Theodore Z. Polley

Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnatal presentation, surgical indications, and natural history

BACKGROUND/PURPOSE Regression of a cystic adenomatoid malformation (CAM) in a fetus is well described. Little, however, is known about the postnatal course of these infants. This study attempts to correlate the prenatal course of CAMs with postnatal symptoms, radiological manifestations, and need for surgery. METHODS The clinical course of patients with a CAM diagnosed prenatally were retrospectively reviewed. Inclusion in the study required a prenatal ultrasound scan documenting a CAM. RESULTS Over 10 years, 14 patients with a CAM were diagnosed prenatally. Six (43%) showed a partial in utero regression. Four patients were symptomatic at birth and underwent a resection as newborns. Ten patients were asymptomatic at birth, and eight of these had normal chest x-rays. Elective resection has been performed in 3 of these 10, and two additional children are scheduled to undergo an excision near 1 year of age. The remaining five patients have undergone follow-up nonoperatively for a mean of 36 +/- 15 months. Of the seven asymptomatic patients not undergoing immediate surgery, only one has shown a slight postnatal regression, despite five of these showing regression in utero. None have become symptomatic. CONCLUSIONS The results suggest that regression of a CAM on prenatal ultrasound scan is common, but this process does not continue after birth. A normal chest x-ray does not indicate complete regression of a CAM; a computed tomography (CT) scan is required to evaluate such patients, and will generally demonstrate a CAM. Asymptomatic patients with a CAM may be followed up nonoperatively with no apparent adverse effects. The decision and timing of an excision in an asymptomatic patient remains controversial among pediatric surgeons.

Reversal of mortality for congenital diaphragmatic hernia with ECMO.

Extracorporeal Membrane Oxygenation (ECMO) has been available to neonates with respiratory failure at the University of Michigan School of Medicine since June 1981. In order to evaluate the impact of this type of pulmonary support, a retrospective analysis of 50 neonates with posterolateral congenital diaphragmatic hernia (CDH) who were symptomatic during the first hour of life and were treated between June 1974 and December 1987 was carried out. The patients were divided into two groups, those treated before June 1981 (16 patients) and those treated after June 1981 (34 patients). Overall survival improved from 50% (eight of 16 patients) during the pre-ECMO era to 76% (26 of 34 patients) during the post-ECMO period (p = 0.06). During the period after June 1981, 21 neonates were unresponsive to conventional therapy and were therefore considered for ECMO. Failure of conventional therapy was defined as acute clinical deterioration with an expected mortality of greater than 80% based on an objective formula previously reported. Six patients were excluded on the basis of specific contraindications to ECMO. Thirteen of 15 infants (87%) supported with ECMO survived. Three patients treated before 1981 met criteria for ECMO; all three died while receiving treatment using conventional therapy. These survival differences are significant (p less than 0.01). In addition, the survival of 87% for the infants treated with ECMO versus the expected mortality of greater than 80% for these same patients when treated with conventional therapy is highly significant (p less than 0.005). Based on this data, ECMO appears to be a successful, reliable, and safe method of respiratory support for selected, critically ill infants with CDH.

The development of necrotizing enterocolitis following repair of gastroschisis: A suprisingly high incidence

We recently observed the development of necrotizing enterocolitis (NEC) in two consecutive newborn infants after gastroschisis repair. Because this association was unexpected, a retrospective review of our 11-year experience was performed using a multivariant computer analysis. The cohort consisted of 54 newborns with gastroschisis. All infants with omphalocele were excluded. Ten of 54 infants (18.5%) developed NEC as defined by classical clinical findings and pneumatosis intestinalis. Twenty-one distinct episodes of NEC occurred with up to three episodes (mean, 2.1) per patient. Twenty of the 21 episodes were successfully treated nonoperatively. Two infants developed pneumoperitoneum, one of whom underwent laparotomy upon which no perforation or intestinal infarction was found. Eight of the ten patients survived--a survival rate no different than for the remainder of the study group. Neither of the two deaths was attributable to NEC. The NEC was atypical in that no significant relationship was established with known predisposing conditions such as prematurity or low Apgar scores. Suspected risk factors such as time of feeding, type of closure, type of formula, total parenteral nutrition (TPN), and composition of TPN were not statistically related. Significant associations included concurrent TPN associated cholestatic liver disease in nine of the ten infants, antecedent intestinal surgery other than abdominal wall closure in five of the ten infants, and delay in initiation of enteral feedings (greater than 30 days) in eight of ten infants. In addition, the NEC occurred significantly later (range, 32 to 79 days; mean, 52 days) in the clinical course than usual; in fact, three of ten infants were rehospitalized with NEC following discharge. We conclude that a relationship exists between NEC and gastroschisis.(ABSTRACT TRUNCATED AT 250 WORDS)

A ten-year experience with ninety-two cases of Hirschsprung's disease. Including sixty-seven consecutive endorectal pull-through procedures.

From July 1974 through November 1984, 92 patients with Hirschsprungs disease (congenital aganglionosis) have been treated at the University of Michigan-Mott Childrens Hospital. This series includes 67 consecutive modified endorectal pull-through (ERPT) procedures in children. Fifty-one of these 67 patients had standard rectosigmoid disease and underwent a successful ERPT with only two major complications. One of these 51 children underwent a successful ERPT but died in the late postoperative period from severe congenital heart disease. Eighteen of the 92 patients suffered from total aganglionosis or long-segment disease. Sixteen of these have undergone an ERPT with no mortality or operative morbidity. The follow-up ranges from 6 months to 10 years. All of the children who have reached 3 years of age are continent. Of the remaining 23 patients, 12 were referred following an unsuccessful pull-through at another hospital. The 12 operations included five Swenson pull-throughs, five Duhamel procedures, one ERPT, and one subtotal colectomy. It was possible to redo or revise the pull-through procedures successfully in all but one patient, who required a permanent colostomy. Finally, 11 children were referred for management of a variety of complications following pull-through procedures performed at other institutions. None of these 11 patients required a reperformance of their pull-through, and all were successfully treated with lesser surgical procedures or with medical management. The excellent functional results and the low morbidity and zero operative mortality are attributed to the technical ease of performing the modified ERPT.

Laparoscopic diagnosis and excision of Meckel's diverticulum

Meckels diverticulum is one of the primary concerns in the differential diagnosis of the pediatric patient with massive, acute gastrointestinal bleeding, intussusception, or abdominal pain of uncertain cause. The hospital course of two children with Meckels diverticulum, successfully treated by laparoscopic excision, is presented, along with details of the operative procedure. Both patients recovered from the procedure without incident and were discharged at 24 and 48 hours after surgery. The authors believe a laparoscopic approach is safe and effective in the diagnosis and treatment of Meckels diverticulum.

Use of abdominal ultrasonography to assess pediatric splenic trauma. Potential pitfalls in the diagnosis.

OBJECTIVE The purpose of this study was to evaluate the accuracy of abdominal ultrasonography (US) for screening and grading pediatric splenic injury. SUMMARY BACKGROUND DATA The use of abdominal US has increased rapidly as a method of evaluating organ damage after blunt abdominal trauma. Despite USs increasing use, little is known about its accuracy in children with splenic injury. METHODS Children (N = 32) suffering blunt abdominal trauma who were diagnosed with splenic injury by computerized tomography (CT) scan prospectively were enlisted in this study. Degree of splenic injury was evaluated by both CT and US. The ultrasounds were evaluated by an initial reading as well as by a radiologist who was blinded as to the results of the CT. RESULTS Twelve (38%) of the 32 splenic injuries found on CT were missed completely on the initial reading of the US. When the ultrasounds were graded in a blinded fashion, 10 (31%) of the splenic lacerations were missed and 17 (53%) were downgraded. Seven (22%) of the 32 splenic fractures were not associated with any free intraperitoneal fluid on the CT scan. CONCLUSIONS This study has shown that US has a low level of sensitivity (62% to 78%) in detecting splenic injury and downgrades the degree of injury in the majority of cases. Reliance on free intraperitoneal fluid may be inaccurate because not all patients with splenic injury have free intra-abdominal fluid. Based on these findings, US may be of limited use in the initial assessment, management, and follow-up of pediatric splenic trauma.

The prediction of hormonal dependency of mammary cancer

The estrogen receptor protein (estrophilin) was determined in specimens from 359 primary breast cancers and from metastatic tumors of 214 patients. Ninety-eight patients were eventually treated by some form of endocrine therapy: 82 patients were treated by ablative therapy, and 16 by hormonal additive treatment. The records of 81 of the 98 patients whose tumors were characterized for estrogen receptor content and who had received some type of endocrine therapy were reviewed by a peer review group that assessed the objective data for these patients regarding objective remission or failure to treatment. A positive estrophilin determination was defined as receptor content greater than 250 fmole per gram of tumor for premenopausal females and greater than 750 fmole per gram of tumor for postmenopausal and previously castrated patients. Estrogen receptor determinations may be made from tumor specimens as small as 200 mg. Sixty-nine patients underwent some form of ablative therapy. Twenty-seven of these patients were found to have significant receptor determinations. Of those patients in whom a definite determination was made of their post-treatment response, roughly two-thirds experienced objective remissions. None of the 42 patients with negative estrogen receptor detemination experienced an objective remission to ablative endocrine therapy. Only one of 6 patients with negative determinations benefited from additive hormonal therapy; 4 of 6 patients with positive determinations benefited from additive therapy. Not all women with receptor containing mammary cancer will respond favorably to endocrine therapy, but those patients whose tumors lack a critical amount of estrophilin have little chance of benefit from either endocrine ablation or hormone administration. The estrogen receptor content of the primary tumor indicates the hormonal dependency of the tumor and may be used to predict the response to endocrine treatment when recurrent disease appears.

Resolution of splenic injury after nonoperative management

Numerous studies have demonstrated success with nonoperative management of splenic injuries in pediatric patients. However, the resolution of the splenic injury has not been previously evaluated. The records of 50 pediatric patients with splenic injuries from blunt trauma treated nonoperatively between 1984 to 1992 were reviewed retrospectively. Abdominal computed tomography (CT) was performed at the time of injury and 6 weeks postinjury in 25 patients. These scans were reviewed and categorized by a modification of a previously reported grading system for parenchymal injury. All patients had healing of the splenic injuries, with complete resolution of the healing process observed at 6 weeks postinjury in 44%. Even those with shattered spleens (n = 6) had consistent improvement in splenic architecture, with resolution of fractures and/or contusions and return of splenic perfusion. Ten (77%) of 13 grade 1 and 2 injuries were completely resolved by the 6-week follow-up examination, whereas only one (8%) of 12 grade 3 to 5 injuries showed radiological resolution of splenic injuries. None of the 25 follow-up CT scans affected clinical decision-making or led to a deviation from the established protocol, which included a 3-month period of reduced activity. All 50 patients did well, without evidence of morbidity, mortality, or complications after return to full activity 3 months postinjury.(ABSTRACT TRUNCATED AT 250 WORDS)

Surgical management of ambiguous genitalia in the infant and child

Proper gender assignment to a neonate born with ambiguous genitalia is a social emergency of the newborn period. Once an appropriate sex assignment has been made, the next critical step is performance, if needed, of a reconstructive procedure in a timely fashion. In an attempt to evaluate our experience with this unique group of patients, we have retrospectively reviewed the course of 69 children with ambiguous genitalia managed surgically at this institution between 1974 and 1989. This series consists of 32 genotypic females with congenital adrenal hyperplasia (CAH), 10 children with mixed gonadal dysgenesis (MGD), 10 male pseudohermaphrodites, 3 true hermaphrodites, 8 genotypic females with urogenital sinus anomalies (UGS), and 6 genotypic males with bilateral undescended testes and penoscrotal hypospadius. All newborns initially seen at this institution received proper sex assignment within the first week of life. Prior to 1980, 17 of the children with CAH underwent clitorectomy and vaginoplasty and three underwent clitoral recession and vaginoplasty. After 1980, 10 patients with CAH were managed with clitoral recession and vaginoplasty and 2 with vaginoplasty alone. Eight of 10 cases of MGD were given a female sex assignment and all 8 underwent gonadectomy due to the high risk of gonadoblastoma; the other 2 children were raised as males. There were 3 true hermaphrodites of which 2 were assigned female gender roles and were managed with a clitoral recession and vaginoplasty. All 10 male pseudohermaphrodites were raised as females and all underwent bilateral orchidectomy. The 8 children with UGS were raised as females and underwent vaginal reconstruction.(ABSTRACT TRUNCATED AT 250 WORDS)

Splenic artery embolization for post-traumatic splenic artery pseudoaneurysm in children.

Although rare, traumatic splenic artery pseudoaneurysm (SAP) can be life threatening. The diagnostic approaches as well as the methods of treatment of SAP are yet to be determined. We present the case of a 10-year-old boy treated conservatively for a grade III blunt splenic injury (BSI). The child was discharged to home after a 5-day uneventful hospitalization but was found on routine follow-up CT scan to have a large SAP. The pseudoaneurysm was successfully angiographically embolized and subsequent abdominal CT demonstrated successful resolution of the pseudoaneurysm with a small residual splenic cyst. We reviewed the eight cases of post-traumatic SAP in children that have been published in the English literature. Unlike SAP in adult patients, the severity of the splenic injury does not have predictive value for development of SAP in children. Abdominal pain was the most frequent symptom of SAP, but three children were asymptomatic at the time of diagnosis. Therefore, the possibility of SAP should be investigated even in the asymptomatic child with mild splenic injury. When a splenic pseudoaneurysm is diagnosed, we believe splenic artery embolization is indicated.