Transoral traumatic perforation of the pyriform sinus by a marker pen: report of an infant case.

BACKGROUND: Perforation of the pyriform sinus, included in hypopharyngeal injury, is a rare condition typically caused by iatrogenic factors. We present a case of an infant who developed deep cervical and mediastinal abscesses due to a traumatic pyriform sinus perforation caused by accidentally falling with a marker pen in the mouth. CASE PRESENTATION: An 11-month-old healthy male infant fell on a trampoline with a marker pen in his mouth. The patient developed swelling in the neck 3 h after the incident and was taken to a regional general hospital. Although a laryngoscopy showed no perforation in the oral cavity or posterior pharynx, a computed tomography (CT) scan revealed significant emphysema extending from the cervix to the mediastinum. The patient was transferred to our tertiary hospital and admitted to the intensive care unit, where he was mechanically ventilated, and antibiotic therapy was initiated. On day 3 of admission, a CT scan revealed deep abscesses in the cervical and upper posterior mediastinum with pneumomediastinum. Although his respiratory status stabilized and he was temporarily weaned, the fever recurred. Pharyngoesopagography revealed significant leakage of contrast from the left pyriform sinus to the mediastinum. Consequently, surgical drainage of the abscess was performed on day 10. Two low-pressure continuous suction drains were placed, one in the posterior mediastinum and the other close to the pyriform sinus. Pharyngoesophagography on postoperative day (POD) 7 demonstrated decreased contrast leakage into the posterior mediastinum. The patient was initiated on enteral nutrition through a nasogastric tube. The patient was discharged on POD 31 after the suction drains were replaced with open Penrose drains, and enteral nutrition via nasogastric tube was continued at home. The Penrose drains were removed on POD 54, and salivary leakage ceased on POD 111. CONCLUSIONS: Although injuries to the oral cavity and posterior pharynx are more easily recognized, the existence of injury in the pyriform sinus can be challenging to evaluate. However, prompt and appropriate management, including intubation, antibiotic therapy, surgical drainage, and nutritional support, is critical in preventing life-threatening complications.

Giant pulmonary bulla causing respiratory compromise in a very low-birthweight infant.

Acquired cystic lung disease in premature infants is a serious respiratory complication, and pulmonary interstitial emphysema (PIE) has been widely reported. We report a rare case of giant pulmonary bulla in an infant treated with bullectomy where chest computed tomography was useful in directing treatment.

Volvulus of the stomach and wandering spleen after repair of congenital diaphragmatic hernia: unexpected manifestations in a neonate.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is sometimes associated with complications involving herniation of intrathoracic organs, which further increase mortality rate. We encountered a case of postoperative gastric and splenic volvulus shortly after left CDH repair in a female neonate who was treated with gastropexy. CASE PRESENTATION: At 39 weeks gestation, a female patient with left Bochdalek CDH was delivered (birth weight: 3748 g, Apgar score: 3/4). The patient was provided ventilator support with nitric oxide. After pulmonary hypertension improved, CDH repair was performed via the abdominal approach on day 7. The stomach, small intestine, large intestine, and spleen were herniated through a diaphragmatic defect of 4 × 2 cm. Although the diaphragm was directly closed, it was tight and the reconstructed diaphragm "dome" was shallow, restricting space for the spleen and stomach. Nonetheless, the spleen was positioned in the left upper abdomen and the stomach was positioned medially. The postoperative course was complicated by organo-axial gastric volvulus, and laparotomy was performed on day 14. In addition to the gastric volvulus, we confirmed a wandering splenic volvulus. The spleen was easily detorted and returned to the left upper abdomen. However, the patient experienced relapse of gastric volvulus without splenic volvulus. Gastropexy was performed electively on day 47. Postoperatively, the patient could be fed orally, and the patient's development was satisfactory 6 years after surgery. CONCLUSIONS: The cause of these rare complications appeared to be tight direct diaphragmatic closure, which reduced space for the spleen and stomach beneath the left diaphragm.

Postoperative hyperthermia-induced multiple organ failure in a child with Down syndrome: a case report.

BACKGROUND: Psychological stress has been reported to cause hyperthermia. Persistent excessive hyperthermia can, in turn, cause hypercytokinemia and organ damage. We report a case of postoperative severe hyperthermia leading to a systemic inflammatory response and multiple organ failure in a child with Down syndrome. CASE PRESENTATION: A 10-month-old native Japanese boy with Down syndrome and Hirschsprung's disease is described. Newborn screening showed congenital hypothyroidism and a ventricular septal defect, but these conditions were stable upon administration of levothyroxine and furosemide. His development was equivalent to that of a child with Down syndrome. He developed a noninfectious high fever twice after preoperative preparations at age 8 months and again at 9 months. He was readmitted to hospital at age 10 months to undergo the Soave procedure to correct Hirschsprung's disease. However, he contracted a fever immediately after the surgical procedure. Hyperthermia (42 °C) was refractory to acetaminophen treatment and deteriorated to multiple organ failure due to hypercytokinemia, with increased serum levels of interleukin-6 (44.6 pg/mL) and interleukin-10 (1010 pg/mL). He died on postoperative day 2 with hypoxemia, respiratory/metabolic acidosis, increased serum levels of transaminases, reduced coagulation, and pancytopenia. Various infectious and noninfectious causes of hyperthermia could not be identified clearly by culture or blood tests. CONCLUSIONS: We speculated that the proximate cause of the fever was psychological stress, because he suffered repeated episodes of hyperthermia after the invasive procedure. Hyperthermia, together with the immune-system disorders associated with Down syndrome, may have induced hypercytokinemia and multiple organ failure. This rare case of noninfectious postoperative hyperthermia leading to multiple organ failure may help to shed further light on the currently unclear pathogenic mechanism of hyperthermia and associated multiple organ failure during the perioperative period in children.

Multiseptate gallbladder coexisting with pancreaticobiliary maljunction treated by laparoscopic cholecystectomy: report of a pediatric case.

BACKGROUND: A multiseptate gallbladder is a very rare congenital malformation in which the lumen is divided into variously sized multiseptal compartments. The pathogenesis and natural history of this disease remain uncertain. We herein describe a pediatric case of a multiseptate gallbladder with pancreaticobiliary maljunction (PBM), which was treated by laparoscopic cholecystectomy. CASE PRESENTATION: A 5-year-old girl was referred to our hospital, because a multiseptate gallbladder had been incidentally detected on abdominal ultrasonography when she presented for transient abdominal pain. Ultrasonography showed hyperechoic septa throughout the lumen of the gallbladder, giving it a honeycomb appearance. The atrophied gallbladder had weak or no contractility. Magnetic resonance cholangiopancreatography performed to detect other coexisting biliary disorders revealed PBM without dilatation of the common bile duct. Although physical examination and laboratory tests revealed no abnormalities, we performed laparoscopic cholecystectomy to prevent cholecystitis and reduce the risk of cancer secondary to the PBM. CONCLUSIONS: In recent pediatric case reports, the indication and timing of cholecystectomy has tended to be determined by the patient's symptoms and the presence of biliary complications. In the present case, however, the combination of a multiseptate gallbladder and PBM may become problematic in the future. Surgical treatment without delay was appropriate even in this pediatric patient.

Congenital communicating bronchopulmonary foregut malformation including ectopic pancreatic tissue in an infant.

BACKGROUND: Several reports have documented that the pulmonary sequestration is in communication with the gastrointestinal tract and the concept of bronchopulmonary foregut malformation (BPFM) has become more widespread. However, there are few reports of the sequestration associated with the pancreas derived from the foregut. We describe the history and pathophysiology of BPFM including pancreatic tissue in a male infant with respiratory distress. CASE PRESENTATION: A male patient was born at 38 weeks of gestation and weighed 2752 g at birth. He developed pneumonia and was hospitalized at 3 months of age. Chest radiographs and CT scans led to the diagnosis of a lung abscess in the left lower intralobar pulmonary sequestration with aberrant arteries from the abdominal cavity. At 4 months of age, when the abscess had resolved, left lower lobectomy and the resection of the intralobar sequestration were performed. The pulmonary sequestration was conjoined with the esophagus. A fistula was found between the lower esophageal wall and the pulmonary sequestration. An additional small segment of the esophageal wall was excised. Histologically, the mediastinal surface of the sequestration tissue contained pancreatic tissue. Furthermore, esophageal and gastric tissue, cartilage tissue, and ciliated epithelium were confirmed. A definitive diagnosis of BPFM was made. CONCLUSIONS: We postulated the rare case of a communicating BPFM with intrapulmonary sequestration on one end and the esophagus on the other forming a mass lesion, which included ectopic pancreatic tissue in a male infant.

Caliceal diverticulum with ureteropelvic junction obstruction in a dysplastic kidney: a pediatric case report.

A caliceal diverticulum is a rare entity in children. Its etiology is closely associated with that of ureteropelvic junction malformations and renal dysplasia. We herein present a case of these complex disorders in an infant. A renal cyst and hydronephrosis were found in the left kidney during the fetal period. The postnatal diagnosis was hydronephrosis due to ureteropelvic junction obstruction and a caliceal diverticulum in the left dysplastic kidney. Although left renal function was severely decreased, the patient had no symptoms. Therefore, we did not perform surgical treatment. At the time of this writing, the patient was 3 years 8 months old and had developed no symptoms.

Non-occlusive intestinal ischemia in the ascending colon and rectum: a pediatric case occurring during encephalitis treatment.

BACKGROUND: Non-occlusive mesenteric ischemia (NOMI) is a rare and severe pathological condition that can cause intestinal necrosis without mechanical obstruction of the mesenteric artery. NOMI often develops during the treatment of severe disease in elderly patients and mostly occurs in the intestine supplied by the superior mesenteric artery (SMA). We experienced a 12-year-old patient with NOMI that was segmentally localized in the ascending colon and rectum during encephalitis treatment. CASE PRESENTATION: A 12-year-old boy was hospitalized with limbic encephalitis. On day 41 after admission, he abruptly developed hypotension following diarrhea and fever, and presented abdominal distension. A computed tomography scan revealed pneumatosis intestinalis localized in the ascending colon and rectum coexisting with portal venous gas. The presence of peritoneal signs required an emergency laparotomy. Intraoperatively, skip ischemic lesions were found in the ascending colon and the rectum without bowel perforation. SMA and superior rectal arterial pulsation were present, and the patient was diagnosed with NOMI. The remaining colon, from the transverse to the sigmoid colon, appeared intact. We performed a distal ileostomy without bowel resection. Postoperative colonoscopies were carried out and revealed rectal and ascending colon stenosis with ulceration but demonstrated the patency of the two lesions. We confirmed the improvement of the transient bowel strictures; therefore, the ileal stoma was closed 14 months after the previous laparotomy. CONCLUSION: NOMI can be present in childhood during encephalitis treatment and can be segmentally localized in the ascending colon and the rectum. Although NOMI is most often seen in elderly patients, we should also consider the possibility of NOMI when pediatric patients with severe illness manifest abdominal symptoms.

Dieulafoy lesion in a two-year-old boy: a case report.

BACKGROUND: Massive gastrointestinal bleeding in children, mostly caused by esophageal varices secondary to chronic liver disease, is uncommon. Dieulafoy lesion in the gastrointestinal tract is a rare but important cause of gastrointestinal bleeding; massive bleeding from this lesion can be fatal unless adequate treatment is promptly initiated. We report a case of gastric Dieulafoy lesion in a 2-year old successfully treated with endoscopic hemoclipping. CASE PRESENTATION: A 2-year-old Japanese boy was admitted to our department with sudden massive hematemesis. He had no significant past medical illness, and he was well just before the episode of hematemesis. A clinical examination revealed anemia (hemoglobin, 8.0 g/dl). The rapidly progressive anemia associated with massive hematemesis indicated the presence of an active bleeding in his upper gastrointestinal tract. We performed emergency gastroscopy under general anesthesia. The gastroscopy revealed the presence of an abnormal visible vessel with an adherent clot on the lower body of his stomach. No mucosal abnormality surrounding the lesion was noted; the lesion was thus diagnosed as Dieulafoy lesion. One hemostatic clip was placed on the Dieulafoy lesion and excellent hemostasis was obtained. He recovered without blood transfusion and was discharged 4 days post-endoscopy. He has recovered well with no recurrence of hematemesis. CONCLUSIONS: Dieulafoy lesion is rare cause of sudden massive gastrointestinal bleeding in children. Nevertheless, it should be considered a differential diagnosis, even in babies. With advances in gastrointestinal endoscopy, as both a diagnostic and therapeutic modality, laparotomy secondary to gastrointestinal bleeding from Dieulafoy lesion has decreased in pediatric cases. Our case report demonstrates the feasibility of endoscopic hemoclipping for gastric Dieulafoy lesion in a child.

A Modified Technique with a New Device of Laparoscopic Percutaneous Extraperitoneal Closure for Pediatric Inguinal Hernia.

INTRODUCTION: Laparoscopic percutaneous extraperitoneal closure (LPEC) in boys is more complex than in girls, because the vas deferens and testicular vessels must be separated from the peritoneum. We therefore developed a needle-shaped surgical instrument that enables blunt separation. Here, we report the effectiveness of this new device. SURGICAL TECHNIQUE: The new device is a ligature carrier consisting of (1) an inner tube (19-G blunt needle) through which slides a rod with a loop wire at its tip that can catch and release suture materials and (2) an outer tube (17-G needle) with a cutting edge. The blunt-structured inner tube enables blunt separation. All actions (stabbing, separation, and catching) in the LPEC procedure that are required to close the hernia orifice using a thread are facilitated through a simple small incision by switching between the inner and outer tubes. SUBJECTS AND METHODS: From January 2013 to September 2015, 73 boys with inguinal hernia or hydrocele underwent LPEC, and the new device was used in 45 of these cases. We compared surgical results between cases wherein the new (group A) and conventional devices (LPEC needle; group B) were used. RESULTS: The mean surgery duration was 33.4 and 34.8 min for groups A and B, respectively. Neither group experienced complications during or after surgery. CONCLUSION: Our findings suggest that the newly developed needle device may improve the safety of the LPEC procedure for boys, although further investigation involving more cases and long-term follow-up is needed in the future.

Use of an appendicitis medical information sheet in the pediatric primary care system.

BACKGROUND: Accurate and prompt diagnosis is required for the primary evaluation of pediatric appendicitis. Among pediatricians and surgeons working in Yamanashi Prefecture, the pediatric appendicitis medical information (PAMI) sheet was edited in April 2011 to reflect the diagnostic results of the pediatric primary and emergency medical service and used as a referral document for surgical consultation to secondary hospitals. METHODS: The PAMI sheet consisted of sections for history taking, symptoms, physical signs and laboratory findings without a scoring system. For 32 consecutive months starting in April 2011, 59 patients hospitalized for suspected appendicitis were retrospectively reviewed. In particular, a total of 17 referral patients evaluated with the PAMI sheet were assessed in order to evaluate the utility of the form. RESULTS: The pediatric surgeons were able to easily determine patient condition from the PAMI sheet. In total, 13 of 17 patients had appendicitis. According to the physical findings of the 17 studied patients, the judgment of right lower quadrant tenderness (κ = 0.63) and guarding (κ = 1.00) was consistent between the pediatric surgeons and primary attending pediatricians. CONCLUSIONS: The PAMI sheet aids in the collection of detailed history and objective data with a high level of accuracy, and provides useful referral diagnostic information to the secondary-level hospitals.

[Thoracoscopic diaphragmatic plication for eventration of diaphragm in children using no-knife automatic suturing device].

UNLABELLED: Diaphragm eventration could inhibit the lung development due to compression. Thus diaphragm plication is required for the diaphragm eventration to prevent lung compression causing lung immaturity. However, we sometimes encounter the difficulty in endoscopic plication for fragile diaphragm without damaging it in narrow thoracic space in children. We demonstrate the plication using no-knife automatic suturing device. METHOD: Two linear ridges are made using stapler on the flaccid diaphragm without cutting the tissue. Then the created 2 ridges are sutured so that the diaphragm is plicated. BENEFITS: Once the stapler was applied to make 2 linear ridges, we easily sutured and gathered them without checking the damage of the intra-abdominal organs. Furthermore, reinforced ridges could be plicated without damaging the fragile diaphragm. We conclude that above described method is preferable for the diaphragm eventration in pediatric patients with fragile diaphragm and limited thoracic space.

Thyroglossal duct cyst accompanied by laryngomalacia and pulmonary sequestration.

A 2-month-old full-term female infant developed nasal stridor, which progressed to respiratory distress and poor sucking ability. Direct pharyngoscopy showed laryngomalacia and a midline cystic mass in the lingual region. The mass pressed on the epiglottis, causing dyspnea. Computed tomography incidentally revealed extralobar pulmonary sequestration. Direct deroofing of the lingual cyst and plication of the epiglottis were performed at 3 months of age, and the patient recovered from the respiratory distress. Histopathology of the cystic mass showed a thyroglossal duct cyst. Thoracoscopic resection of the pulmonary sequestration was then done at 17 months of age. Thyroglossal duct cysts in the lingual region may cause destabilization of the epiglottis and laryngomalacia, resulting in acquired respiratory obstruction. The combination of thyroglossal duct cyst, laryngomalacia, and pulmonary sequestration is rare; therefore, reports must be accumulated in order to explore the embryological origins of such cases.

Costal BCG osteomyelitis developing 1 year after BCG vaccination.

Reported here is the case of a 15-month-old boy with costal osteomyelitis due to the bacillus Calmette-Guérin (BCG) vaccine against tuberculosis. Mild complications of this vaccine, such as localized abscess and regional lymphadenitis, are occasionally recognized, but its association with osteomyelitis is extremely rare.

Non-small cell lung carcinoma therapy using mTOR-siRNA.

Molecular targeting agents play important roles in non-small-cell lung cancer (NSCLC) therapy. Published studies have investigated new drugs categorized as molecular targeting agents that inhibit the mammalian target of rapamycin (mTOR). We focused on a small interfering RNA (siRNA) that specifically inhibits mTOR and has fewer side effects. To evaluate the antitumor effects of the siRNA, cell proliferation, apoptosis, and migration were assessed. In the study group, the siRNA was transfected into NSCLC cells. The number of cells present after 6 days of culture was counted to determine changes in cell proliferation. The level of apoptosis was evaluated by the detection of DNA-histone complexes in the cytoplasmic fraction using an absorption spectrometer. Changes in migration were evaluated by calculating the number of cells that passed through a specific filter using a commercial chemotaxis assay kit. mTOR-siRNA transfection inhibited cell proliferation as indicated by 37.3% (p = 0.034) decrease in the number of cells compared with the control cells. Analysis of the level of apoptosis in NSCLC cells revealed 16.7% (p = 0.016) increase following mTOR-siRNA transfection, and mTOR-siRNA transfection significantly inhibited cell migration by 39.2% (p = 0.0001). We confirmed that mTOR-siRNA induces apoptosis and inhibits the proliferation and migration of NSCLC cells in vitro. Further studies using mTOR-siRNA may aid in the development of an alternative therapy that maximizes the antineoplastic effect of mTOR inhibition.

Two different types of infantile renal cell carcinomas associated with tuberous sclerosis.

Renal cell carcinoma (RCC) associated with tuberous sclerosis (TSC) has been reported in adult patients; however, this association is rare in children. We have encountered 2 different types of RCCs in infants who developed TSC later in childhood. The pathologic diagnoses were papillary RCC in one infant and a clear cell-type RCC in the other. Gene analysis showed that both the patients had a mutation in TSC2 gene on chromosome 16.

Perforation of Meckel's diverticulum manifesting as aseptic peritonitis in a neonate: report of a case.

We report a case of perforated Meckel's diverticulum with aseptic peritonitis in a 17-day-old neonate. The baby had been brought to the hospital with fever and abdominal distention. Abdominal computed tomography showed a 5-cm abscess in the lower abdomen, and emergency laparotomy was performed for suspected perforated appendicitis. However, we found a perforated Meckel's diverticulum. No bacteria were detected in the purulent ascites from the peritoneal cavity. We speculate that the narrow lumen between the small intestine and the diverticulum, accompanied by poor self-emptying had caused acute inflammation resulting in perforation of Meckel's diverticulum. The anatomic limitations in "walling off" the perforated Meckel's diverticulum by the surrounding loops of small intestine prevented the bowel contents from spreading within the peritoneal cavity.