Peutz-Jeghers Syndrome: Lessons to be Learned in the Clinical Diagnosis.

Introduction: Peutz-Jeghers Syndrome (PJS) is an autosomal dominant disease presenting with hamartomatous polyps in the gastrointestinal tract and hyperpigmented macules on lips and oral mucosa. The incidence of this syndrome is approximately 1 in 1,20,000 births. Materials and Methods: In this article, we are presenting 11 cases of PJS which were misdiagnosed and patients were compelled to visit hospital repeatedly. All these cases were diagnosed based on clinical suspicion, family history, and histopathological examination of specimens. Most of the cases presented with intussusception and required emergency surgical management. Results: PJS can be diagnosed by the presence of microscopically confirmed hamartomatous polyps and a minimum of two of the following clinical criteria: Family history, mucocutaneous melanotic spots, and small bowel polyps with bleeding per rectally. The diagnosis can be missed if the melanotic spots on the face are missed. Routine investigations, imaging, and endoscopy were done in all cases. PJS patients need regular follow-up due to chance of recurrence of symptoms and susceptibility to cancer. Conclusion: PJS needs a high index of suspicion for diagnosis in cases of recurrent abdominal pain with bleeding per rectum. Proper family history and meticulous clinical examination for melanosis are very important to prevent the misdiagnosis of these cases.

Twenty-Four Hour pH Study and Manometry in Gastric Esophageal Substitutes in Children.

PURPOSE: Studies on the physiology of the transposed stomach as an esophageal substitute in the form of a gastric pull-up or a gastric tube in children are limited. We conducted a study of motility and the pH of gastric esophageal substitutes using manometry and 24-hour pH measurements in 10 such patients. METHODS: Manometry and 24 hour pH studies were performed on 10 children aged 24 to 55 months who had undergone gastric esophageal replacement. RESULTS: Six gastric tubes (4, isoperistaltic; 2, reverse gastric tubes) and 4 gastric pull-ups were studied. Two gastric tubes and 4 gastric pull-ups were transhiatal. Four gastric tubes were retrosternal. The mean of the lowest pH at the midpoint of the substitute was 4.0 (range, 2.8-5.0) and in the stomach remaining below the diaphragm was 3.3 (range, 1.9-4.2). In both types of substitute, the difference between the peak and the nadir pH recorded in the intra-thoracic and the sub-diaphragmatic portion of the stomach was statistically significant (p<0.05), with the pH in the portion below the diaphragm being lower. The lowest pH values in the substitute and in the remnant stomach were noted mainly in the evening hours whereas the highest pH was noted mainly in the morning hours. All the cases showed a simultaneous rise in the intra-cavitatory pressure along the substitute while swallowing. CONCLUSION: The study suggested a normal gastric circadian rhythm in the gastric esophageal substitute. Mass contractions occurred in response to swallowing. The substitute may be able to effectively clear contents.

A prospective comparison of topical feracrylum citrate versus adrenaline as haemostatic agent in hypospadias surgery in children.

BACKGROUND: A comparative study of topical feracrylum citrate versus adrenaline to minimise haemorrhage-related complications in paediatric hypospadiac patients. PATIENTS AND METHODS: A total of 108 consecutive paediatric hypospadiac patients (48 in the study group and 60 control - random allocation) were studied. In the study group, 1% feracrylum citrate solution was used and adrenaline (1:100,000) in controls. RESULTS: Among the study group, average number of blood-soaked gauge pieces were 2.95/patient, correlating with average intraoperative blood loss of 14.74 ml. In controls, average blood-soaked gauge pieces were 4.83/patient corresponding to an average blood loss of 24.13 ml. The average amount of blood loss during surgery in the <5 years was 13.70 ml/patient in the feracrylum group, while the same in the adrenaline group was 23.45 ml. Average duration of surgery was 79 min in the study group, while the same in controls was 94 min/patient. Average number of cauterisations was 0.255/patient in the study group and 0.583/patient among controls. Postoperative haematoma was seen in 8% study group compared with 18% controls. Wound oedema appeared in 4.17% study group and 11.67% controls. Postoperative complications were higher among controls. CONCLUSIONS: Feracrylum is more efficient and safer topical haemostatic agent than adrenaline. It reduced the frequency of cauterisation and tissue damage, intraoperative blood loss, and postoperative complications.

Epidemiological trends of pediatric trauma: A single-center study of 791 patients.

AIM: To assess the various epidemiological parameters that influence the causation of trauma as well as the consequent morbidity and mortality in the pediatric age group. MATERIALS AND METHODS: A prospective study of 791 patients of less than 12 years age, was carried out over a period of 1 year (August 2009 to July 2010), and pediatric trauma trends, with regards to the following parameters were assessed: Age group, sex, mode of trauma, type of injury, place where the trauma occurred and the overall mortality as well as mortality. RESULTS: Overall trauma was most common in the school-going age group (6-12 years), with male children outnumbering females in the ratio of 1.9:1. It was observed that orthopedic injuries were the most frequent (37.8%) type of injuries, whereas fall from height (39.4%), road traffic accident (27.8%) and burns (15.2%) were the next most common modes of trauma. Home was found out to be the place where maximum trauma occurred (51.8%). Maximum injuries happened unintentionally (98.4%). Overall mortality was found out to be 6.4% (n = 51). CONCLUSIONS: By knowing the epidemiology of pediatric trauma, we conclude that majority of pediatric injuries are preventable and pediatric epidemiological trends differ from those in adults. Therefore, preventive strategies should be made in pediatric patients on the basis of these epidemiological trends.

Hemostasis during hypospadias surgery via topical application of feracrylum citrate: A randomized prospective study.

AIM: Report of our experience with topical feracrylum citrate to minimize hemorrhage-related complications in pediatric hypospadiac patients. MATERIALS AND METHODS: One hundred and fifty consecutive pediatric hypospadiac patients over 3 years (75 in study group and 75 controls - random allocation) were studied. One hundred milliliter of 1% feracrylum citrate solution was used in study cases and equivalent normal saline in controls. The parameters assessed were frequency of cauterizations, intraoperative blood loss, wound edema and postoperative complications. RESULTS: Average number of cauterizations was 1.55 per patient in study group and 5.7 per patient among controls. Among cases, average number of blood soaked gauge pieces was 3.56 per patient, correlating with average intraoperative blood loss of 17.8 ml. In controls, average blood soaked gauge pieces were 6.2 per patient corresponding to an average blood loss of 31 ml. Postoperative hematoma was seen in 8% cases compared with 18% controls. Wound edema appeared in 13.3% cases and 47% controls. Postoperative complications were higher among controls. CONCLUSIONS: Feracrylum is an effective and safe topical hemostatic agent to minimize significantly diffuse capillary oozing and surface bleeding. It reduced the frequency of cauterization and tissue damage, intraoperative blood loss, postoperative hematoma, wound edema and postoperative complications.