Giant Sacrococcygeal Teratoma in a Preterm Neonate-Anesthetic Challenges: A Case Report

INTRODUCTION

Sacrococcygeal teratomas (SCTs) are among the most common congenital tumors in the neonatal period, with an incidence of 1 in 30,000–40,000 live births.^[1,2] Although often diagnosed prenatally, their management requires multidisciplinary planning. Anesthetic management for excision of SCT in a neonate poses challenges due to difficulty in positioning, prolonged duration of surgery, increased fluid and blood loss, susceptibility to hypothermia, and hemodynamic imbalances.^[3] Preterm neonates have poor reserves and additional challenges due to immature myocardium, lungs, kidneys, and higher risks of hypothermia, hypoglycemia, and sepsis.

CASE REPORT

A female baby was delivered by emergency lower-segment cesarean section at 34⁺¹ weeks of gestation due to fetal distress and a prenatally detected sacrococcygeal mass. The extraction was difficult due to the large cystic mass in the sacral region. Baby weighed 2.9 kg, and the weight of the mass measured as 1.4 kg. The baby cried immediately after birth, with a 1-minute appearance, pulse, grimace, activity and respiration (APGAR) of 8, but subxiphoid retraction was noticed, and the baby soon started to desaturate, with SpO₂ (oxygen saturation) reaching 40% on oxygen through a mask. The baby was subsequently intubated by the pediatrician with a 3-mm uncuffed endotracheal tube (ETT) in view of rising oxygen requirement and was shifted to the neonatal intensive care unit (NICU). Clinical examination and X-ray confirmed the prenatal ultrasound findings of a large cystic lesion measuring 15 × 10.5 cm involving the sacrococcygeal region [Figure 1]. Baseline investigations were within normal range, hemoglobin of 15.4 g/dL, total leukocyte count of 8,200, and platelet count of 1,24,000. IV fluids were administered as per the protocol (10% dextrose in water at 5 mL/h) through a peripherally inserted central catheter (PICC) line (Fr gauge 1) in the right cubital vein. 2-D Echo revealed situs solitus, levocardia, with no shunt lesion and normal biventricular function. Postnatal ultrasound showed a cystic solid lesion with vascularity and an area of calcification, and a diagnosis of Altman type I SCT was made. Magnetic resonance imaging (MRI) could not be done due to the risk involved in shifting the patient to the MRI suite.

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Figure 1: X-ray of the baby with a sacrococcygeal mass.

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The baby was scheduled to undergo surgical excision of the mass, the next day (day 2 of life). Pre-operative evaluation was done in the NICU, which included birth history and examination of vitals, airway, and sacral mass. Note was made of the available investigations. The patient was brought to the operating room (OR) from the NICU in a transport incubator with attached monitors and on oxygen support through the ETT in situ. After establishing appropriate interdisciplinary communication among the surgeon, pediatrician, and anesthesiologist, we proceeded with the anesthetic management. IV fluids were attached to the PICC line, and two more 24-G cannulae were secured, one in each upper limb. Anesthesia was induced with atropine (0.02 mg/kg), propofol (1.5 mg/kg), and atracurium (0.5 mg/kg). A check laryngoscopy was done with Miller’s 00 blade, and the ETT position was confirmed by bilateral chest auscultation and ETCO₂. ETT was attached to the anesthesia machine through the pediatric circuit and put on pressure-controlled ventilation (PCV) mode with inspiratory pressure of 18 cm H₂O, respiratory rate of 35–40/min, peak end-expiratory pressure (PEEP) of 5 cm H₂O, and FiO₂ of 45%. A mixture of oxygen with air and sevoflurane was used for maintenance of anesthesia. Fentanyl (2 mcg/kg) and paracetamol (15 mg/kg) were administered for analgesia. Standard American Society of Anesthesiologists monitoring was done including temperature, and a precordial stethoscope was fixed. Warmed 5% dextrose solution was infused at 10 mL/h. The OR temperature was maintained between 25°C and 26°C, and the baby was kept warm with a forced-air warming device (Bair Hugger).

Our next concern was the optimal positioning of the patient for surgery, in view of the huge size of the sacral mass. After discussion with the surgeon, it was decided to place the patient in the lateral decubitus position as the usual prone position might compromise the baby’s respiratory excursions and hemodynamic parameters [Figure 2].

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Figure 2: Patient positioning for surgery after induction of anesthesia.

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The maximum allowable blood loss (MABL) was calculated as 22 mL. Total blood loss was 50 mL as measured from the suction container and soaked gauze pieces, which exceeded the MABL. Hence, 30 mL of packed red blood cells and 50 mL of fresh frozen plasma were transfused.

Immediately after part preparation with betadine solution, the baby developed hypothermia (33°C by forehead skin temperature). Active warming measures were taken and it gradually increased to 33.8°C. At the same time, heart rate fell to 80 beats/min. 20 mL of crystalloids were given and adrenaline infusion (0.1 mcg/kg/min) started.

The total intraoperative duration was 4 h. Extubation was not done in view of continuing inotropic support and hypothermia. The baby was shifted intubated to the NICU for post-operative respiratory support. NICU stay was uneventful and adrenaline was gradually tapered and stopped on post-operative day (POD) 4. The baby was weaned off mechanical ventilation and extubated the same day. Drains and catheters were removed the next day and oral trophic feeds were started. The baby was on room air from POD 6 and remained in NICU for observation. The patient was discharged on 23^rd day of life, weighing 1.835 kg.

Histopathology reported gray-brown soft tissue measuring 17.5 × 13.2 × 8.5 cm, cyst wall thickness of 0.1–0.3 cm, composed of all three germ cell layers – ectoderm, mesoderm, and endoderm. Ectodermal derivatives were seen as stratified squamous epithelium, eccrine glands, along with mature neural tissue and ganglion cells, mesodermal derivatives as fibroadipose to fibromuscular tissue and cartilage, and endodermal derivatives as respiratory lining epithelium. The intervening areas showed moderate chronic lymphomononuclear inflammatory cells and congested blood vessels along with foci of calcification, suggestive of mature teratoma.

DISCUSSION

SCT is the most common neonatal germ cell tumor, derived from remnants of the primitive streak. with an incidence of 1 in 30,000–40,000 live births.^[1,2] In under 5-month age, they are mostly benign in 75% cases, malignant in 12%, and remaining are termed as “immature” and the American Academy of Paediatrics Surgical Section classified four types: Altman Type I: Predominantly external tumor or with minimal presacral component, Altman Type II: Mostly external but with some intrapelvic extension, Altman Type III: Apparent externally but predominantly intrapelvic mass extending into the abdomen, and Altman Type IV: Entirely interna – Presacral or retrorectal.^[3] Large SCTs, particularly in preterm infants, pose significant anesthetic challenges due to immature organ systems, potential cardiopulmonary compromise, high metabolic demands, and risk of rapid decompensation.^[4]

In our case, total weight at birth was 2.9 kg but the tumor itself weighed 1.4 kg, making the baby’s actual birth weight as 1.5 kg. The baby cried soon after birth (APGAR of 8 at 1 min) but developed respiratory distress soon after. Although the sacral mass was largely external, factors such as its high vascularity and venous pooling, increased metabolic demand, and compromised preload could have contributed to altered diaphragmatic mechanics in the form of reduced excursions and reduced lung compliance, leading to respiratory distress and desaturation in the immediate postnatal period, necessitating early airway management.

Positioning for surgery was a critical challenge in this baby. Commonly used surgical positions for resection of SCT include prone, lateral, supine-lateral, and modified prone positions. From surgical point of view, the prone position is a favored one. However, after taking due consideration of the airway, hemodynamic parameters, a decision was taken for the left lateral decubitus position.

During maintenance of anesthesia, PCV mode for ventilation was used with minimal PEEP and optimal FiO₂ of 45%. This allowed maintenance of oxygenation and gas exchange while preventing pulmonary barotrauma in the premature baby.

Preterm neonates have minimal physiological reserves and reduced tolerance to fluid and blood loss.^[4] Intraoperative blood loss of 50 mL exceeded the MABL (22 mL) which was duly replaced with packed cells and fresh frozen plasma. It is important that vigilant monitoring and accurate and meticulous estimation of such small blood losses (50 mL) are essential.

Hypothermia is a common hazard in neonatal surgery, due to the high surface area-to-weight ratio, prolonged exposure, use of cold antiseptics, and cleaning solutions should be prevented.^[5] We maintained the OR temperature at 25–26°C with the aim to prevent hypothermia, reduce metabolic demand, and maintain hemodynamic stability. Forced air warmers and warmed IV fluids were also used. Still, there was significant hypothermia accompanied by bradycardia. This was managed by inotropic infusion, active warming, and necessitated post-operative ventilation. Temperature monitoring is essential for neonates undergoing prolonged surgeries.

Bradycardia in our patient had a multifactorial etiology: Hypovolemia, vagal stimulation, reduced systemic vascular resistance, fluid and blood loss, and hypothermia. The rationale for choosing adrenaline infusion over atropine as a choice for treatment of bradycardia was in view of the combined inotropic and chronotropic effects of adrenaline, to avoid a situation of refractory hypotension and persistent low cardiac output, following bradycardia, as the response to a crystalloid bolus was poor.

Multidisciplinary coordination between an anesthesiologist, pediatrician, and surgeon was pivotal in this case. Literature emphasizes that outcomes improve with planned perioperative strategies, including early airway management, pre-established vascular access, readiness for transfusion, and post-operative intensive care.^[6] In our patient, despite the challenges of prematurity, large tumor burden, and intraoperative hemodynamic instability, hypothermia, blood loss, vigilant monitoring, meticulous estimations, timely interventions, and post-operative care in the NICU facilitated smooth sailing through the intraoperative period and survival to discharge in the post-operative period.

CONCLUSION

Managing a preterm neonate with a large SCT is challenging because every aspect of care has very little margin for error. In this case, we were faced with multiple issues such as respiratory compromise soon after birth, difficulty in positioning, blood loss that quickly exceeded the infant’s allowable blood loss, hypothermia, and bradycardia. Each of these required prompt recognition and timely intervention. What made the difference was a flexible approach. The decision to operate in the lateral position was dictated purely by the baby’s respiratory status, reminding us that physiology must guide surgical convenience. Similarly, having adequate vascular access lines and ready availability of blood and blood products allowed us to act in a timely.

The key factors for a successful outcome in critical neonatal surgical patients include a close coordination between the anesthesia, pediatrician/neonatologist, and surgical teams, good perioperative planning, optimal positioning for surgery, and vigilant monitoring. This case highlights that the successful anesthetic management in such fragile patients depends less on following a set formula and more on anticipation, vigilance, and teamwork.