Francesco Cozzi

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.

Pattern of cardiovascular anomalies associated with esophageal atresia: support for a caudal pharyngeal arch neurocristopathy.

Patients with cephalic neurocristopathy (an abnormality of neural crest differentiation) present a striking pattern of associated cardiovascular anomalies (CVA). Therefore, to support the hypothesis that esophageal atresia (EA) may be related to a defective contribution from the cephalic neural crest, we studied the pattern of CVA associated with EA. Medical records of 99 patients with isolated EA, 101 with isolated anorectal malformations (ARM) and 15 with both EA and ARM, consecutively admitted to our unit, were reviewed. The prevalence and pattern of CVA associated with isolated EA or isolated ARM were compared on the assumption that the cranial or caudal location of a major malformation is related to a different regional patterning of associated anomalies. The prevalence of CVA was 39% in patients with isolated EA and 7% in those with isolated ARM (p < 0.01). Neural crest–related CVA (aortic arch anomalies, conotruncal defects, and superior vena cava malformations) accounted for 72% of all CVA in patients with isolated EA versus 14% in those with isolated ARM (p < 0.02). In patients with isolated EA, anomalies of the fourth and sixth aortic arch derivatives accounted for 75% of all neural crest related CVA. The present pattern of CVA in infants with EA supports the concept that EA may be related to an abnormal contribution from caudal portion of cephalic neural crest.

Renal function adaptation up to the fifth decade after treatment of children with unilateral renal tumor: a cross-sectional and longitudinal study.

Mild‐to‐moderate renal function loss may be an independent risk factor for cardiovascular disease and overall mortality. As in adults with renal carcinoma nephrectomy is associated with an high risk for moderate renal function loss, we aimed to assess the renal function adaptation over a long period of time in children with unilateral renal tumor (URT).

Infantile Abdominoscrotal Hydrocele: A Not So Benign Condition

PURPOSE Infantile abdominoscrotal hydrocele is a rarely described condition. We report the outcome of the largest single institution experience managing these lesions. MATERIALS AND METHODS We retrospectively reviewed descriptive case series of all infants treated between January 1998 and December 2007. Postoperative followup ranged from 3 to 107 months (median 48). RESULTS A total of 18 consecutive patients underwent abdominoscrotal hydrocele repair at age 3 to 21 months (median 8). The first 13 patients underwent inguinal hydrocelectomy, which consisted of high ligation of the processus vaginalis and complete excision of the abdominal component of the lesion. The remaining 5 patients underwent plication of the tunica vaginalis accomplished through the scrotum. Included in this group were 2 patients initially treated expectantly because the lesion was without undue tension at diagnosis. Both cases demonstrated spontaneous resolution of the abdominal component of the lesion but ultimately required surgery for scrotal hydrocele. Overall 23 testes (5 bilateral lesions) were operated on, of which 18 had some degree of testicular dysmorphism detected by ultrasonography or during surgery and 15 fully recovered postoperatively. A total of 11 patients experienced 1 or more postoperative complications, including persistent scrotal swelling (7), hypoplastic testis (3), scrotal hematoma (2), inguinal hernia (1) and cryptorchidism (1). Morbidity related to scrotal hydrocelectomy was negligible. There were no recurrences of abdominoscrotal hydrocele in the series. CONCLUSIONS Tense infantile abdominoscrotal hydroceles are associated with a high rate of testicular dysmorphism, which is often reversed by early intervention. Simple transscrotal plication of the tunica vaginalis represents an effective procedure with decreased postoperative complications.

Radial artery pseudoaneurysm successfully treated by compression bandage

In children, surgery for radial artery pseudoaneurysm (PA) may be followed by growth retardation of the hand because of inadequate blood flow. We believe this is the first report of a child with PA of the radial artery cured by compression bandage. Conservative management is a safe and valuable initial treatment option for uncomplicated radial PA.

Angiographic diagnosis of jugular venous system dilatation in children. A report of five cases.

We report five children with a soft mass in the neck due to congenital jugular venous ectasia. Three had fusiform dilatation of the internal jugular vein, which in one case was associated with dilatation of the ipselateral external jugular vein. Two children had aneurysmal dilatation of the superficial cervical communicating vein. The first four cases required angiographic studies for final diagnosis. Venography via the femoral vein was most valuable for visualization of the dilated segments of internal jugular veins but failed to show the vascular mass communicating with the superficial vein of the neck. These were best visualized by direct injection of the contrast medium into the vessel. In the fifth case a correct diagnosis was obtained with xeroradiography alone.

In Reply: Enucleative Surgery for Stage I Nephroblastoma with a Normal Contralateral Kidney

PURPOSE Tumor enucleation is not recommended for children with nephroblastoma and a normal contralateral kidney. However, in adults with unilateral low stage renal cell carcinoma tumor enucleation may offer an alternative to radical nephrectomy, since functioning renal tissue is preserved without a greater risk of residual microscopic disease. Enucleative surgery may be more reasonable in children with nephroblastoma, because the risk of relapse can be reduced with chemotherapy. Therefore, we prospectively evaluated the feasibility of enucleative surgery in children with stage I unilateral nephroblastoma. MATERIALS AND METHODS Between 1992 and 1995, 13 children with nephroblastoma and a normal contralateral kidney were consecutively admitted to our surgical unit. Possible candidates for tumor enucleation were evaluated according to certain criteria, including stage I disease at diagnosis, well-defined margins on post-contrast computerized tomography and at least 50% of the functioning kidney could be preserved. Preoperative and postoperative chemotherapy was given in all cases. RESULTS Of 4 children with preservation of more than 50% of the functioning kidney 3 were considered eligible for enucleation. The tumors, which were confined to the mid kidney in 2 children and upper renal pole in 1, were successfully enucleated without hypothermia or vascular occlusion. All 3 children are disease-free at 49, 48 and 26 months of followup, respectively. Renal function has been almost completely restored postoperatively. CONCLUSIONS These preliminary data suggest that enucleative surgery may be a reasonable option in select children with stage I nephroblastoma and a normal contralateral kidney.

Elective partial splenectomy in childhood

BACKGROUND & PURPOSE The indications and results of elective partial splenectomy in children remain uncertain. The aim of this study was to determine (1) the indications for elective partial splenectomy (EPS), (2) the extent of splenic resection, and (3) the complications of EPS. METHODS Patients who underwent EPS from three centers over a 10-year period (1986 through 1996) were studied. Variables analyzed included clinical presentation and indications for EPS, extent of resection performed, perioperative transfusions, and complications. Postoperatively, splenic viability was determined by regular Doppler ultrasound scan and splenic function on peripheral blood film analysis. RESULTS EPS was attempted in 12 children for various conditions: giant epithelial cyst (n = 6), hypersplenism and metabolic disease (n = 4), pseudotumor (n = 1), and abscess (n = 1). One procedure was converted to total splenectomy because of thin splenic tissue around an infected giant cyst. No child required transfusion for splenic bleeding. There were no postoperative complications or deaths. Only 7 of the 12 children received prophylactic preoperative immunisation. In the 11 successful cases splenic remnant viability remains confirmed by ultrasound scan and normal blood film. There has been no significant splenic regrowth or recurrent hypersplenism in the four children with metabolic disorders. CONCLUSIONS EPS is suitable for benign splenic conditions and can be performed without major blood loss. Preoperative vaccination is advisable. Up to 95% of the spleen can be safely removed, basing the blood supply of the residual spleen tissue on peripheral polar vessels, with adequate postoperative function.

Chronic Kidney Disease in Children With Unilateral Renal Tumor

PURPOSE In patients who have undergone nephrectomy lower stage chronic kidney disease may develop, which is an independent risk factor for cardiovascular disease and overall mortality. We investigated whether the prevalence of lower stage chronic kidney disease is related to the amount of renal parenchyma excised in children with unilateral renal tumor. MATERIALS AND METHODS A total of 15 patients treated with nephrectomy and 10 treated with nephron sparing surgery were enrolled at a single academic center. The Kidney Disease Outcomes Quality Initiative guidelines were used to classify patients by chronic kidney disease stage based on estimated glomerular filtration rate values. The Modification of Diet in Renal Disease study equation and Schwartz equation were used in patients older and younger than 17 years, respectively. RESULTS At a mean followup of more than 12 years 8 patients who had undergone nephrectomy and 1 treated with bilateral nephron sparing surgery presented with stage II chronic kidney disease (estimated glomerular filtration rate 60 to 89 ml/min/1.73 m(2)). Sequential measurements from diagnosis to 12 to 17 years postoperatively showed that stage II chronic kidney disease in patients who had undergone nephrectomy manifested as a negligible postoperative increase in mean ± SD estimated glomerular filtration rate (75.7 ± 25.5 vs 79.4 ± 3.9 ml/min/1.73 m(2), p = 0.6). Five of the 8 patients presented with stage II chronic kidney disease even before nephrectomy. The other 7 patients who had undergone nephrectomy and those treated with nephron sparing surgery presented with a significant postoperative increase in mean ± SD estimated glomerular filtration rate (81.1 ± 24 vs 102.3 ± 3 ml/min/1.73 m(2), p = 0.02, and 88.7 ± 2 vs 107.4 ± 14 ml/min/1.73 m(2), p = 0.005, respectively). CONCLUSIONS A subset of children with unilateral renal tumor presents before and/or after nephrectomy, and not after nephron sparing surgery, with stage II chronic kidney disease, probably due to a reduced renal reserve capacity. Whether patients with preoperative renal dysfunction may benefit from nephron sparing surgery should be studied in a cooperative clinical trial setting.

Management of primary megaureter in infancy

The natural history of primary megaureter was evaluated in 26 infants (20 boys, 6 girls) with 38 nonrefluxing primary megaureters. Prenatal ultrasound diagnosis had been made in 21 infants. In all patients, primary megaureter was associated with pelvicaliceal dilatation. Two patients with urinary tract infection and two with agenesis of the contralateral kidney underwent surgical treatment. Asymptomatic infants with good differential renal function on DTPA scanning were managed conservatively regardless of their postfurosemide drainage curve. One of these infants later developed a slight deterioration in function and was submitted to surgery. One patient was lost to follow-up. Twenty patients, after a period ranging from 4 months to 8 years, showed partial or complete regression of ureteral dilatation with stable good renal function. In conclusion, many asymptomatic primary megaureters in infancy improve spontaneously and do not require surgical treatment.