Munechika Wakisaka

Digestive tract disorders associated with asplenia/polysplenia syndrome.

The authors reviewed experience gained over a 20-year period of asplenia or polysplenia syndrome, focusing on patients with associated digestive tract disorders (DTDs). Eleven of 27 patients (40%) with asplenia/polysplenia had associated DTDs. The DTDs comprised malrotation of the intestine in nine, both preduodenal portal vein and gastric volvulus in three, esophageal hiatal hernia in two, and biliary atresia in one. Laparotomy was carried out on four patients with symptoms of acute bowel obstruction and on one patient with biliary atresia. One patient with both malrotation and gastric volvulus, and another with only associated malrotation survived. Nine patients died, eight of cardiac insufficiency and one because of hepatic insufficiency. When infants are diagnosed with heterotaxia, they should be examined for other combined DTDs, because they may have a chance for survival if they undergo surgery when their condition is still stable.

Demonstration of apoptosis in neuroblastoma and its relationship to tumour regression.

The in vivo occurrence of apoptosis in neuroblastomas was investigated. Histologically, a number of tumour cells showed typical apoptotic changes, including cell shrinkage, condensed and fragmented nuclei, eosinophilic cytoplasm, and absence of the inflammatory response. These cells coincided closely with the so-called karyorrhectic cells. An electrophoretic DNA ladder, a functional hallmark of apoptosis, was demonstrated in four of six tumours, and DNA fragmentation was detected in situ by terminal deoxytransferase-mediated nick end-labelling in 26 of 35 tumour specimens (74%). The labelled cell counts ranged from 5 to 62 per 5000 tumour cells (mean±SD: 15.0±14.5). Immunoperoxidase staining revealed that an apoptosis-suppressing protein, bcl-2, was expressed abundantly in advanced-stage tumours, whereas it was absent from karyorrhectic-apoptotic cells. Several tumours with the potential for spontaneous regression were bcl-2-deficient. Immunostaining of the Fas receptor for apoptosis demonstrated that the tumour cells expressed this molecule on their cell surfaces. Our results provide evidence of apoptosis in neuroblastomas and suggest that bcl-2 and the Fas receptor may play a role in its regulatory mechanisms.

Hemangioma of the upper arm associated with massive hemorrhage in a neonate

A rare case of an ulcerated hemangioma of the upper arm in a neonate is described herein. Resection of the huge hemangioma, which occupied the axilla and extended down the entire length of the upper arm, was necessary due to massive hemorrhage from the ulcer and progressive heart failure caused by the arteriovenous fistulae in the hemangioma. After complete resection of the hemangioma, extensive defects of the skin and subcutaneous tissue were replaced by a skin flap closure technique which involved considerable difficulty. However, the arm gradually recovered its size and function with good wound healing. The present case illustrates that rapid surgical intervention may be required to prevent clinical deterioration in such cases.

The usefulness of laryngotracheal separation in the treatment of severe motor and intellectual disabilities

Intractable aspiration is a life-threatening medical problem in patients with severe motor and intellectual disabilities (SMID). Laryngotracheal separation (LTS) is a surgical procedure for the treatment of intractable aspiration which separates the upper respiratory tract from the digestive tract. We performed LTS for 14 patients with SMID to prevent intractable aspiration, performing two types of operation. The standard diversion procedure connected the upper trachea to the esophagus. The modified diversion includes closure of the proximal trachea and a high tracheostomy, avoiding a tracheoesophageal anastomosis. LTS was performed on 14 patients. Operations performed before the LTS included tracheostomy in four patients, fundoplication in six and gastrostomy in two. A standard diversion was performed in 11 patients and a modified diversion in 3. There were no operative complications. Eleven patients were safely transferred to home-care after their LTS. Twelve patients are still alive and two died some months after operation. One patient died from their primary disease and the other died a tracheo-innominate artery fistula (TIAF). We recently experienced a patient who was at high risk of developing a TIAF. LTS is an effective operation, preventing intractable aspiration in patients with severe motor and intellectual disabilities. The results are similar for the standard or modified diversion procedure with the procedure chosen being related to the initial tracheostomy site. The most serious complication is a lethal TIAF.

Giant transdiaphragmatic duodenal duplication with an intraspinal neurenteric cyst as part of the split notochord syndrome: report of a case.

We report the case of a 6-month-old girl with a giant transdiaphragmatic duodenal duplication connecting to a cervical intraspinal cyst, which compressed the heart and lung, causing severe respiratory distress. We removed the intraspinal neurenteric cyst through a posterior cervical incision at the C6 and C7 level, and the duodenal duplication cyst in the mediastinum was removed through a right thoracotomy. Her respiratory symptoms were completely resolved by these operations.

Efficacy of exogenous fibronectin in wound healing in malnourished rats.

BACKGROUND/PURPOSE Fibronectin (FN) plays an important role in fibrin matrix formation during the wound healing process. The authors investigated whether exogenous FN increases the bursting strength (BS) of surgical wounds in malnourished rats. METHODS Ninety rats were grouped according to three nutritional conditions (n = 30 in each group). All animals underwent a transverse celiotomy after 3 weeks of feeding, and FN (2 mg/body/day) was given postoperatively to 15 rats in each of the three groups. Wound BS was measured on postoperative days (PODs) 3, 5, and 7 after removal of the sutures. RESULTS BS in normally nourished rats (group N; 142.6+/-23.4 mmHg) was significantly higher than that in protein malnourished rats (group PM, 110.2+/-11.2 mm Hg) and protein/ calorie (Cal)-malnourished rats (group PCM, 76.5+/-10.7 mm Hg) on POD 7 (P<.01). However, BS values for groups PM + FN (147.0+/-21.1 mmHg) and PCM + FN (115.1+/-28.9 mm Hg) were intensified significantly in comparison with groups PM and PCM (P<.01). Plasma FN levels in rats of the three FN nontreated groups were similarly decreased on POD 3 or 5, but returned to the preoperative level on POD 7, whereas those for the other three FN-treated groups increased after POD 3. CONCLUSION Intravenous administration of FN might strengthen the weakened wounds of malnourished animals.

An infant with bilateral adrenal neuroblastoma found by mass-screening: Report of a case

A male infant with bilateral adrenal neuroblastoma found by mass-screening is herein reported. The patient presented with almost equal-sized tumors, each remaining confined to the respective adrenal gland without crossing the midline. No other tumorous lesion was evident. The two adrenal tumors had similar histologic features and appeared to have grown concurrently. Such simultaneous occurrence of primary adrenal neuroblastoma is unusual, and appears to reflect the multicentric origin of this tumor.

Renal salvage procedure for synchronous bilateral Wilms' tumor

Abstract This report describes a renal salvage procedure performed in a 2-year-old girl with bilateral renal tumors comprising a multilocular, cystic tumor of the right kidney and a solid and cystic nephroblastoma of the left kidney after chemotherapy. Surgery was performed because the right kidney became hydronephrotic due to compression by the enlarged cysts, while the left tumor showed only minimal shrinkage even after three courses of chemotherapy. The right-sided cysts were simply unroofed and the left-sided tumor was extirpated by partial nephrectomy. Her postoperative course was uneventful with considerable recovery of the function of each kidney. This procedure should be considered if the pathological features of the tumor are relatively favourable.

A Case of Congenital Infantile Fibrosarcoma of the Bowel Presenting as a Neonatal Intussusception

Neonatal intussusception of the intestinal tract is rare. However, most neonatal intussusceptions have an organic lead point. For the lead point to be a neoplasm is extremely rare. We report a case that presented with neonatal intussusception with a congenital infantile fibrosarcoma as the lead point. The detection of ETV6‐NTRK3 gene fusion was useful, although the definitive diagnosis was achieved by a comprehensive evaluation including this gene analysis, standard histology and immunohistochemistry. Neonatal intussusception should be suspected to be caused by a neoplasm. If pathological diagnosis is difficult, molecular analysis should be utilized to diagnose congenital infantile fibrosarcoma.

High-dose chemotherapy followed by autologous peripheral blood stem cell transplantation for recurrent primary mediastinal malignant germ cell tumor: A case report

A 15‐yr‐old boy presented with an anterior mediastinal mass, multiple lung metastases and obstruction of the left brachiocephalic vein, the superior vena cava and the subclavian vein. Tumor biopsy by CT guidance confirmed a diagnosis of GCT. Five courses of BEP therapy were performed, and CT of the chest revealed reduction in the anterior mediastinal mass and disappearance of the multiple lung metastases. We performed the anterior mediastinal mass extraction followed by adjuvant chemotherapy consisting of ICE and TIP. However, the AFP levels became elevated soon after. Abnormal accumulation was observed in the right upper lung by DW‐MRI. After the operation, two courses of TI chemotherapy and two courses of HDCT followed by auto‐PBSCT were performed. He was complicated with auditory disorder and renal dysfunction. Although HDCT followed by auto‐PBSCT was effective for the relapsed primary mediastinal GCT, a treatment strategy avoiding late complications is warranted.