The nomenclature of congenital abnormalities has relevance in that specified diagnosis may signal other associated abnormalities of anesthetic significance. Useful terminology:

A child with any lesion more complex than a malformation should probably not be operated on-site in an LDC. Evaluation and consideration of transporting the child back to the more developed world where complex care is available are indicated. While it is disheartening to have to pass on difficult cases, such challenges can lead to catastrophes if undertaken without necessary sophisticated backup.

Examples of syndrome which are best operated in developed countries include:

A cleft lip is defined as any defect in fusion that occurs anterior to the incisive foramen. This point can be easily located in the adult as a small depression in the anterior portion of the midline of the roof the mouth. A cleft palate refers to any defect from this point posteriorly. Thus a cleft lip may be anything from a minor incomplete skin cleft to a complete interruption of the skin, gingiva, alveolar ridge, and part of the primary palate.

Cleft lip and palate is the most common congenital craniofacial anomaly and the fourth most common birth defect after congenital heart deformities, spina bifida, and limb deformities. In the United States, cleft palate alone has a prevalence of 6.35 per 10,000. Cleft lip with or without cleft palate occurs in 10.63 per 10,000 [1]. There are distinct differences in the occurrence of cleft lip and/or palate between various ethnicities, with an incidence of 1 in 1,000 live births in Caucasians, 3.6 in 1,000 in Native Americans, 2.1 in 1,000 in Japanese, and only 0.3 in 1,000 in African Americans. The overall incidence of isolated cleft palate is 1 in 1,500 live births and shows no ethnic variation.

Two thirds of orofacial clefting involve the lip and/or palate, and nearly one third involve the palate alone. The majority of cleft lip and/or palate cases are unilateral (80 %), and are more common on the left side. Midline clefts of the nose and/or lip are rare deformities. Isolated cleft palate occurs twice as often in females, while cleft lip with or without a cleft palate occurs more frequently in males.

Tanaka et al. [2] studied the number of live births with cleft lip or other congenital anomalies by soliciting data from national and international organizations covering for American states and 30 countries for the years 2002 to 2006. All data were normalized and reported per 10,000 live births. Descriptive statistics, in addition to correlation and regression, were used for analysis. Over the 5-year period studied the overall congenital anomaly rate increased in the United States and decreased internationally. The states with the highest and lowest rates were Maryland (21.46 per 10,000) and West Virginia (2.59 per 10,000), respectively. The United States cleft lip national rate averaged 7.75 per 10,000. Countries with the highest and lowest rates were Japan (19.05 per 10,000) and South Africa (3.13 per 10,000), respectively. Internationally, the rate of cleft lip declined, with an average overall prevalence of 7.94 per 10,000. The trends diverged over the 5-year period, as the rate was stable in the United States and the international rate declined.

When a mission arrives on-site, there are several priorities:

First, the surgeon must evaluate the child to see if he/she is a suitable candidate for repair and, along with a logistics person, assess to see where the case can be scheduled. If running a 4 table setup (2 OR tables per room), a spreadsheet is created which lists the expected duration of surgery. The anesthesiologist with the most pediatric experience should manage the youngest cases. The duration of the operative schedule should not exceed the endurance of the team. While the urge is to book as many cases as possible, the reality is that after 10–12 h, vigilance decreases and risks increase. There are other concerns in making the schedule, i.e., the success of the mission may be judged on the total numbers of cases done, and it is immaterial if there were 16 difficult cleft palates rather than 40 cleft lips. Only the total number of cases performed counts. Since the duration of a palate may be two to three times the length of a cleft lip, if the case volume is of concern, then it is definitely easier to do cleft lips and/or revisions than more palates. The adage that younger and sicker patients go earlier in the day certainly holds true here. Another consideration is timing the OR carefully so that the children are not fasted for prolonged periods, although that should be less of a concern if current recommendations to drink clear fluids up to 2 h before anesthesia are met.

If a pediatrician is available, he/she should be the first to screen for obvious signs of infection while conducting a history and physical examination. Birth history is a key factor. Those born before 37 weeks gestation, i.e., preterm have an increased incidence of perioperative respiratory complications. Many of these babies experience episodes of idiopathic apnea (defined as cessation of breathing for 20 s or more) until approximately 60 weeks post-conceptual age and therefore require additional monitoring postoperatively. These apneic episodes can occur up to 12 h after the completion of surgery. Since the resources for post-op monitoring may be very limited in the LDCs, surgery in premature babies may be problematic and therefore best avoided.

The degree of maternal–child bonding should also be assessed as this may be utilized during the induction period, i.e., the mother can hold and calm the child while a mask induction is performed. The mother also needs to be questioned about feeding problems. In cleft palate cases, the normal separation between food and air is absent, resulting in the mixing and deposition of food in the turbinates. This abnormality may impair nutrition, which in turn affects growth and development.

Dehydration may also be of concern and manifest subtly on physical examination. These children often do not suck well and may be relatively dehydrated. In all but the mildest cases, the child should be fluid repleted before starting the case.

Chronic rhinorrhea, which is caused by the cleft palate, needs to be differentiated by history and physical examination from the new onset of an infectious process. If the patient has a new fever, is irritable, and has an elevated white blood cell count, rescheduling the case until the process is winding down is judicious.

Carefully weighing the patient is important to assure that accurate dosage calculations can be made. The overall length and head circumference should be measured with the result plotted on standard childhood development curves. Children who are below the 10th percentile in weight demonstrate significantly delayed development and the cause needs to be determined prior to surgery. While it is very rare for the anesthesiologist to be the first to recognize a previously unrecognized syndrome in a child, it should be remembered that the anesthesiologist in the LDC setting may be one of the few physicians to actually examine the child and issues may have been missed by other examiners.

With respect to laboratory studies, a spun hematocrit is the barest minimum required since these patients are often anemic. If the patient has a hematocrit below 30 %, due consideration has to be given to the potential blood loss of the proposed procedure. A cleioplasty rarely loses a significant amount of blood while a cleft palate repair can involve significant blood loss even with the use of vasoconstrictors. The decision to proceed requires careful discussion as the blood bank facility may be limited or nonexistent.

The physical examination of the heart must be carefully done. The presence of a murmur needs to be discerned and a diagnosis made on-site as there is often no other consultation or investigations available, i.e., no echocardiography or cardiology consultation. The most common cleft lip/and or palate-associated cardiac problem is a ventricular septal defect and less commonly, atrial septal defects. Other physical signs such as clubbing or cyanosis may not be present in an infant.

The rest of the physical exam should be devoted to looking for other congenital abnormalities as up to 10 % of these patients also have a second significant defect.

The ASA standards for preoperative fasting were recently updated [3]. The European Society of Anesthesiologists (ESA) published guidelines [4] that closely mirror the ASA guidelines albeit with measures of common sense added, e.g., chewing gum or a cough lozenge is not a reason to cancel a case. These guidelines are summarized in Table 17.1.

It is appropriate to fast from intake of breast milk at least 4 h before elective procedures requiring general anesthesia, regional anesthesia, or sedation/analgesia.