Lorna Spagnol

The effect of glossopexy on weight velocity in infants with Pierre Robin syndrome

AIM In infants with Pierre Robin syndrome (PRS), mandibular distraction may be more advantageous than glossopexy as it not only relieves oropharyngeal airway obstruction but also reverses body growth retardation. Because no data are available on body weight velocity after glossopexy, we assessed longitudinally the body weight velocity in a cohort of children undergoing glossopexy. METHODS The records of 48 infants with PRS undergoing glossopexy after unsuccessful nonoperative treatment between 1981 and 2005 were reviewed. Weight measurements were analyzed at 4 time-points: at birth, on admission for glossopexy, on admission for lysis of lip-tongue adhesion (TLA), and at follow-up. Weight velocity was assessed using Tanners tables. MAIN RESULTS Adhesion dehiscence occurred in 9 patients (18.7%). Lip-tongue adhesion resolved airway compromise in 36 infants (75%). Release of TLA was accomplished in 34 patients. Data on weight velocity from birth to follow-up (mean, 5.57 +/- 0.59 years) were available for 31 patients. After glossopexy, mean body weight increased from the 9.7 +/- 2.6th to the 17.5 +/- 4.6th percentile (P > .05), whereas mean weight velocity increased from the 19.1 +/- 4.9th to the 74.2 +/- 4.7th percentile (P < .001). No temporal correlation was found between glossopexy and oropharyngeal dysphagia. CONCLUSION In infants with PRS, glossopexy is a valid alternative to mandibular distraction because it does not cause decline in body growth.

The fate of prenatally diagnosed primary nonrefluxing megaureter: do we have reliable predictors for spontaneous resolution?

OBJECTIVES To compare predictive values of current morphologic parameters with congenital renal damage associated with severe megaureter. METHODS A retrospective analysis was performed using records of 37 patients (50 megaureters) referred before birth for a primary megaureter. Mean follow-up was 26 months (range, 1 to 8 years). RESULTS Dilatation resolved spontaneously in 46 of 50 ureters. Only 4 of 37 patients required surgery (10.8%) after a mean follow-up of 58 months (range, 32 to 80 months). Average time to resolution was 24 months (range, 1 to 82 months) and was independent from sex, side, and bilaterality. A weak correlation was found with initial anteroposterior pelvic diameter, ureteral diameter, and separate function at renogram. A significant correlation (P <0.02) was found between megaureter type and time elapsed to spontaneous resolution. As far as differential function was concerned, mean values were significantly lower among type III megaureters, which had the lowest rate of resolution. CONCLUSIONS The fate of severe megaureter seems strongly influenced by congenital renal damage secondary to a developmental abnormality of the ureteric bud. A poor resolution rate has to be expected in these cases; surgery must be reserved for symptomatic cases but has no influence on pre-existing renal damage.

The functional sequelae of sacrococcygeal teratoma: a longitudinal and cross-sectional follow-up study

AIM The purpose of the study was to determine clinical manifestations, prevalence, and natural history of functional sequelae in patients operated on for sacrococcygeal teratoma (SCT) during infancy. METHODS The medical records of 18 infants operated on for SCT were reviewed. Data recorded during admission and during routine outpatient appointments were analyzed. Moreover, 13 surviving patients (mean age, 25 years) and 65 age- and sex-matched controls were evaluated at a special outpatient clinic. RESULTS Seven adult patients and 25 controls presented with one or more functional disorders including abdominal pain, constipation, diarrhea, soiling, enuresis, urinary frequency, urinary stress incontinence, and urinary straining. In 7 of 8 symptomatic children, one or more functional disorders disappeared during adult life. In comparison with controls, adult patients had a nonsignificant higher prevalence of each individual dysfunction. CONCLUSIONS Sequelae of SCT tend to improve with time, and the disorders reported are relatively common complaints in the general population.

The eyelid crease approach to angular dermoid cysts in pediatric general surgery

PURPOSE We recently adopted an approach described by eyelid surgeons for angular dermoid cyst excision. The aim of this study was to compare the outcome of this technique with that accomplished through a conventional trans-eyebrow incision. METHODS Prospective nonrandomized study of 34 consecutive children treated between January 2000 and December 2006. Twenty-six patients (group I) were operated on through a trans-eyebrow incision, whereas 8 (group II) received an upper eyelid crease incision. Performance measures included cosmesis, operative time, and complications. Cosmesis was assessed blindly with comparative photographs, using a 100-mm visual analog scale. RESULTS No significant differences were found between groups I and II with regard to age at surgery (22 +/- 17 vs 24 +/- 4 months; P = .07) and operative time (42 +/- 21 vs 40 +/- 16 minutes; P = .9). In group II, the scar resulted invisible when the affected side was assessed with the eye open and still significantly better than group I, when evaluated with the eye closed (96 +/- 7 vs 81 +/- 18 mm; P = .0001). All patients and their families reported great satisfaction and an excellent scar. There were neither major postoperative complications nor recurrence of the lesion. CONCLUSIONS Angular dermoid cyst excision using an eyelid crease approach yields excellent cosmesis and represents a safe, simple, and easily reproducible procedure in pediatric general surgical practice.

Preoperative administration of Sudan III and successful treatment of persistent chylous ascites in a neonate

Congenital chylous ascites is a rare entity, and surgical treatment is confined to selected intractable cases. We report 2 cases of refractory congenital chylous ascites successfully treated with preoperative administration of lipophilic dye (Sudan III) followed by abdominal systematic surgical exploration, cauterization, and fibrin glue application.

Delivery in a tertiary Center with co-located surgical facilities makes the difference among neonates with prenatally diagnosed major abnormalities

Objective: Impact of prenatal diagnosis (PD) of major abnormalities on neonatal outcome is matter of debate. Unfortunately cases with and without PD may sometimes not be comparable. This is generally related to a lower maturity (GA) and weight (BW) secondary to a high rate of preterm cesarean sections (CSs) for clinical convenience. Present study tried to find out if in utero transfer to a Center with co-located surgical facilities reduces these potential risk factors. Methods: 152 cases with prenatally detectable conditions were studied and divided according to PD; the following data were compared: GA, BW, obstetrical complications, associated malformations, mode and site of delivery, outcome. Cases with PD delivered in our Center (Inborn, IB) or transferred after birth (Outborn, OB) were compared. Results: 61 cases had a PD (IB/OB ratio 34/27); GA and BW were lower respect to no-PD cases and a higher CS rate was found among OB cases, not justified by complicated pregnancies. No differences in outcome were observed. Conclusions: Elective preterm CS is still largely practiced for fetuses with PD of a major congenital anomaly in Centers without co-located surgical facilities either in the presumption of safer delivery or to facilitate postnatal transfer. This leads to a lower GA and BW and may spoil potential impact of PD on outcome. This may be avoided promoting prenatal transfer to a Center with co-located surgical facilities.

Conservative management of cervicomediastinal chyloma in an infant

The accumulation of chyle into the mediastinum after surgical intervention is considered an extremely rare event, which has been previously described only in adulthood. We report on a 9-month-old boy who developed a cervicomediastinal chyloma after excision of a large left-sided supraclavicular cystic hygroma. Notwithstanding considerable tracheal deviation, the infant remained asymptomatic and was successfully treated by nonoperative treatment. Professionals should be aware of such an unusual condition, which may benefit from expectant management when evaluation establishes a high likelihood of clinical diagnosis, and patients general condition remains stable. This approach may avoid, with little risk, unnecessary early surgical intervention in some of these patients.

Fetal Adrenal Cysts with Hemorrhagic Appearance May Be Cystic Neuroblastomas

Nonetheless, there is a unanimous consensus that cystic neuroblastoma is a particularly benign condition likely to stabilization or spontaneous regression during the first months of life. The exceeding number of prenatally diagnosed lesions over those identified in postnatal life lends further support to the hypothesis that cystic neuroblastoma may undergo spontaneous regression. Therefore, we believe that prenatally diagnosed adrenal cystic masses may be safely managed expectantly, regardless of their origin. Such a policy avoids, with little risk, potential morbidity related to unnecessary general anesthesia and abdominal surgery in these little patients. We read with great interest the article by Schrauder et al. [1] reporting on a neonate with a prenatally diagnosed adrenal cystic mass, whose clinical course and sonographic signs were described as ‘typical’ for adrenal hemorrhage. Although we fully agree with the waitand-see strategy adopted postnatally, we would like to point out that prenatal adrenal hemorrhage might correspond to the more frequently reported cystic neuroblastoma [2, 3] . Differentiation between these two clinical entities may be very difficult without pathological study and is rendered more complicated by the fact that urinary catecholamines are not raised in prenatal cystic neuroblastomas [2, 3] . Additionally, some infants with adrenal cystic lesions have had neuroblastoma with intracystic hemorrhage [4] . Received: November 7, 2008 Accepted after revision: November 14, 2008 Published online: September 22, 2009

Prenatal Diagnosis of Duodenal Obstruction Selects Cases with a Higher Risk of Maternal-Foetal Complications and Demands in utero Transfer to a Tertiary Centre

Objectives: It was the aim of this study to determine if prenatal diagnosis of congenital duodenal obstruction (CDO) selects high-risk pregnancies and demands special perinatal attention. Methods: Medical records of 62 neonates with intrinsic CDO, admitted since 1981 in 2 institutions, were reviewed and divided into 2 groups: 39 cases, detected in utero by a prenatal ultrasonogram (group A), and 23 diagnosed at birth (group B). Prevalence of complete CDO, mean gestational age, mean birth weight, hydramnios, as well as maternal-foetal complications requiring emergency Caesarean section and associated with premature delivery were compared in the 2 groups. Results: A complete CDO was found in 77% of patients in group A versus 48% of patients in group B (p < 0.02). Differences in mean gestational age and mean birth weight in the 2 groups were non-significant. Prenatally diagnosed patients presented a higher prevalence of hydramnios, maternal-foetal complications and premature delivery. Conclusions: Prenatal diagnosis selects patients with complete CDO and hydramnios. These pregnancies present a high incidence of maternal-foetal complications, which may require an emergency Caesarean section, and are frequently associated with premature delivery. These aspects must be considered in prenatal counselling. In utero transfer to a tertiary centre for delivery and appropriate perinatal care should be recommended.