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Fetal Surgery for Neck Masses Periprocedural Care

  • Author: S Christopher Derderian, MD; Chief Editor: Hanmin Lee, MD  more...
 
Updated: Sep 24, 2013
 

Patient Education & Consent

As the progression of cervical masses varies, counseling parents is challenging. Masses identified early in gestation associated with hydrops carry a high risk of fetal demise, and patient counseling is of paramount importance. Patients with masses that progress rapidly, constricting the trachea and esophagus, and survive to a viable gestation should be offered an EXIT-to-delivery procedure. Counseling should also include discussing fetal risk associated with EXIT-to-delivery, which may include asphyxia and prematurity.

From a maternal standpoint, based on placental location, a classic hysterotomy may be required, prohibiting the possibilities of future vaginal deliveries. Additionally, the risk of uterine dehiscence and rupture in subsequent pregnancies is not trivial and has been reported to occur following 14% of EXIT procedures.[10] Therefore, all mothers should be counseled on associated risks.

Elements of Informed Consent

Pre-Procedure Planning The EXIT procedure requires careful planning with a multidisciplinary team, typically including two pediatric/fetal surgeons, one obstetrician, one neonatologist, one ultrasonographer, one otolaryngologist, and one nurse scrub.

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Equipment

A direct laryngoscope, a rigid and flexible bronchoscope, and a tracheostomy kit should be immediately available.

A uterine stapler with absorbable staples (US Surgical Corporation, Norwalk, CT) is used to minimize blood loss during the hysterotomy.

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Patient Preparation

Anesthesia

Inhaled anesthetics, particularly isoflurane,[11, 12] are necessary for uterine relaxation and uteroplacental gas exchange, which have been demonstrated to be normal up to 54 minutes on uteroplacental support during EXIT delivery.[13]

Alpha-adrenergic agonists are frequently required to maintain maternal blood pressure, as high levels of isoflurane, which are needed for uterine relaxation, often result in hypotension.

In addition, nitroglycerin can assist with uterine relaxation, as the activation of guanylyl cyclase leads to increased levels of cGMP and decreased levels of intracellular calcium, resulting in uterine relaxation. Typically, a fetal cocktail consisting of a paralytic and narcotic is injected into the fetus intramuscularly.

Positioning

The patient is typically placed in the supine position on the operating room table. Occasionally, the patient may be placed in lithotomy position to increase available space for operating room staff.

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Monitoring & Follow-up

After birth, a follow-up CT scan or MRI should be obtained to confirm the diagnosis. Surgical management can be elective once an airway is established.

Postnatal management depends on the type, size, and location of the lesion. Cervical teratomas are frequently resected in the neonatal period to minimize risk of malignant transformation. They may involve the thyroid gland, so care should be taken during the dissection, and consultation with an endocrinologist for hypothyroidism may be required postoperatively. Vascular malformations are frequently managed medically, while lymphatic malformations benefit from sclerotherapy, surgical resection, or both.

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Contributor Information and Disclosures
Author

S Christopher Derderian, MD Post-doctoral Research Fellow, University of California, San Francisco, School of Medicine

S Christopher Derderian, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, South Carolina Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Shinjiro Hirose, MD Assistant Professor, Clinical, of Surgery, Pediatrics, and Obstetrics, Gynecology and Reproductive Sciences, University of California, San Francisco, School of Medicine; Clinical Director, Division of Pediatric Surgery, Director of Surgical Services, Fetal Treatment Center, Surgical Director of ECMO Services, Division of Pediatric Surgery, Department of Surgery, UCSF Medical Center

Shinjiro Hirose, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, International Pediatric Endosurgery Group, Pacific Association of Pediatric Surgery, International Fetal Medicine and Surgery Society

Disclosure: Nothing to disclose.

Chief Editor

Hanmin Lee, MD Professor of Surgery, Pediatrics, Obstetrics/Gynecology and Reproductive Health Sciences, University of California, San Francisco, School of Medicine; Chief, Division of Pediatric Surgery, Director, Fetal Treatment Center, Michael R Harrison, MD, Endowed Chair in Fetal Surgery, Surgeon-in-Chief, UCSF Benioff Children’s Hospital

Hanmin Lee, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association for Academic Surgery, Society of American Gastrointestinal and Endoscopic Surgeons, Society of University Surgeons, International Pediatric Endosurgery Group

Disclosure: Nothing to disclose.

References
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  2. Byrne J, Blanc WA, Warburton D, Wigger J. The significance of cystic hygroma in fetuses. Hum Pathol. 1984 Jan. 15(1):61-7. [Medline].

  3. Jordan RB, Gauderer MW. Cervical teratomas: an analysis. Literature review and proposed classification. J Pediatr Surg. 1988 Jun. 23(6):583-91. [Medline].

  4. Kerner B, Flaum E, Mathews H, Carlson DE, Pepkowitz SH, Hixon H. Cervical teratoma: prenatal diagnosis and long-term follow-up. Prenat Diagn. 1998 Jan. 18(1):51-9. [Medline].

  5. Bergé SJ, von Lindern JJ, Appel T, Braumann B, Niederhagen B. Diagnosis and management of cervical teratomas. Br J Oral Maxillofac Surg. 2004 Feb. 42(1):41-5. [Medline].

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  8. Liechty KW, Hedrick HL, Hubbard AM, Johnson MP, Wilson RD, Ruchelli ED. Severe pulmonary hypoplasia associated with giant cervical teratomas. J Pediatr Surg. 2006 Jan. 41(1):230-3. [Medline].

  9. Lazar DA, Olutoye OO, Moise KJ Jr, Ivey RT, Johnson A, Ayres N. Ex-utero intrapartum treatment procedure for giant neck masses--fetal and maternal outcomes. J Pediatr Surg. 2011 May. 46(5):817-22. [Medline].

  10. Wilson RD, Lemerand K, Johnson MP, Flake AW, Bebbington M, Hedrick HL. Reproductive outcomes in subsequent pregnancies after a pregnancy complicated by open maternal-fetal surgery (1996-2007). Am J Obstet Gynecol. 2010 Sep. 203(3):209.e1-6. [Medline].

  11. Biehl DR, Yarnell R, Wade JG, Sitar D. The uptake of isoflurane by the foetal lamb in utero: effect on regional blood flow. Can Anaesth Soc J. 1983 Nov. 30(6):581-6. [Medline].

  12. Dwyer R, Fee JP, Moore J. Uptake of halothane and isoflurane by mother and baby during caesarean section. Br J Anaesth. 1995 Apr. 74(4):379-83. [Medline].

  13. Crombleholme TM, Albanese CT. The fetus with airway obstruction. Harrison MR, Evans M, Adzick N, Holzgreve W,. The Unborn Patient: The art and science of fetal therapy. Third. Philadelphia, PA: W.B. Saunders Company; 2001.

 
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Cervical Teratoma in an infant delivered via EXIT procedure
 
 
 
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