Khalil Al Tawil

Perioperative course of pulmonary hypertension in infants with congenital diaphragmatic hernia: impact on outcome following successful repair

BACKGROUND Pulmonary hypertension (PH) associated with congenital diaphragmatic hernia (CDH) remains a significant cause of morbidity and mortality. For improved outcomes, preoperative stabilization is commonly practiced to control the PH in infants with CDH. Some CDH infants who have been considered stabilized and ready for surgery have nevertheless developed significant PH after surgical repair. In fact, the markers and consequences of the preoperative stabilization are still unclear. Therefore, we examine the perioperative course of PH to evaluate the impact of preoperative PH severity on mortality and morbidity of infants who underwent surgical repair of CDH. METHODS The medical charts of all newborns (n = 49) with CDH who were treated at our institution between January 2000 and December 2009 were reviewed. General management and perioperative data were evaluated for all infants. The ratio of estimated pulmonary artery pressure to systemic pressure (P/S ratio or PSR), based on echocardiographic data, was used to assess the PH severity during the perioperative period. RESULTS The overall survival rate in our group of infants with CDH was 71.4%. Of the 49 infants with CDH, 9 (18.4%) died during the preoperative phase. Forty infants underwent CDH repair at a median age of 3.5 days (range, 1-46 days). Five of these infants (12.5%) subsequently deteriorated and died after surgery. Using receiver operating characteristic curve analysis, a PSR cutoff value before surgery of 0.9 predicted mortality in CDH infants with a sensitivity of 100% and specificity of 84% and with an area under the curve of 0.93 (P = .002). Accordingly, 2 groups of infants with distinct outcomes were identified, as follows: a low-PSR cohort (PSR ≤0.9) with a survival rate of 100% and a high-PSR cohort (PSR >0.9) with a survival rate of 50% (P = .001). The rate of pneumothorax and the frequency of use of several inotropic agents after surgery were significantly higher in the high-PSR group (P = .001 and .007, respectively). Compared with low-PSR infants, infants with high PSR were operated on later (P = .03) and were postoperatively ventilated longer (P = .01). During the entire perioperative period, significant differences in the PH severity were noted between the 2 PSR groups. During the first week of life, infants in the high-PSR group had significantly higher PSRs than those in the low-PSR group (P = .001); and similar tendencies continued to be significant between the 2 groups after CDH repair (P = .04). CONCLUSIONS During the perioperative period, PH severity monitoring via the serial assessment of PSR is beneficial. Better outcomes were observed with a preoperative PSR less than or equal to 0.9, and this association needs to be confirmed by prospective study.

Risk Factors for Isolated Periventricular Leukomalacia

Periventricular leukomalacia, a major cause of neurologic disabilities in preterm infants, can be isolated or associated with intraventricular and periventricular hemorrhage. To determine the risk factors for isolated periventricular leukomalacia, we retrospectively studied the characteristics of all very low birth weight infants affected by isolated periventricular leukomalacia who were delivered over a 5-year period and compared them with a control group of very low birth weight infants, matched within 2 weeks for gestational age, with no central nervous system pathology, and born during the same period. In total, 20 affected infants were compared with 98 control infants. Neonatal sepsis caused by coagulase-negative Staphylococcus (P = 0.014) and neonatal seizure (P = 0.026) were associated with isolated periventricular leukomalacia only on univariate analysis. Three variables demonstrated statistically significant associations with isolated periventricular leukomalacia on both univariate and multivariate logistic regression analysis as independent risk factors: birth weight (odds ratio, 4.31; 95% confidence interval, 1.54-12.06; P = 0.005), early neonatal hypotension requiring combined inotropic therapy (odds ratio, 4.90; 95% confidence interval; 1.22-19.68, P = 0.025), and delayed surgical closure of hemodynamically significant patent ductus arteriosus beyond age 7 days (odds ratio, 1.20; 95% confidence interval, 1.06-1.35; P = 0.003).

Hypoparathyroidism-retardation-dysmorphism (HRD) syndrome in triplets

We report female triplets with the clinical and biochemical manifestations of hypoparatyroidism–retardation–dysmorphism (HRD) syndrome also known as Sanjad–Sakati syndrome. They were born at 35 weeks gestation after assisted pregnancy (in vitro fertilization). The parents are first degree cousins from Saudi Arabia.

Traumatic facial nerve palsy in newborns: is it always iatrogenic?

We sought to report an updated incidence, risk factors, and outcome of traumatic facial palsy (TFP) in newborn infants born at King Abdulaziz Medical City (KAMC), Riyadh, Saudi Arabia. We performed a 12-year retrospective study at KAMC. The records of infants with the diagnosis of TFP during the study period were reviewed and compared with 148 healthy term infants born during the same study period. Among 83,067 infants delivered between January 1994 and December 2005, 29 infants were diagnosed with TFP for an incidence of 0.03%. Forceps delivery and maternal primiparity were the only significant risk factors for TFP. Only 7 (24.1%) of these infants were delivered by cesarean section. The other 22 cases of TFP (75.9%) were delivered without any forceps application. Almost all infants (93%) with TFP had spontaneous recovery within 2 months. Only one child suffered from permanent facial weakness when he was last examined at the age of 12 years. Although forceps delivery was considered the most significant risk factor, currently most of the cases occur spontaneously without forceps application. The majority of infants with TFP will recover spontaneously within the first 2 months of life.

Management of inguinal hernia in premature infants: 10-year experience

Aim: Debatable issues in the management of inguinal hernia in premature infants remain unresolved. This study reviews our experience in the management of inguinal hernia in premature infants. Materials and Methods: Retrospective chart review of premature infants with inguinal hernia from 1999 to 2009. Infants were grouped into 2: Group 1 had repair (HR) just before discharge from the neonatal intensive care unit (NICU) and Group 2 after discharge. Results: Eighty four premature infants were identified. None of 23 infants in Group 1 developed incarcerated hernia while waiting for repair. Of the 61 infants in Group 2, 47 (77%) underwent day surgery repair and 14 were admitted for repair. At repair mean postconceptional age (PCA) in Group1 was 39.5 ± 3.05 weeks. Mean PCA in Group 2 was 66.5 ± 42.73 weeks for day surgery infants and 47.03 ± 8.87 weeks for admitted infants. None of the 84 infants had an episode of postoperative apnea. Five (5.9%) infants presented subsequently with metachronous contralateral hernia and the same number of infants had hernia recurrence. Conclusions: Delaying HR in premature infants until ready for discharge from the NICU allows for repair closer to term without increasing the risk of incarceration. Because of low occurrence of metachronous hernia contralateral inguinal exploration is not justified. Day surgery HR can be performed in former premature infant if PCA is >47 weeks without increasing postoperative complications.

Management of Pulmonary Interstitial Emphysema in a Premature Infant using Nasopharyngeal High-Frequency Oscillatory Ventilation

Pulmonary interstitial emphysema in mechanically ventilated premature infants is a serious complication that is difficult to manage and is associated with a poor prognosis [1]. We describe a premature infant with respiratory insufficiency secondary to severe pulmonary interstitial emphysema that was managed with nasopharyngeal highfrequency oscillatory ventilation, as a non-invasive mode of ventilation. Our case report does not clearly differentiate whether the improvement in gas exchange and eventual resolution of PIE are attributable to the use of non-invasive HFOV or to the use more appropriate low frequency (7Hz) with the non-invasive approach. Further studies of NP-HFOV use in infants with PIE are needed to support our report Conclusion: NP-HFOV may be utilized in infants with PIE associated with compromised respiratory function. NPHFOV may decrease the chance of further pulmonary complications, including pneumonia and lung atelectasis, and facilitate care of sick infants while on NP-HFOV.

Does Meconium Peritonitis Pseudo-Cyst Obstruct Labour?

Meconium peritonitis pseudo-cyst is very rare. Its perinatal management is controversial and can be associated with increased fetomaternal morbidity and mortality. A 34-week gestation infant with large meconium peritonitis pseudo-cyst, detected by intrapartum fetal ultrasound study, had abnormally increased ratio of fetal abdominal circumference to head circumference. Intrapartum aspiration of the pseudo-cyst was performed and was followed by a smooth vaginal delivery. The postnatal course of the baby showed that early laparotomy was essential for stabilisation of the infants general condition.

Neonatal necrotizing fasciitis and fungal infections in preterm infants

Necrotizing fasciitis (NF) in premature infants is a rare, yet life-threatening, soft tissue infection. We report four cases of NF that were encountered over a seven-year period. The onset of the disease was between four and twelve days of age. Predisposing factors were omphalitis in two infants and intestinal perforation in the other two infants, one of whom had necrotizing enterocolitis. Surgical biopsies from the affected sites showed mucormycosis in one infant and bipolaris fungi in another. One of these infants was treated with antifungal therapy in addition to broad spectrum antibiotics. This infant survived, even without surgical intervention while the other three died. We conclude that intestinal perforation and omphalitis are important predisposing factors of NF in premature infants. Fungal infection should be considered as one of the possible causes of NF in preterm infants. Early initiation of antifungal therapy, in addition to broad spectrum antibiotics, is justifiable in this disease entity.