Clinical Perinatal/Neonatal Case Presentation

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Clinical Perinatal/Neonatal Case Presentation Clinical Perinatal/Neonatal Case Presentation nnnnnnnnnnnnnn Lethal Pulmonary Hypoplasia and Hydrocolpos With Transverse Vaginal Septum in a Newborn: A Case Report and Review of the Literature William F. Liu, MD resuscitation. Intubation with positive inspiratory pressure (.50 cm Orestes Borrego, MD water) ventilation was required for the extremely noncompliant Michael Weiss, MD lungs. Apgar scores were 1, 6, and 6 at 1, 5, and 10 minutes, respec- Craig R. Sweet, MD tively. The initial newborn examination was remarkable for a small This is a case report of an unusual cause of Potter thorax. The abdomen was very distended. The infant had a slightly sequence. Autopsy showed lethal pulmonary hypoplasia in flattened nose and excess nuchal tissue, although no other anomalies association with a transverse vaginal septum, hydrocolpos, were identified. The lower extremities and labia majora of the pheno- and a secondary obstructive uropathy. typic female were edematous, and the vagina was difficult to visualize. The normally placed urethra was catheterized, with the bladder drain- ing and the abdominal mass remaining unchanged. Potter syndrome was originally described in infants with bilateral Initial chest x-ray showed a small thoracic volume with a right renal agenesis and associated lethal pulmonary hypoplasia.1 Since pneumothorax while on maximal conventional ventilatory support. A this original report, Potter syndrome or oligohydramnios sequence thoracostomy tube was placed, and the infant’s respiratory status have been associated with renal dysplasia or hypoplasia, polycystic stabilized over the first 36 hours of life on high frequency jet ventila- kidneys, lower urinary tract obstruction from multiple etiologies, tory support combined with a priscoline drip. and chronic leaking amniotic fluid.2 The mass was tapped for 220 ml of straw-colored fluid at 5 hours To our knowledge, this is the first reported case in the English of life. A repeat tap was obtained at 50 hours of life. The initial fluid literature of a newborn with autopsy-confirmed Potter syndrome analysis included the following: sodium of 88 mmol/liter, potassium with pulmonary hypoplasia, hydrocolpos, and a transvaginal of 12.6 mmol/liter, chloride of 60 mmol/liter, creatinine of 14.6 mg/ septum. dl, albumin of 0.6 gm/dl, and a total serum protein of ,2.0 gm/dl. A We present the case with a review of the literature. repeat tap had similar values, but its character was more cloudy yel- low in character. A cell count analysis on this second tap showed 3825 CASE REPORT red blood cells per cubic millimeter, 1850 white blood cells per cubic millimeter (with 91% neutrophils and 9% lymphocytes), and many A 2965-gm, 36-week preterm white infant girl of appropriate gesta- epithelial cells. tional age was delivered from a 29-year-old primigravida by elec- Abdominal x-ray demonstrated a large midline fluid-filled mass tive cesarean section due to a breech presentation. Antenatal ultra- with a suspected area of calcification in the right quadrant (Figure 1). sound had demonstrated hydronephrosis and a large midline Abdominal ultrasound showed a 12 3 5 3 5.6-cm cystic ab- abdominal mass. Oligohydramnios was apparent starting at 30 domino-pelvic mass that appeared separate and posterior to the blad- weeks’ gestation. Amniocentesis and karyotype at 30 weeks re- der. There was moderate bilateral hydronephrosis and hydroureter vealed the infant to be 46, XX (female karyotype), Fluid was col- (Figure 2). A repeat ultrasound 2 days after the initial tap did not lected from the mass, with chemical analysis showing the follow- demonstrate reaccumulation of the abdominal fluid. ing levels: sodium of 68 mmol/liter, chloride of 60 mmol/liter. Cranial ultrasound and echocardiography did not demonstrate Oligohydramnios persisted in this membrane-intact gestation. any definitive abnormalities. Blood, body fluid, and urine cultures Upon delivery at 36 weeks, the infant required immediate were negative for bacterial growth. Serum electrolytes were within Departments of Pediatrics/Neonatology (W. F. L.), Reproductive Endocrinology (C. R. S.), normal limits. Urinalysis was also unremarkable. The infant was Pathology (O. B.), and Radiology (M. W.), Lee Memorial Health Systems, The Children’s Hos- initially oligouric, but responded well to furosemide, with the urine pital of Southwest Florida, HealthPark Medical Center, Fort Myers, FL. output remaining between 1 and 2 ml/kg per hour. Address correspondence and reprint requests to William F. Liu, MD, The Children’s Hospital of Southwest Florida, Healthpark Medical Center, 9981 Healthpark Circle, Fort Myers, FL Respiratory status deteriorated, with progressive and persistent 33908. hypoxemia after 36 hours of life. There was no recurrent air block, Journal of Perinatology (1999) 19(6) 454–459 © 1999 Stockton Press. All rights reserved. 0743–8346/99 $12 454 http://www.stockton-press.co.uk Pulmonary Hypoplasia and Hydrocolpos With Transvaginal Septum Liu et al. Figure 1. Initial x-ray demonstrating the small thoracic volume, right pneumo- thorax, large fluid-filled abdominal mass. A bladder catheter is in place. There is a faint suggestion of intra-abdominal calcification in the right lower quadrant (arrow). Figure 3. Postmortem appearance of a large hydrocolpos (A). Superiorly, a small uterus (B) and right fallopian tube are noted. An autopsy was performed; findings included pulmonary hyp- oplasia with a lung weight/body weight of 0.0081. A hydrocolpos with a transverse vaginal septum of the lower third of the vagina (Figure 3) was also identified. There was moderate bilateral hydronephrosis with hydroureters and a normal appearing bladder (Figure 4). There were no other urogenital anomalies. DISCUSSION This case represents a rare cause of Potter syndrome or oligohydram- nios sequence. In this case, we believe that the transvaginal septum Figure 2. Abdominal ultrasound demonstrating the partially decompressed blad- led to hydrocolpos, which resulted in an obstructive uropathy. Hydro- der with a draining catheter in place. There is a large pelvic mass extending to the colpos has been associated with urinary obstruction causing bilateral abdomen, posterior to the bladder. hydroureter and hydronephrosis.3–9 When this is severe, there may be in utero oligo/anuria resulting in oligohydramnios. Some cases may be less severe and may present later in infancy as an abdominal mass and despite aggressive ventilatory support and pulmonary vasodila- with urinary retention and frequency.3,10 tion, the infant died of refractory respiratory failure at 55 hours of Oligohydramnios sequence is associated with fetal compression. A age. Serum creatinine was 1.7 mg/dl and blood urea nitrogen was 21 flattened nose, redundant nuchal tissue, low-set ears with inner can- mg/dl at death. thal skin folds, and a small, receding chin characterize Potter’s facies. Journal of Perinatology (1999) 19(6) 454–459 455 Liu et al. Pulmonary Hypoplasia and Hydrocolpos With Transvaginal Septum Sfar et al.8 reported two cases of hydrocolpos with neonatal death secondary to pulmonary hypoplasia. The first case was an infant with hydrocolpos and postaxial polydactyly consistent with McKusick- Kaufman syndrome. The precise etiology of the hydrocolpos was not clear. The physical features of Potter syndrome were described in this infant. The second case was an infant with ambiguous genitalia with imperforate anus. Both cases reportedly had postmortem findings of obstructive uropathy and pulmonary hypoplasia. There have been several cases of neonatal death attributable to respiratory symptoms without clear pathologic documentation. Many of these cases may have been due to pulmonary hypoplasia.3,6,7,9,24–26 Nonlethal respiratory difficulties have also been reported and may be attributable to an abdominal mass effect with subsequent Figure 4. Postmortem photograph showing bilateral hydronephrosis. elevation of the diaphragm resulting in pulmonary compres- sion.4,27–31 The extremities may be malpositioned with flexion contractures, and there may be intrauterine growth retardation. These infants typically Antenatal Diagnosis of Hydrocolpos die of respiratory failure from pulmonary hypoplasia.11 Shimada et al.32 reviewed 45 fetuses with antenatal diagnosis of geni- tourinary abnormalities and oligohydramnios. The average gesta- Pulmonary Hypoplasia tional age at detection of the oligohydramnios was 30 weeks, as was Pulmonary hypoplasia has been defined by the following: decreased our case. There were two cases of hydrometrocolpos, with a 50% mor- radial alveolar counts and a lung-to-bodyweight ratio of ,0.012 for tality rate. infants of $28 weeks’ gestation and ,0.015 for infants of ,28 In addition to oligohydramnios, our case presented with a large weeks’ gestation. Histologically, Wigglesworth12 showed that pulmo- midline pelvic abdominal mass and hydronephrosis. We could not nary hypoplasia combined with oligohydramnios detected at ,20 make a definitive antenatal diagnosis because the bladder was so weeks’ gestation showed a failure of growth and maturation of the compressed and poorly visualized. The false diagnosis of primary peripheral parts of the pulmonary acinus. There is a general delay in 24 epithelial maturation, a lack of elastic development, and low concen- lower urinary tract obstruction is common. Antenatal diagnosis of hydrocolpos or hydrometra should be suspected when a cystic midline trations of lung phospholipids. In contrast, pulmonary hypoplasia 24,33–36 seen with normal or
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