Akira Nagasaki

Isolated unilateral absence of left pulmonary artery with peribronchial arteriovenous malformation showing recurrent hemoptysis

Unilateral absence of a pulmonary artery is an uncommon condition and usually complicated by a cardiac anomaly. Our case is a rare one who showed the absence of the left pulmonary artery with left aortic arch and without cardiac anomaly. He suffered from recurrent hemoptysis and pneumonia since he was 9 months old. Angiography revealed peribronchial arteriovenous malformation of the affected lung which was supplied from subclavicular arteries and bronchial arteries. Although he was treated by operative ligation and angiographic embolotherapy of the supplying systemic arteries, the repeated the attacks of massive hemoptysis and necessiated left pneumonectomy at 10 years of age.

Induction of the rectoanal reflex by electric stimulation: A diagnostic aid for Hirschsprung's disease

We induced the rectoanal reflex electrically in three groups of children, following rectal dilatation with a balloon. In normal children, and in children with constipation or ileus due to causes other than Hirschsprungs disease, the rectoanal reflex was induced by electric stimulation as well as by dilatation of the rectum with a balloon. In children with Hirschsprungs disease, however, no typical reflex was obtained by either of these stimuli. Since electric stimulation does not dilate the rectum, passive dilatation of the anus or shift of the probe along with balloon expansion does not take place, so no false-positive reflex is elicited. Electric current, moreover, is capable of providing a constant quantifiable stimulus. We have demonstrated the induction of the rectoanal reflex by electric stimulation alone; distention of the circular muscle of the rectum does not appear necessary for the induction of this reflex.

An Immunohistochemical Study of Serotoninergic Nerves in the Colon and Rectum of Children with Hirschsprung's Disease

SummarySerotonin (5-HT)-like immunoreactive nerve fibers were investigated in gut tissue obtained from seven Japanese children with Hirschsprungs disease. In the control untreated tissues, 5-HT-like immunoreactive fibers were observed neither in the normoganglionic nor in the aganglionic regions. After pargyline treatment, 5-HT-positive neuropils were consistently detected in association with the myenteric plexus in the normoganglionic segment, while in the aganglionic segment immunoreactive fibers could not be demonstrated through the entire layer of the bowel tissue. The occurence of 5-HT-like immunoreactive neuropils by pargyline treatment strongly suggests that the infant bowel is innervated with serotoninergic elements. After treatment with 5-hydroxy-l-tryptophan (5-HTP), the immunoreactive neuropils were localized in the myenteric and submucous plexuses of the normoganglionic segment. On the other hand, another type of 5-HT-positive fibers was characteristically demonstrated in aganglionic segments following the drug treatment; moderate numbers of 5-HT-like immunoreactive fibers appeared in the intermuscular zone and within the circular and longitudinal muscle layers. Several 5-HT-positive fibers were present in the hypertrophic nerve bundles seen in a diseased bowel. A possible origin of this type of 5-HT-positive nerve fibers was discussed.

Assessment of bowel control with anorectal manometry after surgery for anorectal malformation

Bowel function after surgery for anorectal malformation was assessed by Kellys score in 101 children over 3 years of age. Seventy-two were examined using anorectal manometry. In the “good” group, resting pressure of the anal canal was as high as in the normal children, and the frequency of contraction waves in the anal canal was the same as in the normal children. Recto-anal reflex was recognized in about three fourths of the “good” group. On the other hand, in the “poor” group resting pressure was low, in only one were contraction waves evident and in none was there a recto-anal reflex. The findings of the anorectal manometry placed the “fair” group between the “good” and the “poor” groups. The clear contraction waves of the anal canal or the clear recto-anal reflex related to a resting pressure in the anal canal of over 20 cmH2O. This high pressure in the anal canal is important for good anal continence, and depends on a precise pull-through of the rectum into the puborectalis muscle.

Gastric teratoma in infancy and childhood: Report of three cases and review of literature

Three cases of gastric teratoma in infants are described and a review of 26 hitherto reported cases is presented. Our three cases have been treated by excision successfully and all have survived. No recurrence following surgery has been noticed. Male preponderance, appearance in early age of life, abdominal distension and mass including calcification are helpful for diagnosis but a thorough differentiation from nephroblastoma, neuroblastoma and other malignant tumors is needed.

Hirschsprung's disease in an adult

A 24-year-old Japanese woman with Hirschsprungs disease was subjected to the combination of barium enema, anorectal manometry, and a histochemical study of acetylcholinesterase (AChE) activity in rectal suction biopsy. The histochemistry revealed a marked increase of AChE positive nerve fibers. The importance of such a study is stressed in order to diagnose Hirschsprungs disease correctly, even in adults. A Z-shaped anastomosis as a modification of Duhamels method was carried out. The postoperative course was uneventful and she now has normal bowel evacuation.

Esophageal manometrical assessment after esophageal circular myotomy for wide-gap esophageal atresia

We treated 3 children with wide-gap esophageal atresia by a circular myotomy of Livaditis technique, after which no postoperative complaints, such as heartburn, vomiting or dysphagia, were seen. Esophageal manometry, performed to evaluate the postoperative esophageal motor function revealed; (1) that lower esophageal sphincter pressure (LESP) increased gradually with time, (2) esophageal contraction waves (ECW) were evident at the site of the circular myotomy with swallowing, though these contractions were simultaneous; and (3) relaxation of the LES with swallowing was evident. These findings, as determined by the esophageal manometrical assessments, indicate that there is no difference between the postoperative esophageal function after either repair with a circular myotomy or primary anastomosis for esophageal atresia.

Radiologic diagnosis of Hirschsprung's disease utilizing rectosphincteric reflex

Artificial balloon distension of the rectum caused a reflex opening of the anal canal as seen during barium enema in 10 out of 10 normal children and in 7 of 7 children with idiopathic constipation. Reflex opening of the anal canal was not observed in any of 15 children with proven Hirschsprungs disease. This lack of response was independent of the length of aganglionosis or previous diverting colostomy and corresponded to the fact that on manometric study the intraluminal pressure of the anal canal fell in normal children but not in the children with Hirschsprungs disease. This finding greatly improves the reliability of barium enema in the diagnosis of Hirschsprungs disease.

Histochemical acetylcholinesterase reactions in total colonic aganglionosis

Reliability of the histochemical diagnosis in total colonic aganglionosis is controversial. We studied histochemical acetylcholinesterase (AChE) reactions in three children with total colonic aganglionosis. In all three, there was an increase in AChE positive nerve fibers in the rectal mucosa, obtained when the patients were over the neonatal period. However, a proximal extension of the increase in AChE positive nerve fibers was noted in the distal end of the resected bowel obtained at the definitive operation in two cases out of three. In one, there was no proximal extension in the resected bowel, and in another, there was an increase in AChE positive nerve fibers in the mucosa of the ganglionic ileum. Our findings indicate that the histochemical study of AChE activity in rectal suction biopsy is useful, even in cases of total colonic aganglionosis.