Hypotonia and Lethargy in a Two-Day-Old Male Infant Adrienne H. Long, MD, PhD,a,b Jennifer G. Fiore, MD,a,b Riaz Gillani, MD,a,b Laurie M. Douglass, MD,c Alan M. Fujii, MD,d Jodi D. Hoffman, MDe A 2-day old term male infant was found to be hypotonic and minimally abstract reactive during routine nursing care in the newborn nursery. At 40 hours of life, he was hypoglycemic and had intermittent desaturations to 70%. His mother had an unremarkable pregnancy and spontaneous vaginal delivery. The mother’s prenatal serology results were negative for infectious risk factors. Apgar scores were 9 at 1 and 5 minutes of life. On day 1 of life, he fed, stooled, and voided well. Our expert panel discusses the differential diagnosis of hypotonia in a neonate, offers diagnostic and management recommendations, and discusses the final diagnosis. DRS LONG, FIORE, AND GILLANI, birth weight was 3.4 kg (56th PEDIATRIC RESIDENTS percentile), length was 52 cm (87th aDepartment of Medicine, Boston Children’s Hospital, d e percentile), and head circumference Boston, Massachusetts; and Neonatology Section, Medical A 2-day old male infant born at Genetics Section, cDivision of Child Neurology, and 38 weeks and 4 days was found to be was 33 cm (12th percentile). His bDepartment of Pediatrics, Boston Medical Center, Boston, limp and minimally reactive during physical examination at birth was Massachusetts routine care in the newborn nursery. normal for gestational age, with Drs Long, Fiore, and Gillani conceptualized, drafted, Just 5 hours before, he had an appropriate neurologic, cardiac, and and edited the manuscript; Drs Douglass, Fujii, and appropriate neurologic status when respiratory components. The infant Hoffman edited the manuscript; and all authors fi evaluated by the same nurse. He had uneventfully roomed with his mother approved the nal manuscript as submitted and agree to be accountable for all aspects of the work. breastfed once in the interim. during the first 2 days of life. He breastfed well every 2 to 3 hours, with DOI: https://doi.org/10.1542/peds.2018-0788 His mother received routine prenatal good latching during lactation Accepted for publication Dec 18, 2018 care in the United States without assessments. He appropriately stooled Address correspondence to Adrienne H. Long, MD, complications. Prenatal ultrasounds and voided. Routine physical PhD, Department of Pediatrics, Boston Children’s and maternal prenatal serologies were assessments were performed every Hospital, 300 Longwood Ave, Hunnewell Building, unremarkable. His mother had a history 8 hours per protocol, which revealed Pavilion 129, Housestaff Lounge, Boston, MA 02115. E-mail: [email protected] of a positive purified protein derivative a consistently normal physical tuberculin test and was treated for examination including neurologic PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). latent tuberculosis 3 years before. She status. The new finding on day of life 2 had no other medical history. The that he was limp and minimally Copyright © 2019 by the American Academy of Pediatrics parents immigrated from El Salvador reactive represented a significant 1 year before and had not traveled out change from his previous clinical status. FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this of the country since that time. The article to disclose. couple’s first child was a girl who died The pediatric resident on call presented FUNDING: No external funding. in her sleep in El Salvador on the to evaluate the patient. The infant was second day of life of unknown cause. found ill appearing with a temperature POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest The parents denied consanguinity. of 35.4°C, pulse of 110 beats per minute, respiratory rate of 55 beats per to disclose. The patient was born via spontaneous minute, and blood pressure of 92/50 vaginal delivery to a now gravida 2, mm Hg. Oxygen saturation was 94% on To cite: Long AH, Fiore JG, Gillani R, et al. para 2 mother with Apgar scores of 9 room air with intermittent brief self- Hypotonia and Lethargy in a Two-Day-Old Male Infant. Pediatrics. 2018;144(1):e20180788 and 9 at 1 and 5 minutes of life. His resolving desaturations to 70%. He was Downloaded from www.aappublications.org/news by guest on September 30, 2021 PEDIATRICS Volume 144, number 1, July 2018:e20180788 DIAGNOSTIC DILEMMAS sleepy with a weak cry. He was age, being an infant of a mother with for hypoglycemia and sepsis and normocephalic with no distinctive diabetes, or having a maternal history provide respiratory support. I would features. On pulmonary examination, of b-blocker usage.1,2 This patient did obtain a point-of-care glucose, he had mild supraclavicular and not have these risk factors. Although complete metabolic panel, complete subcostal retractions with good air the absence of such risk factors is less blood count (CBC) with differential, entry bilaterally. He was diffusely common, it does not exclude the blood cultures, and a lumbar hypotonic with a prominent head lag. possibility that hypoglycemia was the puncture with cerebrospinal fluid The Moro reflex was absent. He had instigating factor that led to the (CSF) studies and culture. Urine a good suck reflex. Palmar grasp and presentation. culture in this setting is of low utility plantar grasp were present but weak. because isolated urinary tract Muscle bulk was normal. He had Neonatal sepsis can also present infections are exceptionally normal cardiac, abdominal, similarly. Indications of neonatal uncommon in infants ,1 week old.10 fi dermatologic, and genitourinary sepsis are often nonspeci c and I would empirically start broad- examinations. He had strong radial include poor feeding, lethargy, spectrum antibiotics. Regarding the and femoral pulses and was well temperature instability (including patient’s desaturations and increased perfused throughout. hypothermia), and respiratory work of breathing, I would obtain symptoms. Furthermore, sepsis can a chest radiograph and venous blood lead to hypoglycemia through poor gas. I would tailor respiratory HOW WOULD YOU APPROACH THE feeding and increased metabolic support to the degree of respiratory INITIAL MANAGEMENT OF THIS demands. Although this patient does distress. NEWBORN WITH HYPOTONIA, not have risk factors associated with HYPOTHERMIA, AND DESATURATIONS early neonatal sepsis, namely positive Drs Long, Fiore, and Gillani IN THE NICU? WHAT INITIAL maternal group B streptococcus EVALUATION IS WARRANTED? (GBS) status, prolonged rupture of The infant was transferred to the NICU for further management. Dr Fujii, Neonatology membranes, or elevated maternal intrapartum temperature, sepsis Laboratory studies, including a full The constellation of symptoms, should still be high on the sepsis workup with lumbar puncture, including hypotonia, hypothermia, differential.3 Neonatal sepsis occurs were obtained (Table 1). Point-of- and desaturations, is alarming for in 1 in 1000 births and causes 15% of care glucose was 32 mg/dL. The CBC a serious process in this 2-day-old all neonatal deaths.4–6 Furthermore, was reassuring, with a normal white 3 3 infant. Although a broad differential although GBS infections remain the blood cell count for age (16.8 10 m should be considered, the acuity and leading cause of early-onset neonatal cells/ L) and normal immature-to- timing of the presentation can narrow sepsis (∼36%),7 ∼80% of GBS total granulocyte ratio (1%). The CSF the differential to a smaller set of infections occur in patients whose cell count was consistent with more likely diagnoses. For example, mothers had negative perinatal test a mildly traumatic tap without fi had the patient developed symptoms results.8 Thus, this patient warrants signi cant pleocytosis. CSF immediately after birth, hypoxic complete workup for sepsis and chemistries revealed a low glucose of ischemic encephalopathy (HIE) would empirical treatment. 26 mg/dL, consistent with systemic be high on the differential. However, it hypoglycemia and a mildly elevated would be unlikely for HIE to manifest In the general population, other protein of 165 mg/dL. Serum acutely after 48 hours of otherwise diagnoses beyond these are chemistry was normal except for an normal activity. On the basis of significantly rarer. Such diagnoses isolated elevation in aspartate pathophysiology and epidemiology, could include in utero insults, aminotransferase (AST) (432 U/L) hypoglycemia or sepsis would be high neuromuscular disorders, metabolic with a high-to-normal alanine on my differential. and/or genetic disorders, aminotransferase (ALT) (58 U/L). endocrinological issues, and Venous blood gas from the infant was Severe hypoglycemia itself can lead to 9 congenital heart disease. However, unremarkable. His chest radiograph a presentation similar to this infant’s, such diagnoses should remain higher was normal. with altered mental status, poor tone, on the differential in patients such as and an inability to maintain body this who have a positive family Given concern for a possible temperature. Desaturations in this history of infant death. infectious etiology, empirical setting could be explained by ampicillin, gentamicin, and acyclovir hypoglycemic seizures. In term Initial management should be focused were started. Dextrose containing infants, common risk factors for on stabilizing the infant and intravenous fluids was provided with hypoglycemia include being small for evaluating