Tha development of anal sphincter muscles is a most important prognostic factor in children with imperforate anus. In order to evaluate the development of anal sphincter muscles, CT examination was carried out and the area of muscles distribution in inferior pelvic aperture was calculated with micro-computer. Our results are as follows ; 1. In the low type anomalies which blind pouch was below I line, muscle distribution was as wide as in the normal cases. 2. Whereas, in the high type anomalies which blind pouch was on M line, muscle distribution was narrower. Furthermore, in the case which blind pouch was on P-C line, muscle distribution was extremely limitted. 3. A ratio of the area of muscle distribution to that of inferior pelvic aperture may be a valuable index for evaluating the development of anal sphincter muscles.
One hundred and twenty cases of imperforate anus, including 70 cases of high type and 50 cases of low type, were retrospectively analyzed in respect to the associated vertebral anomalies. Various kinds of vertebral anomalies were associated with 28.3% of the cases. The association rate was 37.1% of high type and 16.0% of low type. It was obvious that the high type has significantly more chance to accompany the vertebral anomalies than the low type anomalies. Some specific types of imperforate anus, such as rectal agenesis, rectovestibular fistula, rectovesical fistula, and anal stenosis, had high association rate than others. The most vulnerable protion of the vertebral column was sacral region. Lower lumber and middle thoracic spines were the next to it. High type anomalies occasionally involved multiple spines. The vertebral deformities in high types frequently advanced to congenital scoliosis. The vertebral anomalies accompanied with high types were severer than with low ones. From the classification of accompanied vertebral anomalies, it was suggested that critical period of vertebral anomalies in high type imperforate anus would be earlier stage of prenatal period than that in low type anomalies.
A rat model relevant to pediatric praxis was designed in order to evaluate possible differences between continuous TPN and intermittent TPN. 1) Intermittent TPN resulted in stastically significant higher serum albumin level and nitrogen utilization. Plasma aminogramms showed similar pattern between intermittent TPN rats and orally fed rats. 2) Intermittent TPN rats had circadian rhythm in hepatic weight, glycogen, and protein content which were similar to those of orally fed rats. While continuous TPN rats lost the rhythmic changes. 3) Fat accumulated in the liver of continuous TPN rats, but not in the liver of intermittent TPN rats. These findings suggest that intermittent TPN may be nutritionally more beneficial than continuous TPN.
The Introduction of total parenteral nutrition (TPN) by Dudrick in 1968 has led to its wide spread acceptance and use in nutritional support. As the result, in pateints suffering from massive small bowel resection or severe chronic bowel disease, long-term nutritional support became necessary. The hospitalization of these patients has caused some probrems like restriciton of activity, psychological stress, and so on. To solve these problems, we have treated 8 patients, with ranges of age 6 months to 15 years, on an intermittent TPN program for 157 patients months. Patients in this program achieved a satisfactory physical growth and mental development. All resumed group activities and six patients were able to return to their home. No major complications were noted in this method. Administration of the intermittent TPN is a safe and succesful techniqe for maintaining an optimal nutritional status in children with severe digestive disorders, and permits resumption of a more normal daily lifestyle.
Twe types of ECMO units for newborn repiratory failure were developed in our laboratory. One utilizes a roller pump for venoarterial bypass (V-A bypass) or venovenous bypass of a continuous perfusion system (continuous V-V bypass). Another unit utilizes a pneumatic driving system for venovenous bypass of a to-and-fro perfusion system (to-and-fro V-V bypass) which could easily be performed by inserting a single cannula via the jugular vein. With these units, three types of bypasses were investigated experimentally by using puppies whose weights ranged from 2.5 to 5.0kg. General aneathesia was maintained with hypoventiration and ECMO was started. Hemodynamic and blood gas data were measured to evaluate the effect of ECMO. And, the three types of bypasses were performed for twelve hours to examine the effect on the liver, kidneys, lungs and blood. Conclusions were as follows : 1) Our ECMO units were safe and easily operated. 2) Hemodynamics during ECMO were stable and there were no differences between three bypass methods. 3) As to the effect on the reapiratory support, V-A bypass was most effective, but even in the to-and-fro V-V bupass, oxugen transfer of 2.4ml/kg/min was obtained with a bypass flow of 50ml/kg/min. 4) The effect on the liver, kidneys, lungs were almost none, and blood was only slghtlty damaged during twelve-hour perfusions. 5) Clinical applicasion of the to-and-fro V-V bypass is warranted.
Seventeen cases of testicular yolk sac tumor, which we have experienced in the past 17 years, are reviewed in this paper. Orchiectomy combined with the retroperitoneal lymphadenectomy had been carried out in the beginning of our experiences, but since 1976, chemotherapy was introduced. Initially, vincristine, actinomycin D and cyclophosphamide were mainly chosen. Nevertheless, we lost 3 cases who had lung metastasis at initial operation. At 1983, we introduced cis-platinum as a chemotherapeutic agent. Since then, all patients, including 1 case with obvious lung metastasis, have been survived.
In the surgical treatment of anorectal malformations, posterior sagittal anorectoplasty (Pena) allowed direct exposure of the anal sphincter and levator ani muscle enabling the rectum to pass through the ideal portion. For female imperforate anus such as ano- (recto) vestibular fistula. "cut back" operation and anal transplantation have been widely used for many years. We adopted the idea of Pena in the treatment of these malformations, and proposed a new approach "anterior sagittal anorectoplasty." in which all muscles were indentified by the use of an electrical stimutation at the wound of perineal incision, followed by anorectoplasty. We have performed this procedure in 5 cases with fairly satisfactory results. Further fllow-up studies are necessary in the furture.
Recently encounted were four patients with acquired glottic stenosis, occurring due to granulation tissue arising immediately from behind the vocal cords. The management of glottic stenosis of this type used to be conservative, leaving tracheostomy for a long time during infancy and childhood without knowing when the tube comes off. However, based on recent reports on the literatures describing no deleterious effect in the growth of the larynx after laryngofissure performed in infancy, surgical intervention was made in these four patients. In each patient, non-operative care with tracheostomy was first attempted for a few months without improvement. In some of the patients, endoscopic excision of the granulation tissue was repeated but was unsuccessful. The important points peculiar to this operation are ; 1. Median thyrotomy made strictly on the midline to avoid an injury to the vocal cords. 2. Meticulous excision of the granulation tissue facilitated by a bronchoscopic guidance, using microsurgical technique. The outcome of this operation was satisfactory in each patient. All of the patients started phonation within 6 mouths after operation and have been doing well without airway problems from 1 to 3 years of follow-up.
Complications are evaluated on 516 patients with cryptorchidism 6 months after orchidopexy. The rate of complications is 1.9%, mainly composed of retraction, wound infection and testicular atrophy. To prevent these complications, sufficient elongation of the spermatic cord at the initial operation is mandatory.
A 13-year-old girl of splenic abscess was reported. She had past history of perforative appendicitis. A month later, splenic abscess was detected using ultrasonography. Conservative treatment had been continued but multiple abscess were not diminished. So she was transffered to our hospital for splenectomy. Resected spleen was markedly enlarged and involved multiple abscess. There are two types of splenic abscess. One is solitary abscess, and another is multiple ones. The former was caused by primary splenic infection and the latter was from the infection of the other organs. Our case had previous history of acute appendicitis and abscess formation was multiple, so it is supposed that her splenic abscess was caused by appendicitis. She was discharged from our hospital 27 days after operation and she had no complaint on her life.
A case of congenital diaphragmatic hernia associated with esophageal rupture is reported. Apgar score of this full-term female neonate weighing 3650gms was 6. She developed tachy-pnea and occasional cyanosis soon after her birth. Chest and abdomial X-ray was compatible with congenital left-sided diaphragmatic hernia, but no free air was detected on the films. She was operated on 9 days after birth. In addition to the left posterolateral diaphragmatic hernia without sac, a rapture probably due to incarceration was found in the anterior wall of the lower esophagus. The diaphragmatic defect as well as rupture were repaired successfully and her postoperative course was uneventful. Since complication of the esopageal rupture in congenital diaphragmatic hernia is very rare but serious, the authors described in detail.
Adrenal cortical tumor is a very rare disease, especially in childhood. More than 80% of virilization caused by adrenal cortical tumor are malignant and adrenal cortical adenoma is extremely rare. A case of precocious puberty due to adrenal cortical adenoma was reported. A 2-year-old boy was admitted with complaints of enlarged penis pubic hair, and accelerated body growth. Urinary excretion of 17-KS and concentration of serum androgen were markedly elevated, but both urinary 17-OHCS and serum cortisol were within normal ranges. IVP, CT scan, and arteriography demonstrated a calcified left suprarenal mass. A well-capsulated oval tumor originating from the left adrenal gland, 5×4×3cm in size, 40 gw was completely removed. Histological diagnosis was adrenal cortical adenoma without capsular and vascular invasion, with low frequency of mitosis. Postoperative course was uneventful and he has been well without a sign of recurrence for 2 years by the time of this report. Sixty-four cases of virilization caused by adrenal cortical tumor were collected from Japanese literature, and 9 cases (16%) of adrenal cortical adenoma were included. Adrenal cortical adenoma showed pure virilizing symptoms except a case mixed with feminization, in comparison with carcinoma.
Hernia through the foramen of Winslow is extremely rare lesion, and only 10 cases, including one pediatric patient, have been reported in Japan. A 14-year-old boy of this condition is presented. The abdominal X-ray films show some characteristic findings, intestinal gas-fluid levels in the lesser omental sac separating from the stomach and colon cut-off sign of the hepatic flexure due to the dilated intestinal loop. At surgery, length of 90cm of the ileum herniated into the lesser omental sac through the foramen of Winslow, and that intestinal loop was easily replaced on the outside of the omental sac. The postoperative course was uneventful.
Laboratory findings revealed mild anemia and liver dysfunction in a boy, one year and eight months old, who was admitted to this hospital with vomiting and anorexia as chief complaints. During examination, a liver tumor was detected by ultrasonography of the abdomen. Since the tumor suddenly ruptured while preparations were being made for surgical treatment, emergent laparotomy was applied to right hepatic lobectomy. Bleeding and necrotic tissues were observed due to the ruptured tumor wall. Histological diagnosis was hepatoblstoma of the well-differentiated type. Since the postoperative course was favorable, multidrug chemotherapy using cis-platin was initiated on the 12th postoperative day. After five cycles of treatment, the patient is now 3 years and 6 months old and is healthy and free from disease.
A 3 day-old boy with bloody stool, abdominal distension and bilious vomiting was transferred to our hospital in a preshock condition. The diagnosis of malrotation with midgut volvulus was made by upper GI series. The patient underwent laparotomy immediately. The massive involvement of the small bowel from the proximal jejunum to the terminal ileum was found. The reduction of volvulus and Ladd's operation were performed. The second-look operation done 22 hours later showed no dramatic improvement in the ischemic bowel. High jejunostomy just oral to the proximal end of the lesion and peritoneal drainage were done without resection of the grossly ischemic bowel. There were no immediate postoperative problems. The third operation undertaken for a closure of jejunostomy one month after second-look operation revealed recovery of all the small bowel from a pregangrenous condition and the stricture of the jejunum anal to the distal stoma. The stricture and jejunostomy were taken down and able to be reconstructed by primary anastomosis, preserving one meter of small bowel. TPN was weaned within 2 months after closure of jejunostomy and the following course was satisfactory. He showed neither growth nor intellectual retardation at the time of follow-up of 2 years old. This new technique which had been used for massive necrotizing enterocolitis was applied successfully for a patient with midgut volvulus. It is suggested that this approach is a new surgical strategy of avoiding massive small bowel resection.