapy of acute and chronic gastrointestinal hemorrhages]. / Angiographische Diagnostik und Therapie der akuten und chronischen gastrointestinalen Blutung.

In acute gastrointestinal bleeding visceral angiography has been showing its importance for years. It contributes to diagnosis especially in cases with persistent acute hemorrhage. In chronic gastrointestinal bleeding conventional radiographic procedures such as upper gastrointestinal series and barium enema will be preferred to angiography. The function of the radiologist goes beyond mere diagnosis of gastrointestinal bleeding. Treatment with vasopressin via the angiographic catheter has proven its clinical value. This method will be indicated especially in cases with high risk anesthesia and surgery. It will help to postpone necessary surgery to a more favorable moment following hemostasis. Side effects such as hypertension and antidiuresis are relatively rare and easy to manage. Numerous substances are used for embolization showing that ideal material has not been found yet and further development seems necessary. In contrast to vasopressin treatment, vascular occlusion is often irreversible, complications (unwanted reflux of embolization material, necrosis and plugging of the catheter) are more difficult to manage. Superselective visualization of a bleeding artery is always needed. Embolization is justified in cases when a possibility for anesthesia and surgery cannot be foreseen. The electrical vascular occlusion using direct current is still in the phase of animal experiments; its clinical value has not sufficiently been assessed as yet.