Akira Hayashi

Congenital pyloric atresia and junctional epidermolysis bullosa: a report of long-term survival and a review of the literature.

The coexistence of congenital pyloric atresia (PA) and epidermolysis bullosa (EB) in newborns is a rare but distinct association. Mortality is high. In particular, a universally fatal outcome has been reported in neonates born with the junctional type of EB and PA. This has led some investigators to advocate that surgical correction of PA be withheld to obviate needless suffering. We treated five patients, including one set of siblings. Maternal hydramnios and nonbilious vomiting were constant features. Delayed passage of meconium was found in four. Plain x-rays demonstrated gastric dilatation in an otherwise gasless abdomen. Blistering skin lesions were noted at birth in four and developed soon after in the last patient. All lesions were determined to be junctional EB based on electronmicroscopy. The clinical course for these children has been far better than the literature predicts. Successful repair of PA was performed after appropriate stabilization. One infant died at 4 months of age of staphyloccal septicemia, malnutrition, and sepsis from chronic urinary tract obstruction. Another child, born with dysmorphic features to consanguineous parents, is 9 years old and has a seizure disorder. The remaining three are alive and well at 17 months, and 9 and 16 years. The oldest two are siblings. In all four surviving patients, the blistering nonscarring lesions were found to significantly improve in severity, duration, and occurrence with age. Presently, these lesions are mild and require little therapy. Their nails, initially normal at birth, have become discoloured, thickened, and dystrophic. The management of pitted, carious, and yellow teeth is currently the major problem.(ABSTRACT TRUNCATED AT 250 WORDS)

Radionuclide imaging study of long-term pulmonary function after lobectomy in children with congenital cystic lung disease.

PURPOSE We evaluated the long-term pulmonary function after lobectomy for congenital cystic lung disease, in both infants and children, using radionuclide imaging (RI). METHODS We performed a retrospective review of 93 patients who underwent resection of cystic lung lesions between 1974 and 2001. The results of postoperative lung volume/perfusion scintigraphy at 1 (n = 64), 5 (n = 32), and 10 years (n = 18) after surgery (V1, 5, 10/Q1, 5, 10) and mean transit time (MTT-a marker for air-trapping) at 1, 5, and 10 years after surgery (MTT1, 5, 10) were compared with respect to age at operation, preoperative infection, underlying disease, and type of surgery. RESULTS Patients who were younger than 1 year at the time of surgery showed a significantly lower MTT5 (1.09 +/- 0.08) and MTT10 (1.15 +/- 0.11) than patients who were older than 1 year at the time of surgery (MTT5, 1.49 +/- 0.67; MTT10, 1.54 +/- 0.33). The noninfected group had significantly higher Q10 and lower MTT10 values (P < .05) compared to the infected group. No significant differences were observed between patients with single lobe vs multiple lobe resection. CONCLUSIONS The optimal age for surgery in patients with congenital cystic lung disease appears to be less than 1 year.

The absence of clinically significant tracheomalacia in patients having esophageal atresia without tracheoesophageal fistula

The association of tracheomalacia (TM) with esophageal atresia (EA) has been well described. This study attempted to find a correlation between the severity of TM and the presence or absence of an associated tracheoesophageal fistula (TEF) in patients with EA. A review of all patients presenting at this institution with EA through the years 1970 to 1990 was carried out. Six patients with EA without fistula (type A) and 61 patients having EA with fistula (types B, C, D, and E) were analyzed. Five of the six type A patients required esophageal dilatations and antireflux procedures; none had clinically significant TM. Thirty-five of the 61 patients with fistula required esophageal dilatation, and 8 had an antireflux procedure; 11 (18%) required either long-tube tracheostomy or aortopexy for TM. This small study supports a recent hypothesis of different embryological events resulting in different types of esophageal and tracheal anomalies. When EA occurs without fistula, it appears that no significant alteration in tracheal development occurs.

Topical sucralfate: Effective therapy for the management of resistant peristomal and perineal excoriation☆

Peristomal and perineal excoriation commonly occurs despite preventive measures. Drainage from around gastrostomy tubes or ongoing perineal soilage after a pull-through procedure can lead to chemical irritation, cutaneous denudation, and chronic discomfort. A multitude of topical agents have been tried with variable results. We present our experience using topical sucralfate. Fifteen patients with stomal or perineal skin ulceration were treated with topical sucralfate only after other agents had failed. Clinical photographs were obtained first. Sucralfate, prepared as either a powder or an emollient, was liberally applied to the affected area during diaper changes or when the stomal appliance was emptied. For tube gastrostomy sites, sucralfate was applied every 4 to 6 hours as required to maintain a visible layer. In 13 patients, complete healing occurred. Recovery time was dependent on the severity and extent of skin denudation. Partial healing occurred in one patient. In another patient, the skin excoriation healed but a residual candidal rash required addition of an antifungal agent. General observations included: (1) a lag time of 2 to 3 days before visible healing was evident; (2) healing occurred from the perimeter; (3) sucralfate was soothing and reduced discomfort; (4) it was ineffective for fungal dermatitis; and (5) sucralfate did not appear to have toxic or systemic effects. Topically applied sucralfate is soothing, safe, and effective.

Management of foreign-body bronchial obstruction using endoscopic laser therapy.

A case is presented in which the Nd:YAG laser obviated the need for a major thoracic procedure. A 7-year-old boy presented with an 18-month history of persistent nonproductive cough, preceded by a choking spell while eating pistachio nuts. High-grade left mainstem bronchial obstruction was caused by friable granulation tissue secondary to an underlying foreign body. By advancing a laser fiber through the working channel of a 5-mm Storz ventilating bronchoscope, the granulation tissue was vaporized, showing an irregular piece of pistachio nut shell embedded within the bronchial wall. After retrieval of the foreign body, bronchial patency was restored. Recovery was complete and uneventful. The bronchoscopic laser permits the delivery of precise and effective treatment to lesions within confined areas, and has a useful role in the management of some central airway lesions in children.

Balloon catheter dilatation for hypertrophic pyloric stenosis.

Balloon dilating catheters (BDC) have provided a non-operative means of managing obstructive lesions within the gastrointestinal tract. Its potential utility in infants with hypertrophic pyloric stenosis (HPS) was studied. Six patients with HPS underwent balloon catheter dilatation of the pylorus under the direct observation of the surgeon. The pylorus was exposed using a standard right upper quadrant incision. The BDC was passed transorally into the stomach and manipulated into the pyloric canal by the surgeon. The balloon was inflated with saline to a maximum pressure of 50 psi for 2 minutes. Four patients were dilated with a 10-mm diameter balloon catheter, and in two patients, a 15-mm balloon was used. Success was defined as the complete and longitudinal disruption of the seromuscular ring without violation of mucosal integrity. Using this criterion, none had successful pyloric dilatation. No disruption occurred in three patients, partial disruption in two. These patients subsequently underwent a Ramstedt pyloromyotomy. Complete disruption was observed in one; however, a breach of the mucosa was evident. This was repaired without incident. All seromuscular breaks occurred at the point of vascular entry along the lesser curve, presumably the weakest point of the ring. Pyloric dilatation using BDC does not reliably disrupt the muscular ring. This preliminary report recognizes that major refinements must occur before this method will supplant the time-honored surgical pyloromyotomy for HPS.

Emergency separation of conjoined twins

Female omphalopagus conjoined twins were successfully delivered vaginally and required emergency surgical separation shortly after birth for gastroschisis. Shared tissue included conjoined bowel; one twin also had a complex cloacal abnormality and patent urachus. A 2-year follow-up is presented. A review of the relevant literature confirms that this is the first example of gastroschisis conjoining omphalopagus twins.

Heterotopic pancreas as a rare cause of gastrointestinal hemorrhage in the newborn: Report of a case

We report herein a female infant in whom a heterotopic pancreas in the stomach caused gastrointestinal hemorrhage during the newborn period. Endoscopy was essential for ruling out other causes of hemorrhage and to follow the patient until the time of elective surgery at 6 months of age. Heterotopic pancreas should be borne in mind as a rare cause of gastrointestinal hemorrhage in the newborn when an unexplained gastrointestinal hemorrhage continues, and the lesion was discussed in relation to our experience of upper gastrointestinal endoscopies and a review of the literature.

Three-dimensional display of the pelvic structure of anorectal malformations based on CT and MR images

Three-dimensional images of the pelvic structure of patients with anorectal malformation (ARM) were constructed by computer graphics based on radiographic computerized tomography (CT) and magnetic resonance (MR) images. Organ contour data from CT images and raw MR image data were transferred to a personal computer and to a graphic workstation respectively. On MR image processing, organs were extracted semiautomatically by thresholding enclosed areas. After several steps of image processing, three-dimensional anatomy of each anomaly was visualized with emphasis on position and shape of the muscle complex. In control patients without an anomaly, images showed that the rectum is supported by the levator muscle from behind and descends along with the urethra. In the male patient, the anal canal separates from the urethra and penetrates through the middle of the sphincter complex to reach the orifice. In those with low-type anomaly with a fistula opening to the perineum or the vestibule, images showed the fistula descending through the anterior portion of the sphincter complex. Images of those with a rectourethral fistula could show the muscle complex behind the rectum and at the region where the external sphincter should be. In those with cloacal anomalies, anatomical position and the shape of three different viscera were easily recognized, and the muscle complex was shown like that of rectourethral-type anomalies. This study is a new approach to the anomaly to facilitate understanding it and can assist a surgeon in planning a procedure. This kind of application would make it possible for a surgeon to consider the strategy on a display screen before the real surgery.

Modified slide tracheoplasty for congenital tracheal stenosis

Slide tracheoplasty has become a standard procedure to treat long-segment congenital tracheal stenosis because it is a reasonable and simple technique. Slide tracheoplasty does not affect the carina during long-segment congenital tracheal stenosis management, and thus lesions of the carina, such as stenosis and tracheobronchomalacia can become important causes of extubation failure after surgery. In this manusript, we describe the effectiveness of our modified slide tracheoplasty, which includes reconstruction of the carina. We have performed this technique on three patients, all of whom were extubated without developing any respiratory symptoms.