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Purpose: The Nissen fundoplication fails to control gastroesophageal reflux (GER) in up to 25% of children with neurological impairment or chronic lung disease. The uncut Collis modification lengthens the intraabdominal esophagus, improving the antireflux function without opening the stomach. This study reviews the results of the uncut Collis-Nissen fundoplication in a pediatric series., Methods: Seventy-nine children had an uncut Collis-Nissen fundoplication performed over a 5-year period. The median age was 1.4 years. Associated problems included neurological impairment (77%), chronic lung disease (38%), and esophageal atresia (3%). Surgery was undertaken only in children with objective documentation of pathological GER, who had GER complications unresponsive to medical treatment. The usual complications that led to surgery were pulmonary (73%), esophagitis (67%), or failure to thrive (35%). Liquid gastric emptying was assessed routinely preoperatively, and was delayed in 42% patients who then had concomitant pyloroplasty., Results: GER was controlled in 97% of patients after a median follow-up of 1.8 years. All children with recurrent symptoms were restudied, and only two children had documented recurrent GER. One of these required a repeat fundoplication. Thirty-three percent were on promotility medication for feeding difficulties, gagging, or retching. There were postoperative complications in 26% (minor 23%, major 3%) and one postoperative mortality. Eleven late deaths were unrelated to surgery or GER., Conclusion: The uncut Collis-Nissen fundoplication provides excellent control of GER in children and is associated with acceptable morbidity and low mortality. It should be particularly considered in children with neurological impairment or chronic lung disease.

The NIMH Treatment Strategies in Schizophrenia (TSS) collaborative study group investigated the efficacy of antisychotic drug maintenance strategies involving reduced medication exposure in interaction with applied and supportive family management for the long-term treatment of schizophrenia. Therapy was provided at five centers by 25 clinicians who did not participate in the development of the therapies. They were trained by two of the authors, I.R.H.F and C.W.M, in applied family management, a homebased treatment derived from the behavioral family therapy developed by them. Clinicians' characteristics, selection, and training methods, as well as patient rehospitalization rates, are reported for the two family management conditions. The TSS study represents a bridge between the development of a novel therapy and its dissemination in general clinical practice.

Severe caustic injury with stricture may require esophageal reconstruction. The available methods of esophageal substitution do not satisfactorily replace the peristaltic and antireflux properties of the native esophagus. The authors report the results of two children treated with colon patch esophagoplasty and preservation of the injured esophagus. Both were 2 years of age when they accidently swallowed lye. Long esophageal strictures developed, which were resistant to repeated dilatation. Eleven and 13 months after the injury, the patients underwent colon patch esophagoplasty. A segment of the left colon was tailored to the length and shape of the opened stricture and was anastomosed side-to-side to the esophagotomy. One child later had a short recurrent stricture and diverticulum, which required revision of the distal end of the anastomosis. Follow-up endoscopic examination has shown healing of the esophagus and resolution of the strictures; both children are eating normally 3 years after surgery, without symptoms of gastroesophageal reflux. Colon patch esophagoplasty is an alternative to esophageal replacement for long caustic esophageal strictures. Its advantage is preservation of the peristaltic and antireflux mechanisms of the conserved esophagus.

Environmental stress may contribute to the clinical morbidity of established cases of schizophrenia treated with optimal neuroleptic drugs. A family-based approach that aimed to enhance the problem-solving capacity of the index patient and his family caregivers was compared with a patient-oriented approach of similar intensity over a two-year period. Thirty-six patients who returned to stressful parental households after florid episodes of schizophrenia (CATEGO and DSM-III) were stabilized with optimal neuroleptics before being randomly assigned to family or individual therapy in a comprehensive community management program. After nine months, family-managed patients had fewer exacerbations of schizophrenia, lower ratings of schizophrenic psychopathology, fewer hospital admissions, and a trend toward lower deficit symptoms and reduced neuroleptic dosage. This reduced clinical morbidity was sustained throughout the second year of less intensive follow-up. The relative efficacy of the family approach in this clinical management study did not appear to be due to prognostic factors, rater bias, stressful life events, or the effectiveness of pharmacotherapy. Definitive tests of these findings with respect to efficacy require further well-designed studies.

Radiation injury to arteries can represent a significant complication of therapeutic irradiation, even when the dosage used has not been excessive as judged by approved protocols. Children in whom therapeutic abdominal irradiation has been used should be monitored indefinitely for the development of hypertension. The presence of hypertension in such children with normal blood urea nitrogen (BUN) and creatinine, and without proteinuria, should prompt investigation for a renovascular lesion. Standard bypass procedures are usually effective, although the long-term success may be compromised by continuing changes in affected vessels.

Malrotation of the intestine may become symptomatic in the older child and may manifest itself in atypical presentations. Older children may present with symptoms of less than 72 hours duration which are typical of acute duodenal obstruction. More frequently, however, the older child with malrotation will present with chronic abdominal pain with or without vomiting or chronic diarrhea. The diagnosis of malrotation should be considered in any child with intermittent abdominal pain, vomiting, diarrhea, or malabsorption. Surgical intervention is curative and should be implemented as soon as possible after the diagnosis is made.

Five of 22 infants operated upon in the last 4 yr for gastroschisis had an associated loss of intestinal continuity. Four of the infants had an intestinal atresia and one had a gangrenous segment of ileum. Each had primary closure of the gastroschisis and decompression of the proximal intestine. Four had cutaneous enterostomy and one had long tube intubation. Four infants were returned to the operating room between 2 and 4 wk of age and their intestinal continuity was established. At the second operation the intestine, which had presented as a matted mass at birth, appeared normal with resolution of the serosal edema and few adhesions. The length of the intestine that initially had appeared shortened may be more accurately evaluated and is usually considerably greater than was appreciated at birth. If distal atresias are present, they will become apparent. A primary anastomosis can be carried out using the principles of repair for an isolated intestinal atresia in the newborn. All five of these infants have survived.

In the aftermath of deinstitutionalization, when current public policy dictates greater community management of schizophrenia, patients and their families play an increasing role in the treatment of the illness. Since mental health professionals often neglect to educate them on the causes, symptoms, and treatment of schizophrenia, patients and their families are often misinformed, fearful, and ill-equipped to aid in treatment. The authors describe a brief family educational intervention and the questionnaire that was used to compare the knowledge of patients and their families who received the intervention with the knowledge of those who did not. The findings suggest that patients and their families in family treatment programs can acquire and retain information about schizophrenia more readily than patients and families involved in individual treatment. This knowledge enables them to become more effective participants in aftercare and helps surmount the problems associated with posthospital adjustment.

Fourteen infants with intestinal stenosis as a late sequela of necrotizing enterocolitis were treated at Texas Children's and Ben Taub General Hospitals from 1972 to 1979. Barium enema studies are the keystone in making the diagnosis and should be performed in any infant with abdominal distention or poor feeding after recovery from acute necrotizing enterocolitis. Spontaneous resolution of stenosis is a definite clinical entity, and nonobstructed infants should be given a trial for resolution. This trial should be for a limited time. The operative therapy for the infant with obstruction or the infant who fails to have spontaneous resolution of stenosis must be individualized. Resection with primary anastomosis best serves those infants with limited stenotic lesions, minimal dilatation of the intestinal lumen and no other complicating medical problems. Intestinal diversion with later resection and enterostomy closure should be reserved for infants with intestinal obstruction and complicating medical problems.

Necrotizing enterocolitis (NEC) usually occurs in low birth weight infants who have had perinatal stress, and the mortality remains significant. There are a few reports of NEC in the postoperative period, especially in young infants. Nine neonates developed NEC following operations and form the basis of this report. The interval between operation and the diagnosis of NEC varied from 3 days to 4 mo. The surgical lesions included one case each of esophageal atresia, tetralogy of Fallot, supralevator rectal atresia with rectourethral fistula, and multiple intestinal atresias. Three babies had gastroschisis and two had "apple peel" intestinal atresia. Only 3 of the 9 survived. The usual clinical findings of NEC, abdominal distention, bile stained gastric residuals and diarrhea (with or without blood), can occur in the postoperative period without NEC and are, therefore, not reliable diagnostic signs. Significant changes in the clinical course of these babies occurred from 7 hr to 5 days before the diagnosis was established. In these patients the roentgen findings that established the diagnosis of NEC included intestinal ileus, pneumatosis intestinalis, and portal vein gas. Pneumatosis intestinalis and portal vein gas were the most reliable diagnostic signs, but appeared relatively late in the course of the disease. In one case pneumatosis was seen only in retrospect. None of the patients had definite pneumoperitoneum. Awareness of NEC as a potential postoperative complication may result in early recognition, treatment and survival.

One hundred patients with complications of severe gastroesophageal reflux were treated surgically by the Thal fundoplication. In all patients the symptoms of reflux were eliminated by the operation, although 4 recurred within 8 months. Two of these were due to disruption of the fundoplication and two were due to hiatus hernia not recognized and repaired at initial operation. There were 8 deaths, none related to gastroesophageal reflux or the operation. Four patients required re-operation for intestinal obstruction. The Thal fundoplication is a simple procedure which fixes the distal esophagus within the abdomen and produces an acute angle of His. It is effective in prevention of reflux and the patient is able to burp and vomit if necessary. It has not been associated with dysphagia or "gas bloat" which may follow the Nissen fundoplication.

Morgagni hernias during infancy are associated with significant respiratory symptoms as well as other congenital anomalies, particularly congenital heart disease. During the past 6 years we have had the opportunity to treat five infants less than 1 year of age with Morgagni hernias. A review of the literature revealed an additional seventeen detailed case reports of infants with Morgagni hernias. Each of our patients was symptomatic as were all but two of those previously reported. These patients presented with tachypnea, dyspnea, and cyanosis which was very similar to the presentation of Bochdalek hernias seen during infancy. Thirteen of 16 patients in whom detailed descriptions of associated anomalies were given including four of our own patients, had significant congenital anomalies. Nine involved the heart, including three infants with dextracardia, three with ventricular septal defects, and two with anomalous pulmonary venous return. Five infants were retarded, including three with Trisomy 21. Two of the previously reported patients had large omphaloceles and other stigmata of Cantrell's syndrome. All but one of the patients reported, including ours, had a hernia sac. The liver, colon, and small bowel were most commonly found in the hernia sac; however, the presence of the stomach and spleen have been reported. While both the transabdominal and transthoracic approaches to Morgagni hernias have been advocated and, indeed, repair of the defect can be accomplished through both approaches, the transthoracic approach allows better exposure of incarcerated viscera, particularly the frequently found anomalous left lobe of the liver.

The efficacy of surface cooling and intraluminal antiseptic solutions for short term preservation of intestinal segments in preparation for free tissue transfer were investigated. Five segments of the small intestine and five segments of the colon were allocated to each group as follows: group 1, ischemia at 37 degrees C.; group 2, ischemia at 6 to 12 degrees C., and group 3, ischemia at 6 to 12 degrees C. plus povidone-iodine intraluminally. Histologic grading of segments allowed assessment of the efficacy of short term protection against ischemic changes. Surface cooling effectively prevented histologic evidence of ischemia. No additional benefit was observed using povidone-iodine intraluminally. Significant changes in tissue water content and evidence of bacterial invasion were not observed.

Effective community treatment of schizophrenia involves not merely the removal of florid symptoms, but restoration of effective social role functioning. The efficacy of a family management approach is compared with an individual approach of similar intensity in terms of its impact on the patient's social adjustment after a florid episode of schizophrenia. Clinical reports, patient self-reports, and interviews with the family members of 36 patients, who were randomly assigned to family or individual management, demonstrated a consistent superiority for family management. The advantages for the family approach were sustained over a two-year period. The potential mechanisms through which the family approach may have achieved its greater efficacy are discussed from a multi-determined perspective.

The experience of 73 consecutive infants with gastroschisis and omphalocele is reported. The overall survival rate was 80 percent; however, since 1973 the survival rate for ruptured and intact omphaloceles has been 87 percent and 93 percent for gastroschisis. This remarkable reduction in mortality has been attributed primarily to the advent of total parenteral hyperalimentation, but mortality has also decreased due to the use of pediatric respirators which overcome the effects of increased intraabdominal pressure, and the creation of the neonatal intensive care unit where monitoring of these often fragile infants and the presence of specialty personnel assist in their care.

Primary anastomosis following the resection of bowel for necrotizing enterocolitis (NEC) can be done with acceptable morbidity and mortality rates. Twenty-seven patients are reported with three deaths. Primary anastomosis will avoid problems seen with small-bowel stomas. The concomitant ligation of a significant patent ductus arteriosus (PDA) should be considered for patients undergoing abdominal procedures for NEC.

This report discusses the safety of 89 cuffed, Silastic (Dow Corning, Midland, MI) catheters placed in children between the ages of 1 and 18, a group that may be considered high risk on the basis of age. The overall complication rate was once every 288 days, three times more often than in published results in adults, but less often than in other pediatric series. The sepsis rate of once each 1236 days was almost twice the adult rate. Thirty (35%) of the catheters were used at home, accounting for 85 per cent of the total catheter use. Among this group, the overall complication rate was only once every 815 days, three times less frequent than in comparable adult series. The complication rate due to sepsis was once each 2444 days, the same as noted in adults. Whereas these catheters may be more hazardous overall in this age group, home usage appears to be safer than expected. These catheters are well accepted by this age group and their use, particularly among outpatients, should not be restricted for fear of an unacceptably high complication rate.

Sixty consecutive patients with anorectal agenesis were seen during a ten year period. Among 41 boys and 19 girls, there were 35 supralevator and 25 infralevator abnormalities; associated anomalies were present in 38 patients. The over-all mortality was 23 per cent. A simplified classification of anorectal agenesis, based on therapeutic considerations, is proposed. It consists of four basic groups: male high and low and female high and low. A low lesion is defined as having a visible opening or membrane covered opening in the perineum in the male or a visible opening in the introitus or perineum in the female. These infants are managed with simple dilations of the fistula; definitive perineal anoplasty, if necessary, is deferred until the child is six months of age or older. All other lesions are defined as high lesions and are treated in the newborn period with a colostomy. Sacroperineal or sacroabdominoperineal rectoplasty is deferred until the infant is six to 12 months of age. The use of this classification as well as the judgments it implies in the timing of operative procedures will avoid dangerous perineal dissections in the newborn.

In this review of 50 children with gallbladder disease in Houston, Texas, there were an equal number of patients with and without hemolytic disease and a slight predominance of males in both groups. Nearly all had been symptomatic for a prolonged period before diagnosis and cholecystectomy. Common early diagnoses were hemolytic crisis and appendicitis. Ultrasonography has become a very accurate tool for the preoperative evaluation of these children. Once the diagnosis of cholelithiasis is established, elective cholecystectomy is the treatment of choice.

Families of schizophrenic patients frequently are ill equipped to handle the primary caretaking responsibilities that have fallen to them due to deinstitutionalization. The authors describe a program begun at a medical center in Southern California in 1978 to assist schizophrenic patients living at home and their families. The program, which consists of family therapy sessions in the home, focuses on education about schizophrenia, development of communication skills, and acquisition of structured problem-solving and other behavioral strategies. Preliminary findings of a controlled study of program participants suggest that the family interventions are both cost-effective and highly successful in reducing the incidence of relapse and hospitalization.

Leiomyoblastomas are rare smooth muscle tumors occurring most commonly in the gastrointestinal tract of adults. We report the unusual occurrence of a gastric leiomyoblastoma in a child who presented with refractory iron deficiency anemia. The tumor had a superficial erosion which was apparently the source of intermittent hemorrhage resulting in anemia. Epithelioid cells, spindled cells, and cells arranged in a perithelial pattern were seen by microscopy, characteristic of a leiomyoblastoma. Immunohistochemical staining demonstrated muscle actin, vimentin, desmin, and alpha-1-antichymotrypsin, and electron microscopy showed focal densities alternating with thin filaments. All of these features help to differentiate the tumor from an inflammatory pseudotumor. Leiomyoblastomas are associated with paragangliomas and pulmonary chondromas in children, but these were not present in this child. The prognosis of leiomyoblastomas may be related to tumor size, mitotic rate, and cellular characteristics.

In children, psoas abscess does not head the list in the differential diagnosis of the child who presents with a limp or lower abdominal pain. Therefore, the road to this diagnosis can be long and complicated leading to numerous studies and specialty consultations. Over a 7-year period, seven psoas abscesses have been drained surgically. All were Staphylococcal though one was mixed. In each case, the original admitting diagnosis was that of septic arthritis of the hip. In general, this diagnosis was ruled out by negative hip aspirations and bone scans. Often, the severity of symptoms led to persistent evaluation with noninvasive tests such as gallium scan, intravenous pyelogram, or barium enema. Though these tests were often suggestive, a positive ultrasound or CT scan was the key studies diagnostic enough to warrant surgical exploration and drainage. During this time period, there have been no negative explorations for psoas abscess. Upon surgical drainage, all patients improved, with subsequent recovery of hip function. The child who presents with a limp or painful hip should be considered for ultrasonography or computerized tomography once hip pathology is ruled out. We feel that the results of other tests such as gallium scan, IVP, or barium enema are not sufficiently specific to indicate surgery.

Environmental stress has been implicated as an important factor in the relapse of schizophrenic patients receiving optimal drug therapy. In a randomized controlled study, we compared at-home family therapy with clinic-based individual supportive care in the community management of schizophrenia in 36 patients taking neuroleptic maintenance medications. The family-treatment approach sought to enhance the stress-reducing capacity of the patient and his or her family through improved understanding of the illness and training in behavioral methods of problem solving. The results at the end of nine months revealed the superiority of this approach in preventing major symptomatic exacerbations. Only one family-treated patient (6 per cent of all patients) was judged to have had a clinical relapse, as compared with eight patients (44 per cent) treated individually. Family-treated patients averaged 0.83 days in the hospital, as compared with 8.39 days for the comparison group. Significantly lower levels of schizophrenic symptomatology on blind rating-scale assessments supported these clinical observations of the superiority of family management.

Recent studies indicate a favorable outcome with bilateral Wilms' tumor. From 1971 to 1985, ten children between the ages of 6 months and 5 years were treated for this disease. During the early part of the series, five patients had nephrectomy on one side and partial nephrectomy on the other side, and one patient had bilateral partial nephrectomies at the same operation. More recently four patients initially had a biopsy of both tumors and lymph node sampling followed by chemotherapy. At the second-look procedure two patients had multiple biopsies because there was no obvious tumor. Histologically there was no tumor on the third-look procedure in these two patients. Nine patients had a favorable histology of the Wilms' tumor. One patient had a favorable histology on one side and an unfavorable type of histology on the other side. Eight patients are surviving between 6 months and 13 years. Two died of extensive disease within 16 months of diagnosis. One patient had an unfavorable histology. The good results following partial nephrectomies have led us to attempt to conserve additional tissue, as has been done in the last four patients. Our early results suggest biopsy of the tumor followed by chemotherapy, then a second look, and if necessary, third-look procedures may result in preservation of functioning renal tissue.