Prognosis of isolated congenital diaphragmatic hernia using lung-area-to-head-circumference ratio: variability across centers in a national perinatal network.

OBJECTIVE: Congenital diaphragmatic hernia (CDH) is a severe anomaly. The observed-to-expected lung-area-to-head-circumference ratio (o/e-LHR) has been shown to provide a useful prediction of subsequent survival of fetuses with CDH in referral centers with expertise and a large caseload. However, the accuracy of o/e-LHR measurements in general fetal medicine units with less expertise is not well known. The aim of this study was to evaluate the capacity of o/e-LHR to provide a useful prediction of mortality of fetuses with CDH when the measurement is performed in fetal medicine units with different levels of expertise. METHODS: Between January 2008 and November 2013, 305 live births with expectantly managed left-sided isolated CDH were recorded in the database of the French National Center for Rare Diseases (31 centers) and followed up after birth. Centers were grouped into two categories according to their mean annual CDH caseload over the study period: large centers with an average of ≥ 14 cases and smaller centers with < 14 cases per year. The relationship between o/e-LHR and 28-day and 6-month mortality was modeled using fractional polynomials and the predictive value of o/e-LHR was quantified using the area under the receiver-operating characteristics curve. Comparisons between the two center categories were carried out. Analyses were adjusted for potential confounders such as thoracic herniation of the liver and gestational age at birth and at diagnosis. RESULTS: During the study period, two large centers managed a total of 82 CDH cases and 29 smaller centers a total of 223 CDH cases. Overall, there was a significant inverse relationship between 28-day mortality rate and o/e-LHR, which decreased from 54% when o/e-LHR was 20% to 6% when o/e-LHR was 75% (P < 0.01). When the category of center was considered, adjusted associations between o/e-LHR and 28-day mortality were significantly different (P = 0.032) between large and smaller centers. The ability to predict survival at 28 days postpartum based on o/e-LHR was better in large centers; for a specificity of 0.30, the sensitivity was 0.71 in large centers and 0.55 in smaller ones. The results were similar for 6-month mortality. CONCLUSIONS: Our results show that o/e-LHR measured on two-dimensional ultrasound is a good indicator of neonatal prognosis in cases of CDH that may be used even in fetal medicine centers with a small caseload. However, our results also suggest that LHR measurement may be difficult to perform correctly. Therefore, appropriate training should be offered to professionals. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Testicular prostheses in children: Is earlier better?

INTRODUCTION: The absence of a testis occurs for various reasons in children, but testicular prosthesis implantation in children is uncommon. The optimal time for prosthesis placement is still unclear, and its complication rate has been poorly studied in children. OBJECTIVE: The aim of this study was to determine the risk factors of complications in cases of testicular prosthesis implantation in children. STUDY DESIGN: A monocentric, retrospective review was performed of children implanted with a testicular prosthesis between 2008 and 2014. All implantations were performed through an inguinal incision with a standardized procedure. Children were divided into two groups depending on the interval after orchiectomy: (A) early implantation (delay between surgeries <1 year); and (B) delayed surgeries (delay ≥1 year). Statistical analysis was performed with Student and Fisher tests. RESULTS: Twenty-six patients (A, 15; B, 11) had a total of 38 testicular prostheses placements. Mean follow-up was 36.2 months. First surgery was performed at the mean age of 11.8 years (range 0-17.9) (A, 14.1; B, 8.1; P = 0.01) and testicular prosthesis implantation at the mean age of 14.7 years (range 9-18) (A, 14.3; B, 14.6) with a mean delay of 36.1 months (A, 1.3; B, 80.3). Indications were mainly spermatic cord torsion (27%), bilateral anorchia (27%), and testicular atrophy after cryptorchidism surgery (19.2%). Complications (10.5%) included two cases of extrusion, one infection and one migration. Patient 1 had a history of acute lymphoblastic leukemia with testicle relapse 2 years after induction therapy. High-dose chemotherapy, total body irradiation and bilateral orchiectomies were performed, and bilateral prostheses were implanted 12 years after the end of chemotherapy. Complications happened 85 days after surgery. Patient 2 was followed-up for a proximal hypospadias. The tunica vaginalis flap, which was used during a redo urethroplasty, lead to testicular atrophy. Thirteen years after the last penile surgery, a testicular prosthesis was placed through an inguinal incision, and extrusion occurred 203 days after surgery. Bacterial cultures of the prostheses were sterile and histological review showed no sign of granuloma or graft rejection. The complication rate was significantly higher if the delay between the two surgeries exceeded 1 year (P = 0.01). Indications of orchiectomy, prior scrotal incision, and prosthesis size were not risk factors. CONCLUSIONS: Testicular prosthesis implantation was relatively safe in a pediatric cohort. The complication rate was significantly higher if the delay between the orchiectomy and the prosthetic placement exceeded 1 year. These results suggest that reducing the delay between orchiectomy and prosthesis implantation may lead to fewer complications.

Is laparoscopic management suitable for solid pseudo-papillary tumors of the pancreas?

PURPOSE: Solid pseudo-papillary tumors (SPT) are rare pancreatic neoplasms of low-malignant potential occurring mainly in young women. The purpose of this report is to describe our experience with laparoscopic management of these tumors with 4-year follow-up. METHODS: Three children with SPT were admitted to two hospitals in Paris, France, between February 2000 and December 2006. Diagnosis or treatment was carried out using laparoscopic techniques (biopsy and resection in one case and biopsy only in two). Long-term follow-up data were collected. RESULTS: All three patients presented recurrences within 3 years after resection, i.e., disseminated peritoneal recurrence in two patients and local recurrence in one. The two patients with peritoneal recurrences were treated by surgical resection and chemotherapy. The patient with local recurrence could not be treated due to contraindicating local factors. All three patients were alive at the time of this writing. CONCLUSION: This is the first report describing long-term follow-up after laparoscopic management of SPT. All three patients developed recurrences. These poor results contrast sharply with the low risk of local or disseminated recurrence after open laparotomy without chemotherapy that has been considered as the treatment of choice up to now. Recurrences after laparoscopic management may have been due to diffusion of tumor cells caused by gas insufflation especially during biopsy. Laparoscopic biopsy should not be performed in patients presenting SPT.

Laparoscopic distal pancreatectomy for Frantz's tumor in a child.

Solid pseudopapillary tumor of the pancreas is a rare pathologic entity. Although the role of laparoscopy in surgery of the pancreas is still controversial, laparoscopic distal pancreatectomy has been reported with good results in adults. We report a laparoscopic spleen-preserving distal pancreatectomy in a 9-year-old boy who presented with a low-grade malignant tumor. Needle biopsy was impossible. A laparoscopic spleen-preserving distal pancreatectomy was performed. We used four trocars, and the operative time was 240 min. Conversion to open surgery was not necessary. The boy's postoperative recovery was uneventful, and he was discharged on the 6th day. CT-scan control at 6 months was normal. This case shows that even in advanced surgical cases, such as spleen-preserving distal pancreatectomy, laparoscopic procedures can be done safely, within a reasonable operative time, in children.

Iatrogenic pharyngoesophageal perforation in premature infants.

BACKGROUND: Premature infants are particularly at risk of iatrogenic pharyngoesophageal perforation. It is a rare occurrence but when it does occur it often mimics esophageal atresia. In the light of 10 patients treated in our service and those reported in the literature we have highlighted the diagnostic difficulties and discussed the appropriate management. PATIENTS: Between 1980 and 1995, we treated 10 premature neonates for pharyngoesophageal perforation. Six of these neonates weighed less than 1500 g. Esophageal atresia was the primary diagnosis in 4 cases. The pharyngoesophageal perforation was caused by repeated airway intubation in 3 cases and by overenthusiastic routine postpartum suctioning or nasogastric tube (NGT) insertion in 7 others. Severe respiratory distress occurred in 7 neonates. A plain chest x-ray revealed a large right pneumothorax in 3 cases and an aberrant NGT in 3 other cases. Four neonates had a contrast esophagography and 4 neonates underwent endoscopy. Five cases were treated surgically. In 3 of these, esophageal atresia was the presumptive diagnosis and the perforation was only diagnosed intraoperatively via a right thoracotomy. One neonate required suturing of the perforation and another had a gastrostomy. In all 5 cases a mediastinal drain was left in situ. The 5 remaining neonates were treated conservatively with broad spectrum antibiotics, total parenteral nutrition, a silastic NGT and pharyngeal aspiration. One of these neonates had previously had a laparotomy for a colonic perforation. There was a good outcome in 4 neonates, one of whom required instrumental dilatation for an esophageal stricture. Bronchopulmonary dysplasia developed in 3 cases and necrotizing enterocolitis in 1 other case. Two neonates died. CONCLUSION: An iatrogenic perforation is often difficult to diagnose and can easily be confused with esophageal atresia. Clinical findings, a plain chest x-ray, an esophagography and endoscopy are helpful. Surgery can be avoided in most instances. The outcome is not always favorable especially as premature neonates are at risk of severe concomitant pathology.

Transabdominal amnioinfusion to avoid fetal demise and intestinal damage in fetuses with gastroschisis and severe oligohydramnios.

BACKGROUND/PURPOSE: Despite dramatic improvement in survival rate for neonates with gastroschisis, significant postoperative morbidity and a low mortality rate still occur. Furthermore, even in recent publications, some fetal death has been reported. Does this mean that antenatal diagnosis of gastroschisis is a missed opportunity? In fact, decreased amniotic fluid (AF) volume is observed in some fetuses with gastroschisis. However, oligohydramnios is associated with an increased risk of fetal suffering. When severe oligohydramnios is observed, intrapartum amnioinfusion, to restore AF volume, may help avoid fetal complications. METHODS: Two fetuses with gastroschisis and severe oligohydramnios were treated antenatally with amnioinfusion of saline solution. In one case, fetal heart beat decelerations were observed at 27 weeks' gestation among with the oligohydroamnios and serial transabdominal amnioinfusions were performed. In the second case, severe oligohydramnios was observed at 31, weeks and an amnioinfusion was performed. The 2 babies were delivered at 31 and 34 weeks, respectively. RESULTS: In both cases, exteriorized bowel was nearly normal at birth, and primary closure could be performed. Outcome was favorable, and they were discharged home on day 43 and day 54, respectively. CONCLUSIONS: Because fetuses with gastroschisis and oligohydramnios are part of a particular high-risk group, serial ultrasound examination and computerized fetal heart beat monitoring are necessary during the third trimester. In selected cases of gastroschisis associated with severe oligohydramnios, serial amnioinfusion may be required.

Conservative intestinal surgery to avoid short-bowel syndrome in multiple intestinal atresias and necrotizing enterocolitis: 6 cases treated by multiple anastomoses and Santulli-type enterostomy.

Neonates with multiple sites of intestinal atresia (MIA) may be predisposed to short-gut syndrome. Anastomoses of the intervening segments may prevent this complication. 5 neonates with MIA, one of them with a gastroschisis, were operated on: a proximal enterostomy was constructed, a side-to-end anastomosis as described by Santulli and several end-to-end anastomoses between the intervening intestinal segments (n = 3 to 7) were performed. An additional infant, initially operated on for a necrotizing enterocolitis (NEC) was managed with the same surgical procedure. Without use of this technique, the remaining length of small intestine would have been 28, 27, 40, 58, 70 and 7 cm. This technique enabled an intestinal length of 49, 54, 96, 107, 92 and 93 cm respectively to be achieved. Ileocecal valve was present in all 5 cases with MIA, but resected in the case with NEC. The enterostomy was reversed 7 weeks later. The initial outcome (delay of enteral feeding, duration of parenteral nutrition) was good: the babies were weaned from parenteral nutrition (PN) after a mean time of 90 days (48 to 163 days). The prognosis (mean follow-up: 31 months, range 14 to 57) was good with regards to growth and development and length of time required before adaptation to normal enteral feedings and stools. This surgical method allows complete decompression of the proximal jejunum so that nutriment can pass into the distal bowel allowing it to enlarge. In cases of MIA, a long tapering proximal enteroplasty is a better procedure than resecting more than 5-10 cm of the proximal distended and hypertrophied bowel. We prefer to perform an enterostomy in association with multiple anastomoses between intervening intestinal segments. The enterostomy is preserved for long enough waiting period to enable the reversion of the histochemical and morphological changes that may have taken place in the bowel.

[Traumatic pharyngoesophageal perforation in newborn infants]. / Perforation pharyngooesophagienne traumatique du nouveau-né.

BACKGROUND: Neonatal perforation of the esophagus appears to occur rarely and often can mimic esophageal atresia. This report presents 12 cases of pharyngoesophageal perforation with a review of the literature. PATIENTS: From 1980 to 1995, we treated 12 infants for pharyngo-esophageal perforation. Ten infants were pre-term, seven of them weighing less than 1,500 g. Five infants had severe respiratory distress. Four infants had repeated attempt on intubation of the airway and eight infants had a routine postpartum suctioning and gastric aspiration. On plain X-ray, a large right pneumothorax was observed in three cases and the nasogastric tube deviated widely from its expected course in three cases. Four infants underwent contrast esophagography and three infants esophagoscopy. In five cases esophageal atresia was the initial diagnosis. Five infants underwent a thoracotomy. A gastrostomy was performed in one case. The six remaining neonates were treated non-operatively: broad spectrum antibiotics, total parenteral nutrition, and silastic nasogastric tube. Follow-up was uneventful in five cases. One infant with an esophageal stricture underwent instrumental dilatation. Bronchopulmonary dysplasia occurred in two cases and necrotizing enterocolitis in one. Two infants died. CONCLUSION: Iatrogenic perforation remains a difficult diagnosis. Clinical findings, plain chest x-ray and oesophagography are helpful. Surgery can be completely avoided in most instances. Infants with low birthweight and prematurity are most at risk.

Congenital adenomatoid disease of the lung: prenatal diagnosis and perinatal management

Prenatal ultrasonographic (US) detection of congenital adenomatoid malformation (CAM) was made in 18 fetuses at 17 - 36 weeks' gestation and managed in our institution during a 10-year period (1985-1994). The lesion was left-sided in 13 cases, right-sided in 4, and bilateral in 1. According to Stocker's classification, 12 cases were type I, 4 type II, and 2 type III. The prenatal course was followed with serial US examinations in 13 cases; the size of the lesion was stable in 8 and decreased in 5. Mediastinal shift was usually observed, and amniotic fluid volume was increased in 4 cases. One fetus was aborted. Six infants presented with respiratory distress syndrome and required neonatal surgery; delayed surgery was performed in 9 cases. Spontaneous regression of the lesion was observed on follow-up in 2 cases. Surgery consisted in lobectomy in 8 cases and segmentectomy in 6. The presence of fetal hydrops, type III lesions, and bilateral lung involvement are prenatal factors known to be associated with a poor prognosis. However, this series and a review of the literature suggest that caution should be observed with regard to the initial impression when counseling the parents regarding prognosis.

Congenital adenomatoid disease of the lung: prenatal diagnosis and perinatal management.

Prenatal ultrasonographic (US) detection of congenital adenomatoid malformation (CAM) was made in 18 fetuses at 17 - 36 weeks' gestation and managed in our institution during a 10-year period (1985-1994). The lesion was left-sided in 13 cases, right-sided in 4, and bilateral in 1. According to Stocker's classification, 12 cases were type I, 4 type II, and 2 type III. The prenatal course was followed with serial US examinations in 13 cases; the size of the lesion was stable in 8 and decreased in 5. Mediastinal shift was usually observed, and amniotic fluid volume was increased in 4 cases. One fetus was aborted. Six infants presented with respiratory distress syndrome and required neonatal surgery; delayed surgery was performed in 9 cases. Spontaneous regression of the lesion was observed on follow-up in 2 cases. Surgery consisted in lobectomy in 8 cases and segmentectomy in 6. The presence of fetal hydrops, type III lesions, and bilateral lung involvement are prenatal factors known to be associated with a poor prognosis. However, this series and a review of the literature suggest that caution should be observed with regard to the initial impression when counseling the parents regarding prognosis.