A 6-day-old boy is brought to the emergency department due to spasms involving the whole body. The infant has had 3 days of difficulty latching on the breast, and this morning he was unable to nurse. The parents have been supplementing breastfeeding with a mixture of honey and goat milk. The infant lives in a religious commune with his parents, 3 older siblings, and 2 dogs. The members of the community do not accept vaccinations. The mother did not receive prenatal care and delivered vaginally with the assistance of her sister. The umbilical cord was cut with a pair of kitchen scissors. Temperature is 38.8 C (101.8 F). Examination shows an irritable infant with marked hypertonicity, neck stiffness, and inspiratory stridor. Auscultation of the heart and lungs is normal. The umbilical stump is swollen and has a small amount of purulent drainage. Cerebrospinal fluid analysis shows the following: Cultures of the blood, cerebrospinal fluid, and umbilical stump are pending. Which of the following is the most likely diagnosis?

Question

A 6-day-old boy is brought to the emergency department due to spasms involving the whole body.  The infant has had 3 days of difficulty latching on the breast, and this morning he was unable to nurse.  The parents have been supplementing breastfeeding with a mixture of honey and goat milk.  The infant lives in a religious commune with his parents, 3 older siblings, and 2 dogs.  The members of the community do not accept vaccinations.  The mother did not receive prenatal care and delivered vaginally with the assistance of her sister.  The umbilical cord was cut with a pair of kitchen scissors.  Temperature is 38.8 C (101.8 F).  Examination shows an irritable infant with marked hypertonicity, neck stiffness, and inspiratory stridor.  Auscultation of the heart and lungs is normal.  The umbilical stump is swollen and has a small amount of purulent drainage.  Cerebrospinal fluid analysis shows the following:  Cultures of the blood, cerebrospinal fluid, and umbilical stump are pending.  Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

11ec25c8_d6d6_efed_bc81_1fb4662cb9b4_MD0003_00 This neonate's clinical findings (feeding difficulty, spasms, and hypertonicity), in the setting of an unimmunized mother and poor cord hygiene, is highly suggestive of neonatal tetanus. Clostridium tetani is ubiquitous in the soil, and neonatal tetanus can result when at-risk infants are exposed to C tetani through unhygienic delivery or cord care practices. C tetani produces a toxin (tetanospasmin) that prevents inhibitory neurotransmitter release at the junction of upper and lower motor neurons. Uninhibited lower motor neurons lead to spasms and hypertonicity. tetanospasmin Neonatal tetanus presents in the first few weeks of life. Signs of umbilical infection are generally present, but the diffuse hypertonicity (opisthotonus) is the hallmark of neonatal tetanus. Feeding difficulty is due to trismus (lockjaw) from spasm of the muscles of mastication. Patients are at risk for life-threatening stridor and respiratory failure as the diaphragm and upper airway muscles contract. Treatment includes supportive care (eg, nutrition and respiratory support), antibiotics (eg, penicillin), and passive immunization with tetanus immune globulin. Routine immunization of children and pregnant women against tetanus has virtually eliminated neonatal tetanus in the developed world, but sporadic cases continue to occur among unimmunized communities. (Choices A and G) Neonatal meningitis can present in a similar manner as tetanus, including fever, neck stiffness, and hypertonicity. However, this infant's reassuring cerebrospinal fluid analysis makes meningitis unlikely. (Choice B) Neonatal botulism is transmitted by Clostridium botulinum (present in honey and soil) and presents with weakness, constipation, and poor feeding. In contrast to tetanus, neonatal botulism causes significant hypotonia. (Choice C) Febrile seizures are usually short, generalized seizures that occur during periods of fever in young children age 3 months to 5 years. This patient's age and clinical findings are inconsistent with febrile seizures. (Choice D) Omphalitis is a potentially severe cutaneous infection that begins at the umbilical stump with erythema, swelling, and pus and can lead to sepsis. However, omphalitis does not cause spasms or hypertonicity. (Choice E) Rabies can present with fever, muscle aches, and paralysis but does not cause hypertonicity. In addition, the incubation period of rabies ranges from weeks to months, making it even less likely in a 6-day-old. Educational objective: Neonatal tetanus can occur in infants born to unimmunized mothers, frequently following umbilical stump infection. Affected infants present in the first few weeks of life with trismus, spasms, and hypertonicity. Treatment includes antibiotics and tetanus immune globulin. References: Neonatal tetanus after home delivery: report of one case. (http://www.ncbi.nlm.nih.gov/pubmed/20675244)

A 6-Day-Old Boy Is Brought to the Emergency Department Due